Parotitis: Background, Pathophysiology, Epidemiology Pathophysiology Overview Background The parotid glands are small exocrine glands that rarely call attention to themselves. Perfect function throughout life is normal. Dry mouth, drooling, swelling, and pain are essentially the only symptoms caused by dysfunction of the salivary glands. The major salivary glands and their ducts are strategically situated on either side of the dental occlusal planes to irrigate and saturate a food bolus with saliva during chewing. The parotid gland contacts the mandibular ramus and muscles of mastication, which massage the gland during chewing. The mechanical squeezing and the parasympathetic nervous system, which analyzes a number of sensory inputs, cause the glands to inject an appropriate quantity and quality of saliva into the oral cavity. Minor salivary glands are scattered throughout the oral cavity and pharynx to assist the major glands in moistening, lubricating, and protecting the teeth and mucosa. The normal flow of saliva though the duct prevents oral bacteria from ascending the duct to cause infection. Inflammatory swelling of the glands may present a serious diagnostic challenge. Parotitis presents in many forms and the symptoms vary from modest to prostrating.

Reading the numerous journal articles on parotitis reveals frequent contradictions in the classification, etiology, and treatment of the disorders. A pure viral or bacterial infection, an autoimmune inflammation, or a combination of these can be the etiology. In this article, evolution of the knowledge of parotitis, as well as the diagnosis and treatment, is discussed. Infectious parotitis This group of diseases is caused by known infectious agents. Acute bacterial parotitis Acute bacterial parotitis is now infrequent, but its historical importance and occasional occurrence today necessitate in-depth knowledge of this entity by the otolaryngologist. Mumps and bacterial parotitis were differentiated by 1800, but neither was effectively treated. The mortality rate for bacterial parotitis was 80%. Before antibiotics and intravenous administration of fluids were available, bacterial parotitis occurred in postoperative patients or other severely ill patients who became dehydrated and contributed to their demise as an incurable sepsis . Early in the 20th century, surgeons were hesitant to incise and drain parotid abscesses and frequently used ineffective conservative measures until the process was irreversible. They feared the consequences of the unsightly scar and facial paralysis.

Parotid abscess is depicted in the image below. Elderly man with parotid abscess. View Media Gallery In 1917, Lilienthal described a surgical treatment that was very similar to what is used today. [ 1 ] He called parotid abscesses celiac parotitis because they were believed to be metastatic from abdominal infections. Other authors used names such as acute surgical parotitis, acute necrotic parotitis, acute gangrenous parotitis, and other historical designations according to Hemenway and English in 1971. [ 2 ] Lilienthal designed a vertical incision just anterior to the auricle that coursed posteriorly and inferiorly below the ear to join and follow an upper cervical skin crease that paralleled the lower mandibular border as seen in the image below. [ 1 ] He elevated the outlined skin flap forward to expose the parotid gland and made multiple incisions into the gland parallel to the facial nerve branches. He then opened the fascia behind the angle of the mandible to drain deeper spaces. The wound was packed and healed by secondary intention, resulting in a surprisingly good cosmetic result.

The number of patients treated by this drainage is not known, but this treatment was probably almost anecdotal to Lilienthal’s contemporaries. Incision outlined for incision and drainage of parotid abscess. View Media Gallery In 1919, Zachary Cope, a British Army surgeon, described 7 patients with parotitis that he had treated in Baghdad during the exceptionally hot summer of 1917. [ 3 ] He recorded that these soldiers had heatstroke or were severely affected by the extreme heat. The patients developed parotid swelling accompanied by fever and general malaise. Cope made wide T-shaped incisions in the gland to allow drainage. Four of the 7 survived after sloughing gangrenous parotid tissue. Cope stated that although the disease was a bacterial infection, the excessive heat and debilitating illness predisposed to its development. In 1923, Blair and Padgett of St. Louis published an article stating that early surgical drainage of the infected gland was safe and frequently was life saving. [ 4 ] They stated that acute suppurative parotitis was an ascending duct infection related to decreased salivary flow, fever, and general debilitation.

They cultured the pus and found that Staphylococcus aureus was the most common organism. The treatment proposed by Blair and Padgett did not become the standard practice for several more years. However, the high mortality rate decreased early in the 20th century and was 30-50% by 1930, probably because of more prompt and effective drainage of the abscesses. From the 1930s to the 1960s, irradiation treatment of numerous diseases became popular, and several authors advocated 4-6 Gy delivered over 4-5 days for bacterial parotitis. Most patients with severe infections required surgical drainage despite radiation treatment. By 1960, most published papers stressed large doses of antibiotics, improved oral hygiene, and increased fluid intake as treatment, with incision and drainage for failures. They found that the parotid capsule and septations required wide exposure and extensive deep incisions parallel to the facial nerve branches to exteriorize the diseased gland. Physicians recognized the importance of hydration and oral hygiene for debilitated patients, and the incidence of bacterial parotitis plummeted.

Parotitis is now more common in elderly patients because many take medications with an atropine effect that retards salivary flow and predisposes to ascending infection. Many psychotropic drugs are relatives of antihistamines. Acute parotitis in neonates This rare form of parotitis is lethal without treatment. In January 2004, Spiegel et al reviewed the literature and stated that only 32 cases had been reported in journals during the previous 3 decades. [ 5 ] The characteristic clinical picture was of a sick premature infant with unilateral parotid swelling and inflammation. Seventy-five percent of the cases were in male infants. Pus expressed from the duct cultured S aureus in more than half of the cases. Most all of the cultured bacteria were from organisms present in the oral cavity, which suggests an ascending infection from the mouth. Treatment is prompt administration of gentamicin and antistaphylococcal antibiotics plus adequate hydration, with a cure in approximately 80% of cases. Failure to improve after 24-48 hours of treatment necessitates surgical drainage. Recurrence is uncommon.

