Usual interstitial pneumonia in ANCA-associated vasculitis: A poor prognostic factor.
J Autoimmun
; 106: 102338, 2020 01.
Article
em En
| MEDLINE
| ID: mdl-31570253
ABSTRACT
BACKGROUND:
Progressive fibrosing interstitial lung disease (ILD) is rarely associated with antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV). This study focused on the outcomes of ILD patients with associated AAV (AAV-ILD).METHODS:
AAV-ILD (cases microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA) with ILD) were compared to AAV patients without ILD (controls). ILD was defined as a usual interstitial pneumonia (UIP) or non-specific interstitial pneumonia (NSIP) pattern. Two controls were matched to each case for age (>or ≤65 years), ANCA status (PR3-or MPO-positive) and creatininemia (≥or <150⯵mol/L).RESULTS:
Sixty-two cases (89% MPO-ANCA+) were included. Median age at AAV diagnosis was 66 years. ILD (63% UIP), was diagnosed before (52%) or simultaneously (39%) with AAV. Cases versus 124 controls less frequently had systemic vasculitis symptoms. One-, 3- and 5-year overall survival rates, respectively, were 96.7%, 80% and 66% for cases versus 93.5%, 89.6% and 83.8% for controls (pâ¯=â¯0.008). Multivariate analyses retained age >65 years (hazard ratio (HR) 4.54; pâ¯<â¯0.001), alveolar haemorrhage (HR 2.25; pâ¯=â¯0.019) and UIP (HR 2.73; pâ¯=â¯0.002), but not immunosuppressant use, as factors independently associated with shorter survival.CONCLUSION:
For AAV-ILD patients, only UIP was associated with poorer prognosis. Immunosuppressants did not improve the AAV-ILD prognosis. But in analogy to idiopathic pulmonary fibrosis, anti-fibrosing agents might be useful and should be assessed in AAV-ILD patients with a UIP pattern.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Doenças Pulmonares Intersticiais
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Fibrose Pulmonar Idiopática
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Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos
Tipo de estudo:
Observational_studies
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Prognostic_studies
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Risk_factors_studies
Limite:
Aged
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Female
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Humans
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Male
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Middle aged
Idioma:
En
Ano de publicação:
2020
Tipo de documento:
Article