Adult Onset Still’s Disease Presenting as Fever of Unknown Origin: A Case Report

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  •   Arun Agarwal

  •   Piyusha Choudhary

  •   Rajesh K. Samota

  •   Aditi Sharma

Abstract

26 years’ female patient attended triage with complaints of fever of almost 1-month duration. She was diagnosed as a case of Brucellosis outside. However, her fever persisted and she was finally diagnosed to have new onset adult-onset still’s disease (AOSD) with active macrophage activation syndrome (MAS) and multi organ failure. She met the diagnostic criteria for AOSD. She had high spiky fevers, hepatosplenomegaly, Lymphadenopathy, coagulopathy, anaemia with thrombocytopenia, hemophagocytosis, markedly elevated serum ferritin, low to absent natural killer (NK) cells, and remained unresponsive to steroids, intravenous immunoglobulin (IVIG) and hemophagocytic lymphohistiocytosis (HLH) protocol treatment. Interleukin-1 (IL1) inhibitors were not available. Interleukin-6 (IL6) blockers were not considered due to greater concerns about exacerbating bacterial infections with IL-6 blockade than with IL-1 blockade and much longer half-lives of the IL-6 inhibitors. We were also reluctant in using tocilizumab or sarilumab in new-onset disease where diagnostic uncertainty remains, as is often the case. She succumbed to her disease which had rapid fulminating course with multi organ failure. Her diagnostic and therapeutic challenges are discussed.


Keywords: Adult onset Still’s disease (AOSD), hemophagocytic lymphohistiocytosis (HLH), macrophage activation syndrome (MAS), reactive hemophagocytic syndrome (RHS), secondary hemophagocytic lymphohistiocytosis (sHLH)

References

Magadur-Joly G, Billaud E, Barrier JH, Pennec YL, Masson C, Renou P, et al. Epidemiology of adult Still's disease: estimate of the incidence by a retrospective study in west France. Ann Rheum Dis. 1995; 54(7): 587.

Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol. 1992; 19(3): 424-30.

Cush JJ, Medsger TA, Jr, Christy WC, Herbert DC, Cooperstein LA. Adult‐onset Still's disease. Clinical course and outcome. Arthritis Rheum. 1987; 30(2): 186–194.

Fautrel B, Zing E, Golmard JL, Le Moel G, Bissery A, Rioux C, et al Proposal for a new set of classification criteria for adult‐onset still disease. Medicine (Baltimore). 2002; 8(1): 194–200.

Janka GE, Lehmberg K. Hemophagocytic lymphohistiocytosis: pathogenesis and treatment. Hematology 2013; 2013: 605-11.

Crayne CB, Albeituni S, Nichols KE, Cron RQ. The Immunology of Macrophage Activation Syndrome. Front Immunol. 2019; 10: 119.

Gerfaud-Valentin M, Maucort-Boulch D, Hot A, Iwaz J, Ninet J, Durieu I, et al. Adult-onset still disease: manifestations, treatment, outcome, and prognostic factors in 57 patients. Medicine (Baltimore). 2014; 93(2): 91-99.

Fukaya S, Yasuda S, Hashimoto T, Oku K, Kataoka H, Horita T, et al. Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases, Rheumatology. 2008; 47(11): 1686-1691.

Chang-Bum B, Ju-Yang J, Hyoun-Ah K, Chang-Hee S. Reactive Hemophagocytic Syndrome in Adult-Onset Still Disease, Medicine. 2015; 94(4): e451.

Maria AT, Le Quellec A, Jorgensen C, Touitou I, Rivière S, Guilpain P. Adult onset Still's disease (AOSD) in the era of biologic therapies: dichotomous view for cytokine and clinical expressions. Autoimmun Rev. 2014; 13(11): 1149-59.

Al-Homood IA. Biologic treatments for adult-onset Still’s disease, Rheumatology. 2014; 53(1): 32–38.

Jamilloux Y, Gerfaud-Valentin M, Henry T, Sève P. Treatment of adult-onset Still's disease: a review. Ther Clin Risk Manag. 2014; 11: 33-43.

Lenert A, Yao Q. Macrophage activation syndrome complicating adult onset Still's disease: A single center case series and comparison with literature. Semin Arthritis Rheum. 2016; 45(6): 711-6.

Schulert GS, Grom AA. Pathogenesis of macrophage activation syndrome and potential for cytokine- directed therapies. Annu Rev Med. 2015; 66: 145-59.

Gianella S, Schaer DJ, Schwarz U, Kurrer M, Heppner FL, Fehr J, et al. Retinal microangiopathy and rapidly fatal cerebral edema in a patient with adult-onset Still's disease and concurrent macrophage activation syndrome. Am J Hematol. 2008; 83(5): 424-7.

Kaneko K, Kaburaki M, Muraoka S, Tanaka N, Yamamoto T, Kusunoki Y, et al. Exacerbation of adult-onset Still's disease, possibly related to elevation of serum tumor necrosis factor-alpha after etanercept administration. Int J Rheum Dis. 2010; 13(4): e67-9.

Kobayashi M, Takahashi Y, Yamashita H, Kaneko H, Mimori A. Benefit and a possible risk of tocilizumab therapy for adult-onset Still's disease accompanied by macrophage-activation syndrome. Mod Rheumatol. 2011; 21(1): 92-6.

Banse C, Vittecoq O, Benhamou Y, Gauthier-Prieur M, Lequerré T, Lévesque H. Reactive macrophage activation syndrome possibly triggered by canakinumab in a patient with adult-onset Still's disease. Joint Bone Spine. 2013; 80(6): 653-5.

Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007; 48(2): 124-31

Opoka-Winiarska V, Grywalska E, Roliński J. Could hemophagocytic lymphohistiocytosis be the core issue of severe COVID-19 cases? BMC Med. 2020; 18(1): 214

Agarwal A, Agarwal M, Khandelwal V, Mathur P. Adult-onset Still's disease masquerading as multiple organ failure: Neither benign nor so rare. Journal of clinical rheumatology. 2018; 24(6): 342-346

Agarwal A, Agarwal A. Adult onset stills disease: Advocating for new markers to overcome the diagnostic challenge. Asian Journal of Medicine and Health.2018; 10(4): 1-8.

Agarwal A, Agarwal A, Khan A. A case of Adult Onset Stills Disease: Not so Uncommon Cause of Fever of Unknown Origin. Asian Journal of Medicine and Health. 2018; 11(3): 1-7

Agarwal A, Gondaliya D N. Adult-onset Still's disease: A case report J Acute Dis.2020; 9(4): 179-182.

Agarwal A, Agarwal A. Infection Associated Secondary Hemophagocytic Lymphohistiocytosis in Sepsis Syndromes - A Tip of an Iceberg. J Assoc Physicians India. 2016; 64(10): 44-50.

Carter SJ, Tattersall RS, Ramanan AV. Macrophage activation syndrome in adults: recent advances in pathophysiology, diagnosis and treatment. Rheumatology (Oxford). 2019; 58(1): 5-17.

Fukaya S, Yasuda S, Hashimoto T, Oku K, Kataoka H, Horita T, et al. Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases. Rheumatology (Oxford). 2008; 47(11): 1686-91.

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How to Cite
Agarwal, A., Choudhary, P., Samota, R. K., & Sharma, A. (2022). Adult Onset Still’s Disease Presenting as Fever of Unknown Origin: A Case Report. European Journal of Clinical Medicine, 3(2), 1–5. https://doi.org/10.24018/clinicmed.2022.3.2.187