Case report demonstrates predominant thoracic IgG4-related disease mimicking malignancy in an older male, highlighting the necessity of multidisciplinary evaluation for prompt diagnosis.
Objective To report a rare case of IgG4-related disease (IgG4-RD) with atypical predominant thoracic involvement mimicking lung cancer and sarcoidosis. Case presentation A 74-year-old male with a history of heavy smoking presented with chronic cough and exertional dyspnea for 1 year. Chest high-resolution computed tomography (HRCT) revealed bronchovascular bundle thickening, a right parahilar mass, multiple perilymphatically distributed solid nodules, lymphadenopathy, and pleural effusion. Laboratory tests revealed marked elevations of serum IgG4 (6710 mg/dL) and IgE (2210 mg/dL). Histopathological examination of lung, pleural biopsy, and lymph node consistently demonstrated dense lymphoplasmacytic infiltration with abundant IgG4-positive plasma cells, without storiform fibrosis or obliterative phlebitis. The patient was definitively diagnosed with IgG4-RD via multidisciplinary team (MDT) consultation. Oral prednisone resulted in significant radiological improvement. Conclusions IgG4-RD can mimic common thoracic disorders such as lung cancer and sarcoidosis. Early corticosteroid therapy is effective. An experienced MDT, integrating detailed clinical findings, serology, radiology, and histopathology, is essential for prompt diagnosis and timely intervention.
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Zhu et al. (2026) studied this question.
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