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Respiratory involvement in IgG4-related Mikuliczs disease
Modern Rheumatology, 08/19/2011
Matsui S et al. – The findings suggest that respiratory lesions are not rare in patients with IgG4–related Mikulicz’s disease (MD), and that they present with various manifestations. IgG4–related MD should be differentiated from similar diseases, such as sarcoidosis, bronchial asthma, Sjögren’s syndrome, and malignant lymphoma.
Methods- To clarify the clinical characteristics of respiratory involvement associated with IgG4–related MD, the authors retrospectively assessed 25 patients with MD, 11 (44%) of whom had allergic symptoms, and 7 (28%) of whom complained of respiratory problems.
- Thirteen patients (52%) presented with pulmonary and/or mediastinal lesions (P–MD) on chest computed tomography (CT), and 11 (44%) had lesions limited to the lacrimal and/or salivary glands (L–MD).
- Mean serum total protein, IgG, and IgG4 concentrations were significantly higher and CH50 was significantly lower in the P–MD than in the L–MD group.
- Immune complex was present only in the P–MD group.
- Chest CT images showed bronchial wall thickening, consolidation, nodule(s), interlobular thickening, ground glass opacity, pleural thickening/effusion, and mediastinal lymphadenopathy.
- Five of seven patients who underwent histological examination of the lungs had abundant IgG4–positive plasma cell infiltrates (IgG4/IgG–positive plasma cells >40%), but the other two did not.