An 83-year-old man presented with a three-week history of dyspnea. The clinical features suggested a diagnosis of relapsing polychondritis (RP); however, the patient died of heart failure. An autopsy revealed active chondritis of the tracheal and bronchial cartilage. Furthermore, giant cell myocarditis (GCM) and myositis were detected. To the best of our knowledge, this represents the first report of RP complicated by GCM and myositis. In patients with RP, GCM and myositis, CD163-positive macrophages and T-cells are most common, and the T-cell subset exhibits CD8 predominance. Common mechanisms of tissue damage caused by cytotoxic T-cells are likely to contribute to RP, GCM and myositis.

The full picture of immunoglobulin G4-related lung disease (IgG4-RLD) has not yet been elucidated. A 69-year-old man was referred to us with a more than 2-week history of productive cough and fatigue. Chest CT showed an airspace consolidation along the bronchovascular bundles. The pathological findings that were obtained from an open-lung biopsy showed both organizing pneumonia and interstitial pneumonia. Based on the established, comprehensive diagnostic criteria for IgG4-related disease (RD) as of 2011, this patient was given a definitive diagnosis of IgG4-RD. A further accumulation and analysis of those cases that concomitantly present with both IgG4-RLD and organizing pneumonia, like our patient, may contribute to the elucidation of the pathology of IgG4-RLD and the establishment of the disease spectrum.

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