Acute bacterial parotitis in children between one year of age and adolescence is extremely rare and only a few have been reported. The etiology and treatment is the same as for adults. Chronic bacterial parotitis Chronic bacterial parotitis may exist in the presence of calculi or stenosis of the ducts secondary to injury. A number of articles and book chapters describe that chronic infection is a sequela of acute bacterial infection, but the evidence is scant. Most authors have suggested that decreased salivary flow was the common denominator, but reduced flow may be due to the inflammation. In most instances, the chronic disease is either autoimmune or of unknown etiology with superimposed bacterial infections and should not be designated as a chronic bacterial infection. Acute viral parotitis (mumps) Mumps, one of the classic childhood infections, is spread by droplets or by direct spread from oropharyngeal secretions that contain the paramyxovirus. Universal immunization, which began in 1977, has made the clinical disease unusual in developed countries. The child should receive the first measles, mumps and rubella (MMR) vaccine at age one year and a second at age 4-6 years.

[ 6 , 7 , 8 ] Occasional outbreaks of mumps are seen, mostly in teenagers or patients in their early twenties who did not receive the second shot. Before the vaccines were available, exposure was almost universal, and clinical disease resulted in 60-70% of those who were exposed. The disease was characterized by grossly enlarged and modestly tender parotid glands. Parotid stimulation caused pain in the gland and ear. Mumps was a benign disease in the vast majority of cases but was occasionally complicated by meningoencephalitis, pancreatitis, orchitis, or deafness especially in young adults. Treatment was and is symptomatic and supportive. HIV parotitis Generalized lymphadenopathy has long been associated with HIV , but the localized enlargement of the parotid gland is less well known. The course of the disease is different enough between children and adults to warrant a separate description. HIV parotitis in children Salivary gland involvement in children with HIV is well recognized and is much more common than involvement in adults. Characteristically, the gland is firm, nontender, and chronically enlarged (unilateral or bilateral) and usually causes few symptoms.

Lymphoepithelial cysts are less common than in adults. Xerostomia with decreased salivary flow rates occurs in adults but is infrequent in children. Infiltration of CD8-positive lymphocytes, possibly as a result of HIV, Epstein-Barr virus (EBV), or an interaction between the 2, enlarges the gland. The diagnosis of HIV parotitis is usually clinical with the typical findings. Other forms of chronic parotitis are rare in children. [ 9 ] The picture is not typical for acute bacterial infection. No specific treatment exists for this parotitis, and none is usually required. Some evidence indicates that parotid involvement is a good prognostic sign. HIV parotitis in adults The name lymphoepithelial lesion is frequently applied to HIV parotitis and adds confusion to the many names of parotid inflammatory diseases. A group at the State University of New York (SUNY) presented a series of 50 patients with HIV and masses in the tail of the parotid. Most of the patients were prison inmates and intravenous (IV) drug users.

Parotidectomy was performed in 35 patients, with patients earlier in the series undergoing lateral lobectomy and later patients undergoing local excision of involved tissue. Microscopic examination of the excised specimen revealed 3 types of involvement: (1) follicular hyperplasia of the parotid lymph nodes; (2) diffuse infiltration of the gland by lymphocytes (CD8 T cells) with appearance much like Sjögren disease; and (3) benign lymphoepithelial cysts that are the type of involvement most frequently described in the literature. The cyst walls are lined by stratified epithelium that may be squamous, columnar, cuboidal, or pseudostratified. Aggregates of lymphoid tissue are present within the cyst walls. The salivary parenchyma has a normal appearance. MRI shows the multiple cysts within the enlarged glands and is almost diagnostic. If the enlarged gland causes cosmetic deformity or pain, antiviral therapy, low-dose radiation therapy, or surgical excision may decrease the size of the gland. [ 10 ] Most authors suggest enucleation of large cysts.

In some individuals, the parotid gland symptoms are very similar to and may be confused with Sjögren syndrome. Parotitis in tuberculosis The incidence of pulmonary tuberculosis steadily decreased in the United States until 1985, but the trend reversed and has been slowly increasing since that time with a concurrent rise in extrapulmonary tuberculosis . [ 11 ] Tuberculosis is an uncommon cause of parotitis and is not particularly important, except that approximately 25% of patients have pulmonary tuberculosis and may infect their associates. Approximately 3% of patients with AIDS have tuberculosis, and almost 70% of these patients have pulmonary tuberculosis. In the past, extrapulmonary tuberculosis was apt to be due to atypical tuberculosis, such as the bovine variety, but today, most cases are due to Mycobacterium tuberculosis. Patients have enlarged, nontender, but moderately painful glands. Involvement is most frequently confined to the parotid lymph nodes, but the gland may become diffusely involved with the disease. The diagnosis is made by typical chest radiograph findings, cultures, or histologic diagnosis after the gland has been removed. The long delay for cultures prolongs the public’s exposure to the disease.

A positive skin test is not particularly helpful because of the high incidence of positive skin tests in the general population. Fine-needle aspiration biopsy occasionally yields Langerhans giant cells, which suggest tuberculosis. When diagnosed and treated with antitubercular medications, the gland may return to normal in 1-3 months. Untreated cases progress to draining fistulas and fibrosis. Chronic punctate parotitis (chronic autoimmune parotitis) Although acute bacterial parotitis is fairly well understood, chronic enlargement of the salivary glands with recurring infection has caused confusion for more than a century. Numerous terms found in the literature, such as Mikulicz disease, Sjögren syndrome, benign lymphoepithelial lesion of Godwin, chronic punctate sialectasis, and recurrent parotitis of childhood (as seen in the image below), are confusing to the physician. Sialography was commonly used in the workup of parotid disease in the past, and this group demonstrates punctate sialectasis, which implies point like dilatations within the gland. Six-year-old girl with recurrent parotitis of childhood.

