Autoimmune interstitial lung disease-arthritis syndrome
Autoimmune interstitial lung disease-arthritis syndrome
Definition
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 autoinflammation and autoimmunity, systemic, with immune dysregulation, COPA Syndrome — per MONDO