MPDC and HN took care of the patient, were involved in the interpretation of the clinical and biological features of the patient, and in drafting the manuscript. IB was involved in the analysis of the biopsy sample, and critically revised the draft. AH was involved in the diagnostic procedure, wrote the draft, and coordinated the authors to in order to finalize the manuscript. All author’s approve the final version of the manuscript.
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.
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.
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.
Hiromura K, Nojima Y, Kitahara T, Ueki K, Maezawa A, Kawai H, et al. Four cases of anti-myeloperoxidase antibody-related rapidly progressive glomerulonephritis during the course of idiopathic pulmonary fibrosis. Clin Nephrol. 2000;53(5):384–9. PubMed
Comarmond C, Crestani B, Tazi A, Hervier B, Adam-Marchand S, Nunes H, et al. Pulmonary fibrosis in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a series of 49 patients and review of the literature. Medicine (Baltimore). 2014;93(24):340–9. CrossRef
Network IPFCR, Raghu G, Anstrom KJ, King Jr TE, Lasky JA, Martinez FJ. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med. 2012;366(21):1968–77. CrossRef
- Idiopathic lung fibrosis and anti myeloperoxidase glomerulonephritis: the tree that hides the forest
Marc Pineton de Chambrun
- BioMed Central
Neu im Fachgebiet Innere Medizin
Meistgelesene Bücher aus der Inneren Medizin
Mail Icon II