Autoimmune interstitial lung disease-arthritis syndrome

MONDO:0014629

A respiratory 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.

Also known as: COPA Syndrome, autoinflammation and autoimmunity, systemic, with immune dysregulation, AILJK, autoimmune interstitial lung, joint, and kidney disease

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