CASE PRESENTATION: A 46 year-old African American female with no significant PMH presented with a pruritic, papular rash on her arms and chest. The rash resolved with steroid cream. Over the next 6 weeks she developed dyspnea, fevers, dry cough and hypoxia. Chest radiograph revealed bilateral interstitial infiltrate. Patient failed to respond to antibiotics and diuretics. CT scan showed diffuse ground-glass and consolidative opacities. A bronchoscopy with BAL revealed a lymphocytosis of 36%. Cultures were negative. Labs revealed ANA and dsDNA positivity. Patient denied all rheumatologic symptoms. SS-A, SS-B, Scl-70, Smith, RNP, ANCA, Anti-Jo1, RF, Anti-CCP and HIV were negative. CK and aldolase were normal. Patient was placed on Prednisone 1 mg/kg. She clinically deteriorated. Open lung biopsy showed organizing pneumonia with diffuse alveolar damage. Based on her 100% oxygen requirement and failure to respond to corticosteroids, she was sent for lung transplant evaluation. At the transplant center an extended myositis panel revealed Anti-MDA5 antibody. Patient was diagnosed with CADM-ILD, started on mycophenolate and given rituxamab. She responded well and is now 1 year removed from her initial presentation on minimal oxygen.