Age: 58.0
Sex: F
Vaccine: VARZOS
Symptoms: Angiotensin converting enzyme, Antineutrophil cytoplasmic antibody negative, Antinuclear antibody negative, Blood immunoglobulin G normal, C-reactive protein increased
Clinical narrative: Unilateral ptosis/ left-sided upper eyelid drooping; Proptosis; Orbital myositis; Fever; Chills; Diaphoresis; left-sided headache; prickly sensation in both hands; acute onset of worsening left periocular swelling; diplopia\Double vision; left-sided tearing; Sinusitis/stable left sinus disease/left ethmoid, maxillary, and frontal sinus thickening; left-sided eye pain; Extraocular motility decreased; had edema and erythema of the left upper and lower eyelids; had edema and erythema of the left upper and lower eyelids; left temporal bulbar conjunctiva was injected; left temporal bulbar conjunctiva was injected and chemotic; rectus muscles appeared enlarged; discomfort; This case was reported in a literature article and described the occurrence of ptosis in a 58-year-old female patient who received Herpes zoster (Shingrix) for prophylaxis.   The patient's past medical history included migraine headache, craniotomy (for left parietal (WHO grade 1) meningothelial syncytial meningioma resection 4 months prior to presentation) and chickenpox (as a child but no history of known shingles). Previously administered products included Penicillin with an associated reaction of pruritus and lidocaine with an associated reaction of tachycardia (intolerance: dizziness). Concurrent medical conditions included hypertension, hyperlipidemia, meningioma and penicillin allergy.   On an unknown date, the patient received Shingrix. On an unknown date, 2 days after receiving Shingrix and an unknown time after starting Oxymetazoline, the patient experienced ptosis (serious criteria hospitalization), proptosis (serious criteria hospitalization and GSK medically significant), orbital myositis (serious criteria hospitalization and GSK medically significant), fever (serious criteria hospitalization), chills (serious criteria hospitalization), diaphoresis (serious criteria hospitalization), headache unilateral (serious criteria hospitalization), tingling of extremity (serious criteria hospitalization), periocular swelling (serious criteria hospitalization), diplopia (serious criteria hospitalization), eyes tearing (serious criteria hospitalization), sinusitis (serious criteria hospitalization), eye pain, eye movement disorder, eyelid edema, erythema eyelid, conjunctival injection, chemosis, muscle swelling and discomfort. The patient was treated with fluticasone propionate (Fluticasone), ciprofloxacin, oxymetazoline hydrochloride (Oxymetazoline), doxycycline, vancomycin, sodium chloride (Nasal Saline Irrigation), methylprednisolone sodium succinate (Solumedrol), prednisone and antibiotics nos (Oral Antibiotics (Nos)). On an unknown date, the outcome of the ptosis, proptosis, orbital myositis, fever, periocular swelling, diplopia, eyes tearing, eye pain, eye movement disorder, eyelid edema, erythema eyelid, conjunctival injection, chemosis, muscle swelling and discomfort were recovered/resolved and the outcome of the chills, diaphoresis, headache unilateral, tingling of extremity and sinusitis were unknown.   The reporter considered the ptosis, proptosis, orbital myositis, fever, chills, diaphoresis, headache unilateral, tingling of extremity, periocular swelling, diplopia, eyes tearing, sinusitis, eye pain, eye movement disorder, eyelid edema, erythema eyelid, conjunctival injection, chemosis, muscle swelling and discomfort to be possibly related to Shingrix.  Additional details were provided as follows: This case was reported in a literature article and described the occurrence of unilateral ptosis, proptosis, and orbital myositis in a 58-years old female patient, who was vaccinated with Shingrix (GlaxoSmithKline) for prophylaxis. The patient's medical history included hypertension, hyperlipidemia, left-posterior parietal craniotomy for left parietal (WHO grade 1) meningothelial syncytial meningioma resection 4 months prior to presentation and migraine headache. The patient had a history of chicken pox as a child but no history of known shingles. The patient's allergy list included penicillin (itching) and lidocaine (intolerance: tachycardia and dizziness). Family history included a father with a history of granulomatous with polyangiitis. The patient had no history of an autoimmune condition. No information on patient's concurrent condition or concomitant medication was provided. On an unspecified date, 3 days prior to presentation, the patient received Shingrix (administration route unspecified, dosage unknown; batch number not provided) in the left deltoid. [She had received the Shingrix vaccine (GlaxoSmithKline) 3 days prior to presentation in the left deltoid]. On an unspecified date, within hours after the vaccination, the patient developed fevers, chills, diaphoresis, left-sided headache, and prickly sensation in both hands. The patient presented with acute onset of worsening left periocular swelling and diplopia for 2 days. The patient later noted left-sided upper eyelid drooping and left-sided tearing 1 day prior to presentation and presented to an outside emergency department 2 days after the vaccination. Her initial emergency room record, prior to ophthalmology evaluation, showed no evidence of ptosis or tearing in either eye, and CT head scan on presentation found stable left sinus disease. The patient was discharged with a diagnosis of sinusitis and prescribed oral doxycycline 100 mg twice daily for 10 days and fluticasone nasal spray (50 mcg/actuation) 1 spray nightly for 28 days for sinusitis. The next day the patient presented to institution's emergency department with left-sided eye pain and swelling and new-onset diplopia. Ophthalmology was then consulted, at her second ED visit, but first evaluation with ophthalmology. The patient denied discharge, irritation, foreign body sensation, floaters, flashes, shade over vision, blurred vision, sudden vision loss, photophobia. Ophthalmological examination revealed visual acuity of 20/20 in each