Mediastinal fibrosis as a puzzling presentation: A clinical conundrum in rheumatology: A case report

Granulomatosis with polyangiitis (GPA) is a type of small vessel vasculitis, most commonly involving the respiratory tract and kidneys. It can occasionally present with mass-like lesions at various sites, including the brain, orbit, skin, genitals, retroperitoneum, and mediastinum. Presentation with mediastinal fibrosis is rare and less commonly recognized. The current study presents the case of a mediastinal mass causing pulmonary artery compression and subsequent pulmonary hypertension (PH) that required stenting. Despite inconclusive mediastinal biopsy results, the integration of clinical, radiological, and laboratory findings led to the diagnosis of ANCA-associated vasculitis, highlighting the challenges and necessity of a multi-modal diagnostic approach in real-world clinical scenarios. This case underscores the importance of considering ANCA-associated vasculitis as a potential etiology in patients presenting with mediastinal fibrosis. Early recognition and appropriate management are crucial in optimizing outcomes for patients with this rare and challenging manifestation.