View Media Gallery Mikulicz disease Johann Mikulicz-Radecki (1850-1905), a professor of surgery in Breslau, Poland, trained under Theodore Billroth of Vienna. In 1888, he encountered a 42-year-old farmer who had experienced lachrymal gland swellings followed by enlargement of the submaxillary and parotid glands. The enlarged lacrimal glands interfered with his vision and were excised by Mikulicz. The submaxillary glands were also excised, but the parotid glands were not removed. Mikulicz recorded in his publication that this was a benign disease and was not related to tuberculosis, leukemia, or malignant lymphoma. He believed the causative factor to be infection. No other patient with an identical disease has been described. Unfortunately, the name Mikulicz has been associated with numerous conditions that involve enlarged parotid glands. Early in the 20th century, numerous reports described conditions associated with enlargement or inflammation of the salivary glands.

In 1927, Schaffer and Jacobson presented a simplified grouping with only 2 major divisions: [ 12 ] (1) cases in which tuberculosis, leukemia, or some other disease had enlarged the gland and (2) Mikulicz disease, in which these other diseases were ruled out. The term "Mikulicz disease" probably should be discarded because of ambiguous definition. However, the term is so popular that it will not soon go away. Sjögren syndrome The next major milestone in the understanding of chronic parotitis was the publication of a paper in 1933 by Henrik Sjögren, a Swedish ophthalmologist. [ 13 ] In 1930, Sjögren observed his first cases of keratitis sicca and began a systematic study of the disease that lasted more than 20 years. In his landmark publication, he named the disorder keratoconjunctivitis sicca . Sjögren published so extensively that by 1936, the entity was referred to as Sjögren syndrome, a name that today is widely employed throughout the world.

Sjögren reviewed the available literature on the subject and published histopathologic studies of the cornea, conjunctiva, and lachrymal glands. He reported that this disease affected menopausal women in whom the keratoconjunctivitis sicca was a local phenomenon. He stressed that arthritis was a significant feature of the disease and occurred in most patients. Patients had an elevated erythrocyte sedimentation rate. Hypochromic anemia and fever were occasionally present. Sjögren revived the Schirmer test for measuring tear secretion and popularized the use of the Rose Bengal staining technique for the diagnosis. Sjögren syndrome, similar to other connective tissue diseases, is a multisystem disorder with diverse features. The entity is classified by some as a primary condition (primary Sjögren syndrome) or is associated with autoimmune diseases such as lupus erythematosus or rheumatoid arthritis (secondary Sjögren syndrome). In the fully developed syndrome, most organs seem to be involved. Most authors classify the disease as definite Sjögren syndrome, which includes (1) objective evidence of keratoconjunctivitis sicca or (2) characteristic pathologic features of the salivary glands.

The probable Sjögren syndrome requires 2 out of 3 of the following: (1) recurrent chronic idiopathic salivary gland swelling, (2) unexplained xerostomia, and (3) connective tissue disease. The disease most commonly appears in people aged 40-60 years, but it may affect small children. In Sjögren syndrome, the prevalence of parotitis in women versus men is approximately 9:1. The involved parotid gland is enlarged and tender at times. Massage of the gland produces clear saliva with flocculated clumps of coagulated proteins. The next major development in the evolution of knowledge of chronic parotitis was a collective pathologic study of "Mikulicz Disease" by Morgan and Castleman in 1953. [ 14 ] They reported 18 cases and stated that the pathologic involvement was uniform in all of these cases. The basic features are massive lymphoid infiltration with atrophy of the acini, proliferation of the cells of the small ducts that leads to narrowing of the lumen, and, finally, obliteration.

The functioning parts of the gland (acini) are destroyed, leaving the appearance of large lymph follicles. However, among this distorted architecture, islands of ductal epithelial cells and myoepithelial cells that appear fairly healthy can be observed. These groupings are called epimyoepithelial islands. The large ducts appear to be normal. In the Morgan and Castleman series, some of the surgical procedures were radical parotidectomies, including severing the facial nerve, because the surgeon believed that the condition was a malignancy. For further reading on Sjögren syndrome, please see the Medscape Reference articles on the subject in our Pediatrics , Ophthalmology , Emergency Medicine , Rheumatology , and Dermatology sections. Lymphoepithelial lesion of Godwin John T. Godwin, a New York pathologist, published a series of 11 parotidectomy cases in 1952 that he diagnosed as benign lymphoepithelial lesion of the parotid gland. Two of the patients had undergone radical parotidectomy followed by irradiation because of the mistaken belief that the lesions were malignant. Three other patients were treated with irradiation.

Some had bilateral enlargement of the parotid glands and dry eyes. As a pathologist, he had not interviewed the patients, and the recorded information was incomplete. [ 15 ] Godwin discussed the work of Mikulicz but did not mention Sjögren, although the history of the cases reveals that some undoubtedly were Sjögren syndrome. Several of the patients underwent sialography that revealed punctate sialectasis. He suggested that sialography might be helpful in the diagnosis but that a needle-aspiration biopsy was not adequate for diagnosis. He suggested incisional biopsy for diagnosis and determined that extensive surgery was not necessary. Godwin noted that several of the glands were diffusely involved, while some had well-circumscribed areas of tumor. The Godwin name is most frequently associated with a circumscribed tumor with the histologic features of Sjögren syndrome. Why an autoimmune process directed against the salivary gland tissue would be so localized within the gland is difficult to understand. Pathogenesis of autoimmune parotitis The autoimmune diseases listed above appear to be the same disease process with different manifestations. Initial insult to the gland may be a viral infection.

Peptides derived from viral antigens and autoantigens become associated with class II histocompatibility molecules in the cytoplasm of the epithelial cell, and the human leukocyte antigen (HLA) complex is subsequently expressed on the cell surface. CD4+ T cells recognize these antigens and release a series of cytokines, which promote further T-cell activation. B cells enter the gland and produce autoantibodies, including anti–Sjögren syndrome antibodies (ie, anti–SS-A, anti–SS-B) and rheumatoid factor (RF). B cells with cell surface RF can concentrate immune complexes and present antigens to CD4+ T cells. The acini are destroyed by this autoimmune mechanism. Continued cell division of specific B cells leads to oligoclonal expansion and increased chance of karyotypic error associated with neoplastic transformation. Diseases of uncertain etiology Salivary stone (sialolithiasis) Formation of stones within the duct system is one of the more frequent disorders of the salivary glands. The exact cause is not known, but most agree that the stone begins as a small nidus and grows by concentric deposition of inorganic crystals in an organic matrix.

