Orphanet: Autoimmune interstitial lung disease arthritis syndrome

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Autoimmune interstitial lung disease-arthritis syndrome

Disease definition

A rare genetic systemic or rheumatologic disease characterized by interstitial lung disease (often with pulmonary hemorrhage) and inflammatory arthritis, associated with high-titer autoantibodies (including anti-nuclear and anti-neutrophil cytoplasmic antibodies, and rheumatoid factor). Patients present from infancy to adolescence with tachypnea, cough, hemoptysis, and/or joint pain. Some patients may also develop glomerular disease.


Classification level: Disorder
  • Synonym(s):
    • COPA syndrome
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Infancy, Childhood
  • ICD-10: J84.8
  • OMIM: 616414
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -
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