Rescued from desperation: adult-onset Still's disease with life-threatening interstitial lung disease and macrophage activation : Successful treatment by Tocilizumab.

Z Rheumatol

State Key Laboratory of Quality Research in Chinese Medicine/Macau Institute for Applied Research in Medicine and Health, Macau University of Science and Technology, 999078, Macau, China.

Published: May 2022

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.

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http://dx.doi.org/10.1007/s00393-021-01063-7DOI Listing

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