Idiopathic lung fibrosis and anti myeloperoxidase glomerulonephritis: the tree that hides the forest
Abstract
Background: Although anti-neutrophil cytoplasmic antibodies [ANCA] are frequently found in patients diagnosed with idiopathic pulmonary fibrosis [IPF], current guidance does not recommend serologic testing for vasculitis.
Case presentation: A 71-year old Caucasian male, diagnosed with IPF three years earlier, presented with rapidly progressive glomerulonephritis. ANCA were found both in current and historical sera. A kidney biopsy sample was taken, which revealed a pauci-immune glomerulonephritis, but also areas of glomerular fibrosis, hence strongly suggesting unrecognized flares of an indolent vasculitis in his past. This made the diagnosis of " idiopathic " pulmonary fibrosis very unlikely.
Conclusion: As nephrologists, we argue that testing for ANCA should be performed on a systematic basis, at least in elderly patients, even in the absence of extra-pulmonary signs of vasculitis at presentation.
Origin : Publication funded by an institution
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