bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pulmonary complications of HIV infection
Authors: Choy Julia , Pozniak Anton 
Published: February 2020

Abstract
Most HIV-positive individuals will experience at least one significant episode of pulmonary disease during their lifetime. The immune status of the HIV-infected patient is the primary determinant of the risk of developing specific pulmonary diseases: those with advanced immunosuppression are predisposed to opportunistic infections and malignancies; those with mild or no immunosuppression are at greater risk of conditions including community-acquired pneumonia, chronic obstructive pulmonary disease, pulmonary hypertension, and interstitial lung disease. Pulmonary infections related to HIV infection with severe immunosuppression include Pneumocystis jirovecii pneumonia, tuberculosis, and fungal infections including aspergillosis and cryptococcosis. Lung malignancies related to HIV infection with severe immunosuppression include Kaposi’s sarcoma and lymphoma, which is typically non-Hodgkin’s B-cell lymphoma or primary effusion lymphoma (also caused by HHV-8).
Sleep-related breathing disorders
Authors: Morrell Mary J. , Kelly Julia , McMillan Alison , Hind Matthew 
Published: February 2020

Abstract
Obstructive sleep apnoea and other sleep-related breathing problems significantly impair the functioning of about 0.5–1% of the population. Obstructive sleep apnoea in adults is usually caused by obesity and fat deposits in the neck area (typically collar size of 17 inches (43 cm) or more), when the withdrawal of postural muscle tone during sleep allows the pharyngeal dilators to be overwhelmed, leading to excessive narrowing or collapse of the airway, with consequent apnoea and sleep fragmentation. Mild symptoms may resolve with lifestyle changes such as losing weight; learning to sleep on the side and avoiding sleeping on the back; no alcohol after 18.00 h; no sedatives; stopping smoking; keeping the nose as clear as possible. There is only one fully effective therapy for moderate to severe obstructive sleep apnoea—continuous positive airway pressure.
Vascular mortality is higher than average, although recent data have questioned this link.
Chronic obstructive pulmonary disease
Authors: Hopkinson Nicholas S. 
Published: February 2020

Abstract
Chronic obstructive pulmonary disease (COPD) is a lung condition caused by the inhalation of noxious materials, principally tobacco smoke, and characterized by airflow limitation that is not fully reversible. Key features are cough, sputum production, and breathlessness. There are chronic progressive symptoms and acute exacerbations. The term COPD incorporates several pathological processes, present to a variable extent in any given individual, involving both the airways (chronic bronchitis) and the lung parenchyma (emphysema). Most COPD patients will have one or more other long-term conditions. COPD is the third leading cause of death worldwide. COPD should be considered in those over the age of 35 who have (1) exposure to risk factors, usually, but not exclusively, tobacco smoke; (2) a history of chronic progressive respiratory symptoms; (3) airflow limitation that is not fully reversible.
Cystic fibrosis
Authors: Bush Andrew , Elston Caroline 
Published: February 2020

Abstract
Cystic fibrosis (CF) is a recessively inherited disease caused by mutations in the cystic fibrosis gene, located on the long arm of chromosome 7, which codes for a membrane protein—the cystic fibrosis transmembrane regulator protein—that is a chloride channel. More than 2,000 CF mutations have been identified, with the ΔF508 mutation being the most common of around 200 mutations that definitely cause disease (70% of CF chromosomes in the European population). Birth incidence varies with country of origin from 1 in 2,000 to 1 in 100,000. The most popular hypothesis is that mutant cystic fibrosis transmembrane regulator protein fails to transport chloride ions normally, and there is secondary impairment of sodium, bicarbonate, and water transport.
Idiopathic pulmonary fibrosis
Authors: Molyneaux P.L. , Nicholson A.G. , Hirani N. , Wells A.U. 
Published: February 2020

