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• W2088769712 abstract "<b>Background:</b> Immunoglobulin G4-related disease (IgG4-RD) is a systemic disorder which may involve the lungs, airways and pleura. <b>Objective:</b> To describe novel findings in pleural involvement by IgG4-RD. <b>Methods:</b> A case series of patients with histologically proven IgG4-RD treated for pleural effusion in our clinic. <b>Results:</b> We identified 4 male patients (aged 68-84) with pleural effusion caused by IgG4-RD. Effusions were lymphocytic exudates in all cases, with high protein concentrations (5.6-7.4 g/dL). All patients had hyperglobulinemia, elevated serum immunoglobulin G (IgG) levels and elevated levels of IgG subclasses 1 and 4 (IgG1 and IgG4). In two patients, levels of adenosine deaminase (ADA) were measured in the effusion and were elevated (309 and 108 IU/L). Tuberculosis was excluded in both cases by pleural biopsy. Thoracic lymphadenopathy was prominent in all patients. One patient developed radiological findings compatible with rounded atelectasis (<b>Figure</b>). In all cases, effusion responded to corticosteroids therapy. <b>Conclusions:</b> IgG4-RD may cause an ADA-positive, lymphocytic exudate with a high protein concentration, resembling tuberculous effusion. Thoracic lymphadenopathy, hyperglobulinemia, and an increased total IgG, IgG1, IgG4 may suggest the diagnosis. Previously undescribed, IgG4-RD pleural inflammation may result in rounded atelectasis. <b>Figure: Rounded atelectasis in a patient with IgG4-RD</b>" @default.
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• W2088769712 date "1994-06-04" @default.
• W2088769712 modified "2023-09-26" @default.
• W2088769712 title "Neonatal screening for cystic fibrosis using immunoreactive trypsinogen and direct gene analysis: four years' experience" @default.
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• W2088769712 doi "https://doi.org/10.1136/bmj.308.6942.1469" @default.
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