Calculi are much less common in the parotid gland than in the submandibular gland, possibly because the secretions are more serous than the mucoid saliva of the submandibular gland. Calculi do not cause symptoms until they become large enough to impede the flow of saliva. Partial obstruction causes the gland to inflate itself with marked stimulation to secrete saliva, as occurs in eating. The gland swells while eating and soon becomes painful. The swelling and pain subside in 30-60 minutes only to recur at the next meal. Total obstruction causes pain, swelling, and infection. Complications of the calculi include infection within the gland ( sialadenitis ), scarring with stenosis, fistula formation, and rarely the stone migrates outside the duct to appear as an inflammatory mass in the neck The diagnosis is confirmed by imaging studies including plain radiographs with or without injection of contrast media into the duct. CT scans also show single or multiple stones.

(A retrospective study by Jáurequi et al suggested that it is uncommon to find multiple parotid calcifications in chronic parotitis, with the investigators reporting more than one calcification in the parotid gland region in just 13 out of 133 patients (10%) following parotid sialendoscopy for chronic sialadenitis. [ 16 ] ) Treatment is removal of the stone. Massage of the gland from posterior to anterior may occasionally remove stones, but most require surgical removal. Lidocaine injected around the duct orifice and into the duct allows serial dilatation with graduated dilators. The duct is filleted with sharp scissors and then massage may deliver the stone. This is the simplest method of removal in most instances because the required instruments are available in the ENT or dental office. Extracorporeal shock wave lithotripsy is an alternate treatment to fragment the stone. Interventional sialoendoscopy is growing in popularity and availability and seems to be the best method of treatment. The duct is anesthetized and dilated to insert a telescope for inspection of the large ducts.

A working channel in the telescope permits irrigation, suction, and insertion of forceps, wire loop, or even laser energy via a glass fiber to remove the calculi. The clinician has much more information as to the condition of the duct system. This instrument is useful for the assessment and treatment of several inflammatory disorders of the gland. Chronic recurrent parotitis (chronic nonspecific parotitis) This general term is used for patients in whom no definite etiology is found. Hundreds of papers on chronic parotitis have discussed the nature and treatment of the disease or diseases. The theories of etiology are diverse. Many authors are convinced that sialoliths or scarring of the ducts cause stasis of salivary flow and predispose the gland to infection is the etiology, but this is probably true for only a minority of cases. All authors agree that the spectrum of symptoms varies from mild to incapacitating. Episodes may last for several days, paralleling the time course of a bacterial or viral illness. Others may experience episodes that last only a few hours from onset to resolution. Some episodes may last for several weeks. Quiescent periods between episodes last for hours, days, or even years.

This range suggests that more than one disease may be the cause. The cases in which the painful episodes last for hours are probably caused by mediator release rather than infection. Sialography generally shows marked dilatation (ectasia) of the major ducts, with narrowed areas that give the appearance of a string of sausage. Most authors believe that the narrowed areas represent strictures of the ducts. The minor ducts are frequently not patent and punctuate sialectasis is not seen. This suggests that the disease arises in the ductal system. A number of articles from Great Britain and Japan report a condition called "sialodochitis," which would be included in the chronic recurrent parotitis group. This word is not in most American medical dictionaries but implies inflammation of the ducts. The acini may be histologically normal, at least in the early stages of the disease. Most authors suggest that the treatment of all forms of chronic parotitis should be proportional to the symptoms, which are subjective rather than objective. Treatment escalates with the symptoms, from massage, sialogogues to antibiotics, and analgesics Pain seems to be the driving symptom.

Periodic irrigation of the Stensen duct with saline, antibiotics, and/or steroids has been advocated with good rationale in those patients with sialectasia of the duct. This procedure removes debris from the duct and deposits the drugs to the needed location. Frequent irrigation is probably important, but the patient cannot perform the treatment. If periodic irrigation is successful, it should be performed rather than removal of the gland. Because the pathophysiology is poorly understood, the rationale of several surgical treatments is rather weak. Parotidectomy removes the diseased gland but begs the question of specific treatment. Recurrent parotitis of childhood Another uncommon syndrome that has been recognized for the past 50 years is recurrent parotitis of childhood, in which recurring episodes clinically resemble mumps. Generally, episodes begin by age 5 years, and virtually all patients become asymptomatic by age 10-15 years. The duration of attacks averages 3-7 days but may last 2-3 weeks in some individuals. The spectrum varies from mild and infrequent attacks to episodes so frequent that they prevent regular school attendance. The child, although not ill, is regularly sent home with the diagnosis of mumps until school officials are informed of the nature of the disease.

During the attacks, the parotid gland is enlarged, moderately red, and tender. Massaging the gland from back to front produces clear saliva with lots of "snowflakes" or little white curds from the Stensen duct. The disease is unilateral most commonly and, if bilateral, is most apt to be asymmetric. The child generally is not very sick during the episodes. Bacterial cultures from saliva generally produce Streptococcus viridans or another low-virulence bacterium that is considered normal oral flora. Even between attacks, bacteria are present in the saliva. Ultrasonography and sialography reveal punctate sialectasis as in Sjögren syndrome. Even when symptoms are unilateral, sialectasis is demonstrated by sialography in the opposite gland in most instances. Sialographic changes persist even after all other symptoms have ceased. Findings may eventually disappear, but the natural history of gland findings is not clear. The histopathology is essentially the same as Sjögren and Mikulicz disease. Some children with recurrent parotitis may actually have Sjögren syndrome and may develop the full-blown clinical picture. The cause of the disease is unknown.

