Significance of early treatment in granulomatosis with polyangiitis vasculitis

Vasculitis is a multisystemic disease that affects vessels of different sizes. Its presentation can vary widely depending on the system involved. It may present with constitutional symptoms or with specific features of end-organ involvement. The diagnosis is built on a compatible pattern of clinical features supported by specific serological or radiological investigations and confirmatory biopsy. Tissue biopsy is vital to confirm the diagnosis of vasculitis; however, this should not delay treatment when presentation strongly suggests vasculitis. We describe a case of a 72-year-old man treated with steroids, plasma exchange, and rituximab for suspected granulomatosis with polyangiitis (GPA) given his clinical presentation including suspected scleritis of the right eye, hearing changes, sinusitis, diffuse alveolar hemorrhage, pulmonary lesions, kidney failure, palpable purpura, and radiological evidence of pansinusitis and pulmonary lesions without waiting for serology or tissue confirmation. This case highlights the importance of recognizing the clinical features of GPA to initiate prompt treatment as it can progress rapidly and be fatal.