IgG4-related disease is a rheumatological disorder, affecting multiple organ systems, and displaying dense lymphoplasmacytic inflammatory infiltrate and storiform fibrosis on histology. The pulmonary manifestations of IgG4-related disease are varied. Most commonly, bronchovascular consolidation and perilymphatic parenchymal thickening occur. IgG4-related disease can present as a solitary parenchymal mass and is often mistaken for a primary pulmonary or haematological malignancy. This report presents a case of IgG4-related disease in a patient with 6 months reported haemoptysis symptomatology and CT findings of perihilar lymphadenopathy, multiple pulmonary parenchymal nodules and a single parenchymal mass. Clinician cognisance of the histopathological presentation of IgG4-disease is important. It should be considered as a differential in patients for which investigations have ruled out malignancy, as it is responsive to glucocorticoids in the majority of cases.
- interstitial lung disease
- lung cancer (oncology)
- respiratory system
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Contributors This case report was written by Dr Natalie Simon and Dr Mostafa Negmeldin.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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