Myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a recognised cause of rapidly progressive glomerulonephritis (GN), while cold agglutinin disease (CAD) is a rare autoimmune haemolytic disorder that is frequently associated with infections.The simultaneous occurrence of MPO-ANCA GN and CAD is uncommon and presents significant diagnostic and therapeutic challenges.We describe a patient with longstanding asymptomatic MPO-ANCA seropositivity who developed rapidly progressive renal dysfunction, active urinary sediment, and biopsy-proven crescentic GN in association with cold agglutinin-mediated haemolysis following a recent respiratory illness attributed to Chlamydia pneumoniae infection.This case suggests a second-hit mechanism in which C. pneumoniae may have contributed to the development of two immune-mediated processes in a susceptible host and highlights the importance of evaluating for infectious triggers when AAV presents with atypical haematological features.
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Abdelwahab et al. (2026) studied this question.
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