Abstract
The synonymous terms idiopathic pulmonary fibrosis and cryptogenic fibrosing alveolitis refer to a relentlessly progressive fibrotic lung disorder. Incidence is about 5–15 per 100,000, men are more often affected than women, and it most commonly presents in the seventh and eighth decades. Aetiology remains uncertain. Typical presentation is with progressive exertional dyspnoea without wheeze, a non-productive cough, digital clubbing, and very fine end-inspiratory crackles. Central cyanosis and clinical evidence of pulmonary hypertension are late features. Diagnosis depends on careful exclusion of known causes of interstitial lung disease, followed by demonstration by radiological imaging or biopsy of the pathognomonic lesion of usual interstitial pneumonia. Two antifibrotic compounds, pirfenidone and nintedanib, have been proven to slow functional decline in idiopathic pulmonary fibrosis. Lung transplantation is appropriate in selected cases. Supportive therapy is central to the management of advanced disease. Five-year survival is 10–15%.
The lung in vasculitis
Authors: Margaritopoulos G.A. , Wells A.U. 
Published: February 2020

Abstract
Lung involvement in vasculitic disease can manifest as diffuse alveolar haemorrhage or as other pulmonary vasculopathy. Presenting features of diffuse alveolar haemorrhage include fever, weight loss, and other systemic symptoms in association with cough, breathlessness, and clinical signs suggestive of pneumonia. Haemoptysis may be present but is not invariable. A fall in haemoglobin over a day or longer suggests the diagnosis, and bronchoalveolar lavage is usually diagnostic. Other pulmonary vasculopathies present with breathlessness on exertion. Limited disease is generally treated with oral corticosteroid, given as monotherapy or in combination with a second-line immunosuppressive agent. Oral corticosteroid with either cyclophosphamide or rituximab are typically used to induce remission of generalized disease. Azathioprine or methotrexate with low-dose oral prednisolone are used to maintain remission.
Diffuse alveolar haemorrhage
Authors: Bourke S. J. , Spickett G.P. 
Published: February 2020

Abstract
Diffuse alveolar haemorrhage is characterized by acute respiratory failure, diffuse air space shadowing on the chest radiograph, haemoptysis, and anaemia. There are many different causes including immune-mediated diseases (notably pulmonary vasculitis, connective tissue diseases, and Goodpasture’s syndrome) and non-immune-mediated disease (cardiac failure, infection, coagulation disorders, thrombolytic therapy, toxins, and barotrauma). Prompt identification of the underlying cause is important in directing specific treatments. Goodpasture’s syndrome is an autoimmune disorder characterized by alveolar haemorrhage and glomerulonephritis due to antibasement membrane antibodies. Renal failure is usually the dominant feature, but alveolar haemorrhage can precede renal involvement. Idiopathic pulmonary haemosiderosis is a rare disorder of unknown cause with recurrent alveolar bleeding, which may provoke pulmonary fibrosis, and anaemia.
Eosinophilic pneumonia
Authors: Bourke S. J. , Spickett G.P. 
Published: February 2020

Abstract
Eosinophilic pneumonia is characterized by eosinophilic inflammation of the alveoli, usually with an accompanying eosinophilia of peripheral blood. The diagnosis should be considered when infiltrates on a chest radiograph are associated with blood eosinophilia, and is confirmed by demonstrating an excess of eosinophils in bronchoalveolar lavage fluid. Before concluding that the cause is ‘idiopathic’, the following must be considered: parasitic infestation with blood-borne parasites such as (in tropical eosinophilia) filarial worms; adverse drug reaction; asthma; allergic bronchopulmonary mycosis; vasculitis, notably eosinophilic granulomatosis with polyangiitis (previously known as Churg–Strauss syndrome); hypereosinophilic syndrome, a rare haematological disorder; and other disorders known to be associated with eosinophilic pneumonia. Causal factors need to be treated, but eosinophilic pneumonia otherwise often responds well to corticosteroid medication.
Hypersensitivity pneumonitis
Authors: Bourke S. J. , Spickett G.P. 
Published: February 2020

Abstract
Hypersensitivity pneumonitis is an immune-mediated lung disease in which the repeated inhalation of certain antigens provokes a hypersensitivity response, with granulomatous inflammation in the distal bronchioles and alveoli of susceptible people. A diverse range of antigens including bacteria (Thermophilic actinomycetes), fungi (Trichosporon cutaneum), animal proteins (bird antigens), mycobacteria, and chemicals may cause the disease. The commonest forms are bird fancier’s lung, farmer’s lung, humidifier lung, and metal-working fluid pneumonitis. In some cases no antigen is identified. Acute disease is characterized by recurrent episodes of breathlessness, cough, fevers, malaise, and flu-like symptoms occurring 4–8 hours after antigen exposure. Fever and basal crackles are the main physical signs.
Chronic disease is characterized by the insidious development of dyspnoea and persistent pneumonitis, sometimes progressing to lung fibrosis. Clinical features are similar to those of other varieties of pulmonary fibrosis, but clubbing is uncommon.
Lymphangioleiomyomatosis
Authors: Bourke S. J. 
Published: February 2020