Sialectases may precede infections and may be a site of lowered resistance. A number of etiologies have been suggested. The disease is unrelated to mumps, and when viral studies have been performed, elevated serum titers to numerous other viruses have been found. One theory is that infection of the glands at a young age affects the immune system, and the disease may represent immaturity of the immunologic response. Searches for autoantibodies have not been successful. The benign self-limiting nature of this entity makes autoimmunity doubtful. No evidence suggests that allergy is a cause. Resolution of symptoms with age may be due to regeneration of glandular elements and return to normal function. A study by Xie et al indicated that amylase activity is important in the assessment of juvenile recurrent parotitis. The study, of 44 patients with the disease, found that the degree of parotid gland function was significantly related to serum amylase activity, with the severity of the parotitis associated with the extent of glandular function degradation. [ 17 ] Applying local heat applied to the gland, massaging the gland from back to front, and taking penicillin usually cure individual episodes.

Treatment of individual infections may prevent injury to the gland parenchyma. Severe disease may be treated by parotidectomy. Parotidectomy is rarely indicated. Sialadenosis (sialosis) In this disorder, both parotid glands may be diffusely enlarged with only modest symptoms. Patients are aged 20-60 years at onset, and the sexes are equally involved. The glands are soft and nontender. Biopsy shows the acinar cells to be enlarged to almost twice the normal diameter and the cytoplasm packed with enzyme granules. The cause is unknown, but inappropriate autonomic nervous system stimuli are frequently suggested. Approximately half of the patients have endocrine disorders such as diabetes, nutritional disorders such as pellagra or kwashiorkor, or have taken drugs such as guanethidine, thioridazine, or isoprenaline. If the symptoms are mild, treatment is not required. If the glands are disfiguring, partial parotidectomy should improve the appearance.

Sarcoidosis Sarcoidosis is a chronic multisystem disorder of unknown cause that is characterized by accumulations of T lymphocytes and mononuclear phagocytes, noncaseating epithelioid granulomas, and the derangement of normal tissue architecture. Skin anergy and depressed cellular immune processes in the blood are common. In the United States, the incidence is much higher in African Americans and generally begins in people aged 20-40 years. Diagnosis requires the typical clinical picture and biopsy reveals noncaseating granuloma, plus exclusion of other diseases associated with such granulomas. In most instances, the process does not attack involved organs, but the bulk of the accumulated cells may distort the normal architecture enough to impair function. The lungs, skin, and lymph nodes are most often affected, but the salivary glands are involved in approximately 10% of cases. Bilateral firm, smooth, and nontender parotid enlargement is classic. Xerostomia occasionally occurs. The Heerfordt-Waldenstrom syndrome consists of sarcoidosis with parotid enlargement, fever, anterior uveitis, and facial nerve palsy.

Sarcoidosis is benign in most instances, and treatment is generally not advisable unless organ dysfunction occurs. The only effective treatment is corticosteroids administered for several weeks. Treatment of the parotid glands is not necessary, but most would treat facial paralysis because of the fear of permanent function loss. Pneumoparotitis Pneumoparotitis is air within the ducts of the parotid gland with or without inflammation. The duct orifice normally functions as a valve to prevent air from entering the gland from a pressurized oral cavity. Rarely, an incompetent valve allows insufflation of air into the duct system. Pneumoparotitis most commonly occurs in wind instrument players, glass blowers, and scuba divers. Occasionally, a person learns how to perform parotid insufflation voluntarily. The condition is harmless unless bacteria from the mouth cause parotitis. Rarely, rupture of the ductal system causes extensive subcutaneous emphysema. Miscellaneous causes of inflammation and enlargement of the parotid Several lymph nodes reside within the parotid gland as a superficial and deep group of nodes.

These nodes may be involved with any process that affects lymph nodes, including bacterial, fungal, viral, and neoplastic processes. Rarely, drugs such as iodides, phenylbutazone, thiouracil, isoproterenol, heavy metals, sulfisoxazole, and phenothiazines cause parotid swelling. The parotid glands may be incidentally involved in numerous systemic conditions such as uremia and kwashiorkor. Diseases such as Wegener granuloma or Kimura disease involve the parotid glands as rare causes of parotitis. Next: The pathophysiology varies with the type of parotitis and is discussed under Background. Previous Mumps: Background, Etiology, Epidemiology Epidemiology Overview Background Mumps is an acute, self-limited, systemic viral illness characterized by the swelling of one or more of the salivary glands, typically the parotid glands. The illness is caused by the RNA virus, Rubulavirus. Rubulavirus is within the genus Paramyxovirus and is a member of the family Paramyxoviridae. This virus contains a single-stranded, negative-sense RNA surrounded by a glycoprotein envelope.

Of 2 glycoproteins on the surface of the RNA viral envelope, one mediates neuraminidase and hemagglutination activity, whereas the other is responsible for fusion to the lipid membrane of the host cell. Rubulavirus can be isolated in viral culture from saliva, urine, and cerebrospinal fluid (CSF). Chemical agents (ie, ether, formalin, chloroform), heat and ultraviolet light can inactivate this virus. Serologic assays determine the presence of an antibody response and assess differences between acute and convalescent titers. Affected salivary glands show edema and lymphocytic infiltration. (See the image below.) (See Workup.) Child with mumps. View Media Gallery Even though the mumps virus, Rubulavirus, shares similar morphologic features to human parainfluenza viruses (known as hPIVs, as part of the Paramyxovirus genus), no cross-immunity between these viruses is known.

