Abstract
IgG4‐related disease (IgG4‐RD) is a systemic fibroinflammatory disease characterized by tumefactive lesions in various organ systems, including the lungs. Clinical and radiological manifestations of IgG4‐RD are relatively non‐specific, and we report a case highlighting the importance of histopathological confirmation in cases of suspected IgG4‐related lung disease. A 44‐year‐old male with significantly elevated serum IgG4 levels, patchy consolidation on thoracic CT imaging, and cough was referred with suspected IgG4‐related lung disease. However, surgical lung biopsy revealed an unexpected diagnosis of pulmonary schistosomiasis, and following treatment with praziquantel, cough resolved and IgG4 levels significantly declined. This case highlights the potentially diverse array of conditions that may mimic IgG4‐related lung disease and the importance of comprehensive evaluation including histopathological confirmation where possible.
Highlights
IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disease characterized by elevated serum IgG4 levels and typical histological features, including a lymphoplasmacytic inflammatory tissue infiltrate rich in IgG4-positive plasma cells and varying degrees of tissue fibrosis [1]
We report a case of a 44-year-old male referred with suspected IgG4-related lung disease that highlights the importance of a comprehensive clinical assessment, including a confirmatory tissue biopsy
Despite elevated serum IgG4 levels coupled with suggestive symptoms and radiological changes, a surgical lung biopsy revealed an unexpected diagnosis of pulmonary schistosomiasis
Summary
IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disease characterized by elevated serum IgG4 levels and typical histological features, including a lymphoplasmacytic inflammatory tissue infiltrate rich in IgG4-positive plasma cells and varying degrees of tissue fibrosis [1]. IgG4-related lung disease is increasingly recognized with reported manifestations including mediastinal adenopathy, interstitial involvement, parenchymal nodules, and consolidation [3]. Given the non-specific nature of symptoms and diverse radiological findings, a definitive diagnosis of IgG4-related lung disease often requires histopathological confirmation and clinico-pathological correlation.
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