Abstract
AbstractAdult-onset Still’s disease is a rare, systemic inflammatory rheumatic disease characterized by recurrent fevers, arthritis, and an evanescent rash. One of the most serious hematologic derangements that can be seen with adult-onset Still’s disease is macrophage activation syndrome. Macrophage activation syndrome is characterized by activation of lymphocytes, resulting in a cytokine storm and hemophagocytosis in the bone marrow, along with multi-organ failure. Adult-onset Still’s disease with macrophage activation syndrome first presenting during pregnancy is exceptionally rare; here, we report two unique cases of such a presentation and review the pertinent literature. Both of our cases presented critically ill with end-organ failure, and responded to immunosuppression; fetal demise was present in one and an emergency caesarean section with a viable fetus was performed in the other patient. Maternal outcomes were favorable in both cases and both patients did well long-term with systemic therapy. Systemic immunosuppression, particularly anti-IL1 therapy, may be considered as treatment for this rare and life-threatening condition when presenting during pregnancy.
Funder
University of Southern California
Publisher
Springer Science and Business Media LLC
Subject
General Medicine,Rheumatology
Reference37 articles.
1. Gagnier JJ, Kienle G, Altman DG, Moher D, Sox H, Riley D et al (2013) The CARE guidelines: consensus-based clinical case reporting guideline development. Glob Adv Health Med 2(5):38–43. https://doi.org/10.7453/gahmj.2013.008
2. Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S et al (2007) HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 48(2):124–131. https://doi.org/10.1002/pbc.21039
3. Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H et al (1992) Preliminary criteria for classification of adult Still's disease. J Rheumatol 19(3):424–430
4. Mai TA, Ayyala-Somayajula D, Singh J, Braxton D, Wise L, Yuan L (2022) Adult-onset Still's disease with macrophage activation syndrome during pregnancy: a unique cause of hepatic dysfunction. ACG Case Rep J 9(12):e00921. https://doi.org/10.14309/crj.0000000000000921
5. Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Sève P (2014) Adult-onset Still's disease. Autoimmun Rev 13(7):708–722. https://doi.org/10.1016/j.autrev.2014.01.058