Abstract
Lymphangioleiomyomatosis is characterized by cystic destruction of the lungs due to abnormal proliferation of smooth muscle cells. It is caused by mutations of the genes encoding hamartin and tuberin, sometimes in association with tuberous sclerosis. CT imaging shows characteristic multiple thin-walled cysts. There is usually progressive airways obstruction and impaired gas diffusion, and two-thirds of patients suffer pneumothoraces. Sirolimus can stabilize lung function and improve symptoms. Patients should avoid exogenous oestrogens, including oestrogen contraceptives or hormonal replacement therapy. Pregnancy may be associated with an increased risk of pneumothorax and loss of lung function. Hormonal therapy with progesterone or tamoxifen appears to be ineffective: other antioestrogen therapies, such as letrozole, are being studied. Lung transplantation is the main option for advanced disease.
Pulmonary amyloidosis
Authors: Bourke S. J. 
Published: February 2020

Abstract
Pulmonary amyloidosis is characterized by the deposition of monoclonal immunoglobulin light chain (AL) amyloid protein locally or diffusely in lung tissue. Local amyloid deposits in the airways, produced by B-cell clones within local tissues, may cause stridor, wheeze, cough, and haemoptysis. Diffuse alveolar deposition can occur as a complication of systemic amyloidosis. Local deposits of amyloid in the larynx, trachea, or bronchi may require treatment by endoscopic interventions, with mechanical debulking by forceps resection or laser therapy. Stenting may also be used to maintain airway patency. Radiotherapy has also been deployed successfully, and in certain circumstances may be a better option with less risk of bleeding or recurrence. Systemic AL amyloidosis may be treated by chemotherapy, while diffuse alveolar amyloidosis has a poor prognosis, particularly because it is associated with cardiac and renal amyloidosis.
Drug-induced lung disease
Authors: Bourke S. J. 
Published: February 2020

Abstract
Drug-induced lung disease is common and needs to be considered in the differential diagnosis of many respiratory conditions. The nature and timing of events often provide important clues and are sometimes sufficiently characteristic for drug-induced lung disease to be diagnosed with confidence, with resolution of symptoms on drug cessation providing further supportive evidence. Direct drug effects may arise through toxic, pharmacological, allergic, or idiosyncratic mechanisms (there may also be indirect effects). From a clinical perspective, adverse effects may be classified according to the induced disorder and the site of involvement. Asthma is the most common airway disorder to be induced or exacerbated by drugs. Cough is a well-recognized side effect of treatment with angiotensin-converting enzyme inhibitors. Pulmonary vascular involvement includes venous thromboembolism (e.g. oral contraceptive pill), and pulmonary hypertension (e.g. aminorex, now withdrawn), dasatinib, and interferons. Pleural effusions and thickening may result from drugs (e.g. dantrolene, bromocriptine, methysergide, and dasatinib).
Lung cancer
Authors: Spiro S.G. , Navani N. 
Published: February 2020

Abstract
Lung cancer remains the commonest killing cancer in both men and women in the developed world, and is increasingly common in developing countries, although as a result of decreased tobacco consumption in Western countries there has been a considerable reduction in the incidence among men over the last 20 years, and a slowing down in incidence in women over the last few years. Nevertheless, lung cancer is a greater cause of mortality in women than breast cancer in Western countries. There are several important industrial associations with lung cancer, in particular asbestos, but tobacco remains by far the most important cause.
There are four main cell types of lung cancer, of which adeno-, squamous, and large-cell varieties comprise non-small cell lung cancer, with the more aggressive type—small cell—being regarded as a separate entity from the point of view of treatment.
Joints and connective tissue—structure and function
Authors: Pap Thomas , Korb-Pap Adelheid , Hartmann Christine , Bertrand Jessica 
Published: February 2020

