Allogeneic Hematopoietic Cell Transplant for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome. (24th May 2021)

• Record Type: Journal Article
• Authors: Davidson, Nicole; Rangarajan, Hemalatha G.; Driest, Kyla; Bajwa, Rajinder P. S.; Polishchuk, Veronika; Abu-Arja, Rolla F.
• Other Names: Koarada Syuichi Academic Editor.
• Is Part Of: Case reports in rheumatology. Volume 2021(2021)
• Publication Date: 2021-05-24
• Subjects: Rheumatology -- Periodicals; Rheumatology -- Case studies -- Periodicals; Rheumatic Diseases; Connective Tissue Diseases; Rheumatology; Periodicals; Electronic journals; Case studies; Periodicals; 616.723
• DOI: 10.1155/2021/9323141
• Languages: English
• ISSNs: 2090-6889

Abstract

Abstract : Systemic juvenile idiopathic arthritis (sJIA) is characterized by arthritis, fever, rash, lymphadenopathy, hepatosplenomegaly, and serositis. Macrophage activation syndrome is the most feared complication of sJIA with a high risk of mortality. We report a 16-year-old female diagnosed with refractory systemic juvenile idiopathic arthritis (sJIA) complicated by recurrent macrophage activation syndrome (MAS), severe joint disease, and lung involvement requiring prolonged immunosuppressive therapy. She received a matched unrelated allogeneic hematopoietic cell transplant (Allo-HCT) using a reduced-intensity conditioning regimen and is now, 3 years after the transplant, with complete resolution of sJIA symptoms, off immunosuppressants, and with significant improvement in the quality of life.