The mumps virus does share various epidemiologic characteristics with other well-known viral pediatric diseases, such as measles (RNA virus, of the genus Morbillivirus, in the Paramyxoviridae family) and rubella (RNA virus, of the genus Rubivirus, in the Togaviridae family). Mumps occurs worldwide. Humans are the only known natural hosts. This Paramyxovirus is highly infectious to nonimmune individuals and is the only cause of epidemic parotitis. (See Epidemiology.) Although mumps cases occur at any time of year, an increase in case number is noted during late winter and early spring. [ 1 ] (Also see Management of Acute Presentation of Mumps . Mumps and SARS During the 2003 epidemic of severe acute respiratory syndrome (SARS), it was thought that the SARS-causing virus belonged to the Paramyxoviridae family. However, current case criteria have determined that SARS follows the clinical, laboratory, and transmission characteristics of an RNA coronavirus named SARS-associated coronavirus (SARS-CoV).

[ 2 , 3 ] Vaccination history and timeline The monovalent live, attenuated mumps vaccine was licensed in the United States in December 1967, and the Advisory Committee on Immunization Practices (ACIP) recommended that its use be considered for children nearing puberty, for adolescents, and for adults. At this time, the public health community considered mumps vaccination a low priority; as such, the ACIP believed that mumps immunization should not compromise the effectiveness of established immunization public health programs. By 1972, the ACIP recommendations were changed to indicate that mumps vaccination was particularly important for the initially targeted teenage and adult age groups. Hence, in 1977, routine vaccination was recommended for all children age 12 years and older. (See Treatment) In 1980, further recommendations called for mumps vaccination of susceptible children, adolescents, and adults, unless it was medically contraindicated. Following these comprehensive recommendations and enactment of state laws requiring mumps vaccination for school entry and attendance, the reported incidence of mumps steadily declined.

However, in 1986 and 1987, large outbreaks of mumps occurred among under-immunized cohorts of people born between 1967 and 1977, as based on a single-dose mumps vaccine regimen. This caused a shift in peak incidence from persons age 5-9 years to those age 10-19 years. [ 4 ] In 1989, the ACIP recommended that a second dose of mumps-containing vaccine be given to children age 4-6 years (at time of entry to kindergarten or first grade) and designated MMR as the vaccine of choice. [ 4 , 5 ] Over the next decade (1988-1998), the incidence of mumps decreased among all age groups. The greatest decrease occurred among persons aged 10-19 years. This age group had reversed the trend seen in 1986 and 1987, when a resurgence of outbreaks had occurred among susceptible adolescents and young adults. Subsequent outbreaks have occurred among well-vaccinated populations.

During 1989-1990, a large outbreak occurred among students in a primary and a secondary school. Most students in these schools had been vaccinated, suggesting that a vaccination failure (in addition to failure to vaccinate) might have contributed to the outbreak. Another mumps outbreak occurred a year later, in a secondary school where most of the students had been vaccinated; this outbreak was mostly attributed to primary vaccination failure. The shift in higher risk for mumps to these other age groups (ie, from younger school-aged children to older children, adolescents, and young adults), which occurred after the routine use of the mumps vaccine was initiated, has persisted despite minimal fluctuations in disease incidence that have occurred among the various age groups. Transmission, course, and symptoms Humans are the sole reservoir for the mumps virus. The transmission mode is person to person via respiratory droplets and saliva, direct contact, or fomites. (See Etiology.) The presence of maternal antibodies typically protects infants younger than 12 months old from the disease. Infections can be a symptomatic in 20-30% of persons. Of those with symptomatic infection, adults tend to be more severely affected when compared to children.

Lifelong immunity usually follows clinical or subclinical mumps infection, although second infections have been documented. Mumps has an incubation period of 16-18 days; however, cases can arise 12-25 days after exposure. After this period, prodromal symptoms (such as low-grade fever, malaise, myalgias, headache, and anorexia) occur; these symptoms can last 3-5 days. After this prodromal period (about 48 h), the clinical path of the virus depends on which organ is affected. The most common presentation is a parotitis, which occurs in 30 to 40% of all patients and in 95% of those who are symptomatic. Parotitis is caused by direct viral infection of the ductal epithelium and presents with localized gland inflammation. Other reported sites of infection are the central nervous system (CNS), eyes, pancreas, kidneys, testes, ovaries, and joints. Mumps is the only known cause of epidemic parotitis; as such, parotitis is used as a clinical parameter of maximum communicability of the mump virus.

A patient is considered infectious from about 3 days before the onset of, and up to 4 days after, the start of active parotitis (although it has been suggested that the communicable period is actually longer, lasting from 6 days before, to 9 days after, facial swelling is apparent). After the prodromal period, one or both parotid glands begin to enlarge. Initially, local parotid tenderness and same-sided earache can occur. Ordinarily, the parotid glands are not palpable; but in patients with mumps, parotid swelling increases rapidly over several days. Seventy to 80% of symptomatic cases are bilateral with unilateral swelling occurring first, followed by bilateral parotid involvement. Occasionally, simultaneous involvement of both glands occurs. Edema over the parotid gland presents with non-discrete borders, pain with pressure, and obscures the angle of the mandible. The opening of the parotid duct (also known as Stensen's duct) appears erythematous and enlarged. Parotid swelling can last for 10 days.

Serologically, this inflammatory process can be confirmed with an elevated salivary amylase (s-amylase) level. Despite the classic correlation of mumps and parotitis, mumps is no longer the most common cause of parotid swelling. Other viral causes include cytomegalovirus, parainfluenza virus 1 and 3, influenza A, and HIV. Bacterial infections, drug reactions, and metabolic disorders can also cause parotitis. Refer to the Medscape article on Parotitis for further information. Other sites of viral infection Central nervous system CNS involvement is the most common extrasalivary complication of mumps. Its presentation is most often as aseptic meningitis rather than as a true encephalitis . This complication occurs up to 3 times more often in males when compared with females. [ 6 ] It may precede parotitis or occur in its absence, but it usually presents within the first week after parotid swelling. Aseptic meningitis has been seen in up to 10% of patients with history of parotitis. This percentage increases to 50% in those patients without parotid gland swelling.

