Anti-melanoma differentiation-associated gene 5 antibody associated rapidly progressive interstitial lung disease in a pediatric patient: a case report.

backgroundRapidly progressive interstitial lung disease presents as a severe complication of juvenile dermatomyositis, particularly when associated with anti-melanoma differentiation-associated gene 5. We report a pediatric case that underscores the necessity for clinicians to maintain a high index of suspicion for early identification and management. CASE PRESENTATION: A previously healthy 7-year-old White girl presented with a 6-week history of generalized weakness, fever, joint pain, and abdominal pain. Initial examination revealed hypoxia, tachypnea, and hepatosplenomegaly. Laboratory tests were marked by thrombocytopenia, lymphopenia, elevated liver enzymes, high ferritin, high triglyceride, elevated muscle enzymes, and increased soluble IL-2 receptor, suggesting macrophage activation syndrome that was and managed with dexamethasone 5 mg/kg/m

conclusionsThis case demonstrates the diagnostic and therapeutic challenges in patients with rapidly progressive interstitial lung disease in the context of anti-melanoma differentiation-associated gene 5 associated juvenile dermatomyositis, who may not present with overt muscle and cutaneous features of juvenile dermatomyositis and whose lung disease can progress very rapidly. A high index of suspicion among clinicians is critical, and expedited diagnostic serology may assist with earlier diagnosis and initiation of therapy. Extracorporeal membrane oxygenation can be utilized as a bridge to diagnosis in the setting of severe refractory hypoxemic respiratory failure. However, despite aggressive treatment, the prognosis remains challenging.