Abstract
Synovial joints are complex functional elements of the vertebrate body that provide animals with motion capabilities and hence the ability for locomotion and direct physical interaction with their environment. They are composed of different connective tissues structures that are derived from the same developmental structures in the embryo but have distinct cellular and biochemical properties. Articular cartilage and synovial membrane are key components of synovial joints and show several peculiarities that makes them different from other tissues. An in-depth knowledge of these features is important not only for understanding key features of articular function, but also providing explanations for important characteristics of both degenerative and inflammatory joint diseases. This chapter reviews the structure, biochemical composition, and function of articular cartilage and synovium, and points to important links between physiology and pathologic conditions, particularly arthritis.
Clinical investigation
Authors: Doherty Michael , Lanyon Peter C. 
Published: February 2020

Abstract
Disease markers are pathological or physiological characteristics of an individual that assist in determining the diagnosis, the current activity of disease, or the expected prognosis of the condition in that individual. Some markers relate to just one of these elements; others may relate to two, or occasionally all three. Clinical markers are derived from enquiry and examination of the patient. For many common rheumatic disorders, clinical assessment alone gives sufficient information for patient diagnosis and management. In some situations, however, particularly with inflammatory, metabolic, or multisystem disease, a search for additional investigational markers may be warranted. It is important to emphasize that the requirement for and selection of investigations, as well as their subsequent interpretation, is principally determined by the clinical assessment. Investigations are an adjunct, never a substitute, for competent clinical assessment. There is no place for a battery of screening tests.
Back pain and regional disorders
Authors: Ammendolia Carlo , Southerst Danielle 
Published: February 2020

Abstract
Over 70% of people in industrialized countries suffer from low back pain at some time, and it is one of the main reasons for visits to physicians. Risk factors include heavy physical work, smoking, stress, depression, and job dissatisfaction. In more than 90% of cases the exact anatomical source of back pain cannot be determined, and the preferred diagnostic label is ‘non-specific low back pain’. Investigation should be restricted to patients with red flags and clinical suspicion of serious disease, with magnetic resonance imaging the best imaging modality for the diagnosis of lumbar disorders. In the absence of red flags, patients with acute low back pain should receive non pharmacological care as first treatment option including reassurance, advise to remain active, massage and spinal manipulation followed by non steroidal anti-inflammatory drugs and muscle relaxants if necessary.
Rheumatoid arthritis
Authors: Baker Kenneth F. , Isaacs John D. 
Published: February 2020

Abstract
Rheumatoid arthritis is a common autoimmune disease characterized by both synovial and systemic inflammation. Synovitis classically presents as a symmetrical destructive polyarthritis affecting the hands and feet typified by episodic pain, stiffness, and swelling. Systemic inflammation leads to a range of extra-articular manifestations including organ involvement (e.g. interstitial lung disease, scleritis), constitutional features (e.g. fatigue, depression) and other complications (e.g. accelerated atherosclerosis, nerve and spinal cord compression). Rheumatoid arthritis is a clinical diagnosis based largely upon history and examination, supported by a limited range of investigation findings including elevated acute-phase reactants, autoantibodies (rheumatoid factor and anti-citrullinated peptide antibody), and imaging (e.g. musculoskeletal ultrasound). If left untreated, patients can rapidly develop irreversible joint damage leading to chronic pain, deformity, disability, and premature mortality. However, with early initiation of disease-modifying anti-rheumatic drugs (DMARDs) in treat-to-target strategies, disease remission is now achievable for many patients. Conventional synthetic DMARDs are the anchor of rheumatoid arthritis therapy, with methotrexate the recommended first choice. Biological DMARDs (monoclonal antibodies and soluble receptors) and targeted synthetic DMARDs (Janus kinase inhibitors) are reserved as second-line agents. Glucocorticoids are helpful as bridging therapy, though their considerable side effect profile prohibits their use as maintenance therapy. Non-steroidal anti-inflammatory drugs (NSAIDs) are effective in relieving arthritis pain, but long-term use is limited by their potential cardiovascular, renal, and gastrointestinal toxicities. With optimal care from a multidisciplinary team, many patients achieve and retain disease remission with maintenance of employment and quality of life.
Reactive arthritis
Authors: Stober Carmel B. , Gaston Hill 
Published: February 2020

