Rescued from desperation: adult-onset Still’s disease with life-threatening interstitial lung disease and macrophage activation

• DOI: 10.1007/s00393-021-01063-7
• Publication Date: 2021-08-10T16:05:52Z
• Authors: Guangfeng Zhang; Wenxue Liu; Xiao Zhang; Liang Liu
• Keywords: Adult; Male; 03 medical and health sciences; 0302 clinical medicine; Macrophage Activation Syndrome; Humans; Macrophage Activation; Antibodies, Monoclonal, Humanized; Lung Diseases, Interstitial; Still's Disease, Adult-Onset; 3. Good health

This article presents a case of fulminant interstitial lung disease (ILD) with macrophage activation syndrome (MAS) as a rare complication of adult-onset Still's disease in a 43-year-old male patient. Initial presentation showed severe ILD. Early treatment focused on high-dose corticosteroids; however, disease remission could not be achieved. After an additional inflammatory focus and underlying malignancy were excluded, the triad of pancytopenia, fever, and high ferritin levels indicated MAS; bone marrow biopsy confirmed secondary hemophagocytic histiocytosis. Treatment with tocilizumab induced fast and effective therapeutic success.