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- Title
Clinical and Prognostic Significance of p-ANCA Positivity in Idiopathic Pulmonary Fibrosis: A Retrospective Observational Study.
- Authors
Libra, Alessandro; Muscato, Giuseppe; Ielo, Giuseppe; Spicuzza, Lucia; Palmucci, Stefano; Fagone, Evelina; Fruciano, Mary; Gili, Elisa; Sambataro, Gianluca; Vancheri, Carlo
- Abstract
Perinuclear Anti Neutrophil Cytoplasmic Antibody (p-ANCA) is a serological marker of Microscopic Polyangiitis (MPA), a vasculitis associated with lung involvement potentially mimicking Idiopathic Pulmonary Fibrosis (IPF). In this study, we evaluated the role of p-ANCA in predicting clinical evolution and prognosis in a cohort of IPF patients. In this observational, retrospective, case–control study, we compared 18 patients with an IPF diagnosis and p-ANCA positivity with 36 patients with seronegative IPF, matched for age and sex. IPF patients with and without p-ANCA showed similar lung function decline during the follow-up, but IPF p-ANCA+ showed better survival. Half of IPF p-ANCA+ patients were classified as MPA for the development of renal involvement (55%) or skin signs (45%). The progression towards MPA was associated with high levels of Rheumatoid Factor (RF) at baseline. In conclusion, p-ANCA, mainly when associated with RF, could predict the evolution of Usual Interstitial Pneumonia (UIP) towards a definite vasculitis in patients, with a better prognosis compared with IPF. In this view, ANCA testing should be included in the diagnostic workup of UIP patients.
- Subjects
MICROSCOPIC polyangiitis; RHEUMATOID factor; SCIENTIFIC observation; OPTIMISM; IDIOPATHIC pulmonary fibrosis
- Publication
Diagnostics (2075-4418), 2023, Vol 13, Issue 11, p1882
- ISSN
2075-4418
- Publication type
Case Study
- DOI
10.3390/diagnostics13111882