Rapidly progressive course of pauci-immune pulmonary capillaritis in a 70-year-old Asian male refractory to immunosuppression and plasma exchange

Abstract

A 70-year-old man presented with acute respiratory failure, alveolar infiltrates and haemoptysis requiring supplemental oxygen. Flexible bronchoscopy with bronchoalveolar lavage identifies diffuse alveolar haemorrhage. Clinical and serological evaluations do not identify a precise aetiology and histopathology establishes the diagnosis of isolated pauci-immune pulmonary capillaritis. The patient received induction therapy with high dose methylprednisolone at 1000 mg/day for 5 days and weekly rituximab at 375 mg/m2 scheduled over 4 weeks. Although the patient demonstrated clinical improvement after the first week, he experienced a rapid relapse requiring mechanical ventilation. His induction rituximab regimen was continued and plasma exchange was initiated. Despite these therapies, the patient’s condition deteriorated and passed away. Our case adds insight to the management of this rare entity and describes the use of plasma exchange as salvage therapy.