Salient clinical indicators of CNS involvement include headache, fever, nausea, vomiting, and nuchal rigidity. Marked changes in sensorium, convulsions, paresis, and/or paralysis present in patients with encephalitis, not typically in aseptic meningitis. In clinically diagnosed aseptic meningitis or encephalitis, the CSF profile is similar. A CSF mononuclear pleocytosis occurs, as does normal glucose; although hypoglycorrhachia has been reported. Pleocytosis has a wide range of WBCs per microliter (10-2000 WBCs/µL), with a lymphocytic predominance, although an early polymorphonuclear predominance has been reported. CSF total protein is usually normal or can be mildly elevated. [ 7 ] In addition, the mumps virus can be isolated from CSF early in the illness. Pleocytosis in the CSF has also been shown to occur in persons without clinical signs of CNS involvement. CNS involvement (meningitis or encephalitis) carries a good prognosis and is usually associated with a complete recovery.

Neuritis of the auditory nerve may cause sensorineural deafness . [ 8 ] . A sudden onset of tinnitus, ataxia, and vomiting precedes a permanent hearing loss. Other neurologic complications include facial nerve neuritis and myelitis. Gastrointestinal Another clinical manifestation of mumps is acute pancreatitis . Pancreatitis presents with mid-abdominal pain and distention, fever (typically low grade), nausea, and vomiting. An elevated serum lipase value supports this diagnosis. Genitourinary Approximately one third of post-pubertal male patients develop unilateral orchitis. Orchitis is the most common complication of mumps infection in adult males. This inflammation usually follows parotitis but may precede or occur in the absence of parotid gland swelling. Orchitis usually appears during the first week of parotitis, but it can occur in the second or third week. Bilateral orchitis occurs less frequently (about 10% of cases). Gonadal atrophy may follow orchitis and poses a greater risk with bilateral involvement; however, sterility is rare. [ 9 ] Prepubertal boys may develop orchitis, but it is uncommon in boys younger than 10 years old.

Orchitis presents with high fevers (39-41°C), severe testicular pain accompanied by swelling, and erythema of the scrotum. Nausea, vomiting, and abdominal pain are often present. Fever and gonadal swelling usually resolve in 1 week, but tenderness may persist. Retrospective case studies have investigated a possible link between mumps orchitis and the subsequent development of testicular cancer; no increased risk has been shown to date. [ 10 , 11 ] . Oophoritis occurs in about 7% of postpubertal female patients. A more complete list of complications appears below. (See also Prognosis.) Complications A list of potential complications from mumps infection is cited below: Aseptic meningitis/encephalitis Males and females are affected equally with parotitis. CNS involvement has shown a male-to-female ratio of 3:1. Age predilection Today, most reported mumps cases occur in school-aged children (age 5-14 y). This was also the case in the prevaccine era.

An average of 75% of mumps cases were reported from 1967-1971, whereas 60% of mumps cases occurred in this same age population from 1985-1987, within the first 5-year period post licensure. For 2 consecutive years, 1986 and 1987, the reported peak incidence rate shifted from children, 5-9 years old, to older age groups. Persons aged 15 years or older accounted for more than 33% of the reported total from 1985-1987. During the earlier years, 1967-1971, an average of only 8% of reported mumps cases occurred among this population group. From 1985-1987, the incidence of reported mumps increased in all age groups. The most dramatic increases were noted among adolescents aged 10-14 years (almost a 7-fold increase) and young adults aged 15-19 years (more than an 8-fold increase).

Increased occurrence of mumps in susceptible adolescents and young adults has been documented by several outbreaks in high schools, on college campuses, and in occupational settings. [ 16 ] Nonetheless, despite this age shift in reported mumps, the overall reported risk of disease in persons aged 10-14 years and those aged 15 years or older is still lower risk than that seen in the prevaccine and early postvaccine eras. Previous Mumps | For Healthcare Providers | CDC For Healthcare Providers From the Merck Manual Consumer Version, edited by Robert Porter. Copyright 2015 by Merck Sharp & Dohme Corp., a subsidiary of Merck & Co, Inc, Kenilworth, NJ. Available at merckmanuals.com . Accessed June 2015. The Virus Mumps is a viral illness caused by a paramyxovirus, a member of the Rubulavirus family. The average incubation period for mumps is 16 to 18 days, with a range of 12 to 25 days. Clinical Features Mumps usually involves pain, tenderness, and swelling in one or both parotid salivary glands (cheek and jaw area).

Swelling is first visible in front of the lower part of the ear. It then extends downward and forward as fluid builds up in the skin and soft tissue of the face and neck. Swelling usually peaks in 1 to 3 days and then subsides during the next week. The swollen tissue pushes the angle of the ear up and out. As swelling worsens, the angle of the jawbone below the ear is no longer visible. Often, the jawbone cannot be felt because of swelling of the parotid. One parotid may swell before the other, and in 25% of patients, only one side swells. Other salivary glands (submandibular and sublingual) under the floor of the mouth also may swell but do so less frequently (10%). Nonspecific prodromal symptoms may precede parotitis by several days, including low-grade fever which may last three to four days, myalgia, anorexia, malaise, and headache. Fever may persist for 3 to 4 days. Parotitis, lasts at least 2 days, but may persist longer than 10 days.

