IgG4-related disease is a heterogeneous group of diseases that can affect a single organ or manifest as a systemic disease. We present the case of a 55-yearold female, admitted for dry cough, fever, progressive dyspnea and respiratory failure. Chest CT showed areas of diffuse ground glass, reticular pattern and alveolar consolidation. She received treatment for community acquired pneumonia and systemic corticosteroids with good response. One month later, she was admitted again due to subglottic stenosis, progression of pulmonary infiltrates, and increased palpebral and submaxillary glands volume. Transbronchial and salivary gland biopsies showed infiltration by IgG4. Increased levels of plasma IgG4 were also detected. Immunosuppressive therapy was given with good response.
Keywords: IgG4; IgG4 related lung disease; interstitial lung disease.