Background: Microscopic polyangiitis is a vasculitic disease that may result in a pulmonary renal syndrome. Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis is strongly associated with infection.
Case summary: We describe a case of microscopic polyangiitis that developed in a patient with MPO-ANCA positive pulmonary fibrosis following infection with mycoplasma. A renal biopsy was undertaken following the detection of microscopic hematuria during follow-up but no abnormal findings were evident. The MPO-ANCA titer increased following infection with mycoplasma pneumonia and a second renal biopsy demonstrated crescentic glomerulonephritis. The degree of pulmonary fibrosis was unaffected.
Discussion: The present case suggests that the mycoplasma infection triggered the elevation of MPO-ANCA titer and provoked glomerulonephritis in a patient with MPO-ANCA positive IPF. This case indicates the importance of testing for MPO-ANCA at the time of initial diagnosis, performing urinalysis and examining the urine sediment during follow-up and being alert to the potential onset of vasculitis in cases of pulmonary fibrosis.