Chronic thromboembolic disease and necrotizing granulomatous vasculitis – A case report

A 32 year old male presented to our services with severe, progressive breathlessness. Following a detailed clinical work-up he was found to have severe pulmonary arterial hypertension due to chronic thromboembolic disease. No risk factor for venous thromboembolism,was identified at this time. Vasculitic screen including anti-neutrophil cytoplasmic antibody (ANCA) was normal. He was referred for pulmonary artery endarterectomy which was performed successfully and with good initial improvement in his symptom complex. He represented with breathlessness four months later. He was diagnosed with necrotizing granulomatous vasculitis (known formerly as Wegner's Granulomatosis). While an association between necrotizing granulomatous vasculitis and venous thromboembolic disease is well described, to our knowledge this is the first case of a patient with chronic thromboembolic pulmonary hypertensive disease subsequently developing fulminant necrotizing granulomatous vasculitis.