Abstract
The term ‘reactive arthritis’ is sometimes used rather loosely to cover any form of arthritis that follows infection, and then includes post-viral arthritides, rheumatic fever, Lyme disease, and other forms of arthritis that do not generally share clinical features. Reactive arthritis a subset of post-infectious arthritis, and causative organisms are usually localized to the gastrointestinal or genitourinary tracts. Following infection, organisms or their components find their way to joints, where they provoke inflammatory immune responses. Whether the responses cross-react with self antigens is unclear; arthritis may be maintained by persistent infection. The disease commonly has specific extra-articular features not seen in other forms of post-infectious arthritis, and is genetically and pathologically a form of spondyloarthritis.
Inflammatory myopathies
Authors: Lundberg Ingrid E. , Chinoy Hector , Cooper Robert 
Published: February 2020

Abstract
The idiopathic inflammatory myopathies are a heterogenous group of disorders characterized by muscle weakness, inflammation in muscle tissue, and with frequent extramuscular involvement. Autoantibodies are common, supporting the notion of these disorders being autoimmune. Typically, inflammatory cell infiltrates are found in muscle biopsies. Other organs are frequently involved such as skin, lungs, joints, gastrointestinal tract, and the heart. These heterogenous disorders can be subclassified based on clinical and histopathological features, or by autoantibody specificities. The idiopathic inflammatory myopathies have traditionally comprised polymyositis (PM), dermatomyositis (DM), juvenile DM, PM/DM overlapping with another connective tissue disease, and inclusion body myositis. More recently a subgroup with similar clinical features but with no or scarce inflammation and with pronounced muscle fibre necrosis has been identified and termed immune-mediated necrotizing myopathy.
Large vessel vasculitis
Authors: Luqmani Raashid , Ponte Cristina 
Published: February 2020

Abstract
Large vessel vasculitis describes a group of primary vasculitides predominantly affecting the aorta and its major branches. The two major subtypes of large vessel vasculitis are giant cell arteritis (GCA) and Takayasu arteritis (TA), which have distinctive features. GCA occurs predominantly in white European women aged above 50 years, while TA affects women below 40 years from the Middle East and Asia. However, there are many similarities between GCA and TA, particularly in pathogenic mechanisms, histopathology, and clinical manifestations related to large vessel involvement. It remains unclear whether GCA and TA are distinct entities or represent different phenotypes of the same disease.
Behçet’s syndrome
Authors: Yurdakul Sebahattin , Fresko Izzet , Yazici Hasan 
Published: February 2020

Abstract
Behçet’s syndrome is an inflammatory disorder of unknown aetiology that involves arteries and veins of all sizes. Most cases are from the countries around the Mediterranean basin, the Middle East, and East Asia, with the highest prevalence in Turkey. It typically presents in the second and third decades with recurrent oral ulcers (98% of cases), genital ulcers (85%), acneiform lesions (85%), pathergy reaction (60% in some countries), erythema nodosum (50%), uveitis (50%), arthritis (50%), thrombophlebitis (30%), and less commonly with arterial occlusion/aneurysm, central nervous system involvement, or gastrointestinal lesions. A relapsing/remitting course is usual. Disease is more severe and mortality is higher in men. The diagnosis is clinical, laboratory findings are non-specific, and there is no specific diagnostic test for Behçet’s syndrome.
Polymyalgia rheumatica
Authors: Dasgupta Bhaskar , Matteson Eric L. 
Published: February 2020

Abstract
Polymyalgia rheumatica is one of the common inflammatory rheumatic diseases of older people. It overlaps with inflammatory arthritis and large-vessel vasculitis, particularly giant cell arteritis. Pathogenesis is unclear and may involve recognition of an infectious agent by aberrantly activated dendritic cells. Polymyalgia rheumatica is characterized by abrupt-onset pain and morning stiffness of the shoulder and pelvic girdle muscles, with an acute phase response (elevated erythrocyte sedimentation rate and/or C-reactive protein). Evaluation can be challenging, as many clinical and laboratory features may also be present in other conditions, including other rheumatological diseases, infection, and neoplasia. The mainstay of therapy is glucocorticoids. Adjunctive therapy can be initiated early in the disease course in patients with high risk of glucocorticoid-related side effects, severe or relapsing disease, or poor or ill sustained response to glucocorticoids. The response to standardized therapy is heterogeneous, and a significant proportion of patients do not respond completely.