eye, normal pupillary exam, and intraocular pressures of 16 and 21 mm Hg in the OD and OS, respectively. Visual field examination revealed limited ability to count fingers in the superior field of the OS. Extraocular motility was reduced in the OS (-3 in upgaze and -1 in adduction). Hertel exophthalmometry at a base of 100 mm was 12 mm on the OD and 16 mm OS). Margin reflex distance was 2 mm in the OD and -1 mm on the OS. The left lacrimal gland region appeared without induration or tenderness. The patient had edema and erythema of the left upper and lower eyelids, and her left temporal bulbar conjunctiva was injected and chemotic [On presentation, the patient had erythema and edema of the left upper and lower eyelids, left ptosis, left elevation deficit, and left proptosis]. The rest of the ocular exam was otherwise unremarkable. The patient was afebrile on presentation with a blood cell count of 10.9 k/uL. Orbital CT revealed left preseptal thickening with enhancement, and a small focal thickening adjacent to the left lamina papyracea thought to represent subperiosteal phlegmonous change or early abscess, with confluent opacification of the left anterior ethmoid air cells. The left inferior and medial rectus muscles appeared enlarged with sparing on the tendons. [Coronal CT scan of the orbits on presentation, showing enlarged left medial and inferior rectus muscles. The patient was also noted to have left ethmoid, maxillary, and frontal sinus thickening]. The patient was admitted to initiate intravenous vancomycin (1.25 gm every 12 hours) and ciprofloxacin (400 mg every 12 hours), and oxymetazoline nasal sprays (2 sprays, twice daily), nasal saline irrigation (60 mL to each nostril, twice daily), and fluticasone (2 sprays in each nare, daily). Over the next 18 hours, the left-sided proptosis increased to 6 mm and remained stable over the following day, without any changes in vision, extraocular motility, or cranial nerve exam. Autoimmune workup showed a normal WBC, TSI, ANA, ACE, ANCA, and IgG4. Other serologic testing included ESR = 72 mm/hour (normal 0-20 mm/hour), C-reactive protein = 9.4 mg/dl (normal less than 0.9 mg/dl), and rheumatoid factor = 17 IU/ml (normal less than 16 IU/ml). Thirty-six hours after admission, intravenous solumedrol, 250 mg every 6 hours, was initiated. Nine hours after initiation of steroids, the patient noted drastic improvement in her discomfort and double vision. Extraocular motility improved to -0.5 in upgaze and -0.5 adduction and proptosis was reduced to 3 mm. Approximately 24 hours after initiating IV solumedrol, the proptosis had reduced to 2 mm, with stable extraocular motility (-0.5 in upgaze and -0.5 adduction). The patient was discharged for outpatient management prednisone taper starting at 60 mg orally per day and oral antibiotics. Follow-up examination 1 week after discharge revealed visual acuity of 20/20 in each eye, Hertel exophthalmometry of 12 mm in each eye at a base of 100 mm, full extraocular motility and resolved eyelid edema and erythema. The patient finished her course of oral steroids 15 days later without evidence of relapse. This case has been considered as serious due to hospitalization.  The author commented, "This case of unilateral ptosis, proptosis, and orbital myositis occurred within 2 days after administration of shingles vaccination. Shingrix has a high level of efficacy in reducing the incidence of zoster infection, with 97.2% efficacy in those more than 50 years of age and 89.8% in those more than 70. The most common side effects with Shingrix vaccine included local pain, redness, and swelling and generalized effects such as myalgia, fatigue, headache, shivering, fever, and GI symptoms. No publications were found of new ocular or orbital inflammation after administration of shingles vaccines. Herpes zoster ophthalmicus (HZO) presents in the first division of the trigeminal nerve. Typically, HZO is associated with pain and a periocular vesicular rash in the CN V1 distribution. HZO may also present with eyelid changes (rash, secondary bacterial infection), conjunctival injection and chemosis, corneal changes (pseudodendrites, punctate epithelial keratitis, stromal keratitis, nummular keratitis), uveitis (cell and flare, synechiae, iris atrophy), retinal findings (acute retinal necrosis, progressive outer retinal necrosis), and postherpetic neuralgia. HZO has less commonly been reported to present with orbital inflammation. Case reports include diffuse orbital inflammation and myositis; however, the patient reported here had no other signs or symptoms of HZO. Given the temporal relation with the shingles vaccination, no occurrence of vesicles or rash, and rapid resolution after steroid treatment, the authors propose that this patient experienced an autoimmune reaction to the shingles vaccine. There are alternative possibilities for the presentation. As discussed above, orbital myositis has been described with use of statin medications, which was a long-term medication for the patient. While a serum creatine kinase (CK) level was not obtained in this case, an elevated CK level may have strengthened the diagnosis of orbital myositis. Additionally, if orbital symptoms recurred on the second dose of Shingrix, this may further strength the association between Shingrix and orbital myositis; however, the authors did not obtain patient approval to evaluate this. The likelihood of ADR is separated in 4 categories: doubtful, possible, probably, and definite. Based on that scale, this reaction would be considered possible and the authors propose that this reaction to Shingrix is possible but not definitive. No clear conclusions have been made in the literature as to the mechanism of vaccine-related autoimmune conditions. Vaccine adjuvants may cause an orbital or systemic myositis presentation, and the adjuvants may inadvertently cause an autoimmune myositis. This case of orbital myositis occurring after Shingrix vaccine expands the known list of vaccines that may produce orbital inflammation; however, the exact pathophysiology of orbital inflammation in the context of vaccination remains to be elucidated."