C-ANCA positive GPA with pulmonary involvement: a case report

Abstract

Granulomatosis with polyangitis (GPA) represents a rare cause of systemic vasculitis with relatively even fewer cases reported from Africa. The possible explanations for the relatively low incidence are thought to be because of possibly actual low prevalence in this population, low index of suspicion for the condition and under-diagnosis due to lack of diagnostic services. In this case report we present a 75 year old female with cytoplasmic anti- neutrophil cytoplasmic antibody (c-ANCA) positive granulomatosis with polyangitis with predominantly pulmonary involvement admitted with pneumonia and Acute  Respiratory Distress Syndrome (ARDS) who transiently improved with  immunosuppressive therapy, plasma exchange and rituximab but eventually succumbed due to septic shock.

Key words: C-ANCA, Pulmonary, Granulomatosis, Vasculitis.