IgG4-related lung disease: a case series of 6 patients and review of the literature.

In this report, we describe the clinical and pathologic features of six patients with IgG4-related disease of the lung. Patients were followed 1 - 5 years following their diagnosis. We describe unique features of IgG4- related lung disease, including one patient who presented with alveolar hemorrhage and a positive anti-neutrophil cytoplasmic antibody and two patients whose disease improved after treatment with mycophenylate mofetil. Two patients presented with pulmonary pseudotumor. We conclude that the clinical presentation of IgG4-related lung disease varies widely, and histopathology remains the key to diagnosis. PMID: 26847104 [PubMed - in process]
Source: Sarcoidosis Vasculitis and Diffuse Lung Diseases - Category: Respiratory Medicine Tags: Sarcoidosis Vasc Diffuse Lung Dis Source Type: research