However, mumps infection may present only with nonspecific or primarily respiratory symptoms, or may be asymptomatic. Mumps infection is most often confused with swelling of the lymph nodes of the neck. Lymph node swelling can be differentiated by the well-defined borders of the lymph nodes, their location behind the angle of the jawbone, and lack of the ear protrusion or obscuring of the angle of the jaw, which are characteristics of mumps. People with mumps are usually considered most infectious for several days before and after onset of parotitis. Parotitis and Flu While not a common symptom of flu, swelling of their salivary glands (parotitis) has been reported in persons with laboratory-confirmed influenza infections. To learn more, see 2016-2017 Influenza Update for Health Care Providers: Parotitis and Influenza . Top of Page Background Before there was a vaccine against mumps, the disease was common in the United States and caused complications, such as permanent deafness in children, and occasionally, encephalitis, which could result in death, although very rarely. Before the U.S.

mumps vaccination program started in 1967, about 186,000 cases were reported each year, and many more unreported cases occurred. Since the pre-vaccine era, there has been a more than 99% decrease in mumps cases in the United States. From year to year, the number of mumps cases can range from roughly a couple hundred to a couple thousand. However, outbreaks still occasionally occur. In 2006, there was an outbreak affecting more than 6,584 people in the United States, with many cases occurring on college campuses. In 2009, an outbreak started in close-knit religious communities and schools in the Northeast, resulting in more than 3,000 cases. These outbreaks have shown that when people who are sick with mumps have close contact with a lot of other people (such as among students living in dormitories and students and families in close-knit communities) mumps can spread even among vaccinated people. However, outbreaks are much larger in areas where vaccine coverage rates are lower. Transmission The mumps virus replicates in the upper respiratory tract and spreads through direct contact with respiratory secretions or saliva or through fomites.

The risk of spreading the virus increases the longer and the closer the contact a person has with someone who has mumps. When a person is ill with mumps, he or she should avoid contact with others from the time of diagnosis until at least 5 days after the onset of parotitis by staying home from work or school and staying in a separate room if possible. Top of Page Complications Some complications of mumps are known to occur more frequently among adults than children. Death from mumps is exceedingly rare. In recent U.S. mumps outbreaks (2006, 2009 to 2010), orchitis occurred in 3.3 to 10% of adolescent and adult males. In 60% to 83% of males with orchitis caused by mumps, only one testis is affected. Such orchitis, even bilaterally, very rarely causes sterility. Among adolescent and adult females in recent outbreaks, mastitis rates have been ≤1% and oophoritis rates have been ≤1%.

Other rare complications of mumps include pancreatitis, deafness, meningitis, and encephalitis, which have occurred in less than 1% of cases in recent U.S. outbreaks. There have been no mumps related deaths reported in the United States during recent mumps outbreaks. Mumps during Pregnancy Like other infections, there is a theoretical risk that mumps during the early months of pregnancy may cause complications. One study from 1966 that was conducted before the introduction of MMR vaccine reported an association between mumps infection during the first trimester of pregnancy and an increase in the rate of spontaneous abortion or intrauterine fetal death 1 , but was not found in a more recent study 2 . Another study with methodological flaws showed that congenital malformations may occur from mumps during pregnancy, but because the author did not compare rates with infants born to women not affected with mumps, these findings must be interpreted with caution 3 ; other papers have not reported similar findings 4 . Learn more about preventing infections during pregnancy . Vaccination Vaccination is the best way to prevent mumps.

This vaccine is included in the combination measles-mumps-rubella (MMR) and measles-mumps-rubella-varicella (MMRV) vaccines. Two doses of mumps vaccine are 88% (range 66% to 95%) effective at preventing the disease; one dose is 78% range (49% to 91%) effective. The first vaccine against mumps was licensed in the United States in 1967, and by 2005, high two-dose childhood vaccination coverage reduced disease rates by 99%. The MMR vaccine protects against currently circulating mumps strains. See Mumps Vaccination for vaccination recommendations for different groups. Top of Page Case Classification For information about how to classify mumps cases, visit the National Notifiable Diseases Surveillance System (NNDSS) page for mumps or the Laboratory Testing Section of the Manual for the Surveillance of Vaccine-Preventable Diseases (5th Edition, 2012), Chapter 9: Mumps . Reporting Mumps Cases Each state and territory has regulations or laws governing the reporting of diseases and conditions of public health importance.

These regulations and laws list the diseases that are to be reported and describe those people or groups responsible for reporting, such as healthcare providers, hospitals, schools, laboratories, daycare and childcare facilities, and other institutions. For information about reporting mumps cases to CDC, see the Reporting Section of the Manual for the Surveillance of Vaccine-Preventable Diseases (5th Edition, 2012), Chapter 9: Mumps Mumps in Vaccinated People During mumps outbreaks in highly vaccinated communities, the proportion of cases that occur among people who have been vaccinated may be high (see example below). This should not be interpreted as meaning that the vaccine is not effective. The effectiveness of the vaccine is assessed by comparing the attack rate in people who are vaccinated with the attack rate in those who have not been vaccinated. In outbreaks in highly vaccinated populations, people who have not been vaccinated against mumps usually have a much greater mumps attack rate than those who have been fully vaccinated.

Vaccination and Mumps Outbreak: an Example Example: Let’s say that an outbreak occurs among 1,000 people and that 950 of these 1,000 people have received two doses of the vaccine and 50 are unvaccinated (i.e., vaccine coverage is 95%). If there is a 30% attack rate among people who haven’t been vaccinated, 15 unvaccinated people would get the disease. Among the 950 vaccinated people, the attack rate would be 3%, so 29 vaccinated people would get the disease. Therefore, of the 44 people who got sick during the outbreak, the majority (29, or 66%) would have been vaccinated. This doesn’t imply that the vaccine didn’t work. In fact, the people who hadn’t been vaccinated were 10 times more likely to get sick as those who had been vaccinated, it’s just that there were a lot fewer unvaccinated people at risk. Furthermore, if none of the 1,000 people had been vaccinated, the outbreak would have resulted in 300 cases rather than only 44.

In this scenario, we would say that the vaccine is 90% effective in preventing the disease after two doses, which is the same as saying that the attack rate in the unvaccinated group is 10 times higher than the attack rate among people who have received two doses of vaccine. The formula to calculate vaccine effectiveness is (attack rate in unvaccinated group minus attack rate in vaccinated group) divided by attack rate in unvaccinated group, or (ARU-ARV)/ARU.

Question: Usually causing face pain and most common in children aged 2 - 12, whic disease has the alternative name 'Epidemic Parotitus' ?
Answer: MUMPS