A case of adult-onset Still’s disease that does not fulfill Yamaguchi’s and Fautrel’s criteria: Sensitivity limitations and improvement proposal

Abstract

Adult-Onset Still's Disease (AOSD) is an uncommon inflammatory condition that lacks a universally accepted diagnostic test. The clinical presentation comprises symptoms such as fever, rash, joint pain, sore throat, swelling of lymph nodes, and enlargement of the liver and spleen. The diagnostic criteria developed by Yamaguchi and Fautrel are commonly employed because of their great sensitivity and specificity. However, there are cases in which individuals may not meet these criteria but still demonstrate symptoms of AOSD. In this case, we report an elderly patient who has been diagnosed with AOSD but does not meet the criteria for any of these criteria. We discussed the factors contributing to impaired sensitivity and put forth various suggestions to enhance the sensitivity of these criteria.

Keywords

• Fever
• Sensitivity and Specificity
• Adult-Onset Still

References

1. Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Sève P. Adult-onset Still’s disease. Autoimmun Rev. 2014 Jul;13(7):708-22. doi: 10.1016/j.autrev.2014.01.058.
2. Feist E, Mitrovic S, Fautrel B. Mechanisms, biomarkers and targets for adult-onset Still’s disease. Nat Rev Rheumatol. 2018 Oct;14(10):603-18. doi: 10.1038/s41584-018-0081-x.
3. Efthimiou P, Kontzias A, Hur P, Rodha K, Ramakrishna GS, Nakasato P. Adult-onset Still’s disease in focus: Clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies. Semin Arthritis Rheum. 2021 Aug;51(4):858-74. doi:10.1016/j.semarthrit.2021.06.004
4. Kakehi M, Amano S, Sano C, Ohta R. Atypical adult Still’s disease complicated by hemophagocytic syndrome in an older patient: A case report. Cureus. 2023 Oct 12;15(10):e46922. doi: 10.7759/cureus.46922.
5. Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, Kashiwazaki S, Tanimoto K, Matsumoto Y, Ota T, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol. 1992 Mar;19(3):424-30.
6. Fautrel B, Zing E, Golmard JL, Le Moel G, Bissery A, Rioux C, Rozenberg S, Piette JC, Bourgeois P. Proposal for a new set of classification criteria for adult-onset still disease. Medicine (Baltimore). 2002 May;81(3):194-200. doi: 10.1097/00005792-200205000-00003.
7. Roghmann MC, Warner J, Mackowiak PA. The relationship between age and fever magnitude. Am J Med Sci. 2001 Aug;322(2):68-70. doi: 10.1097/00000441-200108000-00003.
8. Kim MJ, Ahn EY, Hwang W, Lee Y, Lee EY, Lee EB, Song YW, Park JK. Association between fever pattern and clinical manifestations of adult-onset Still’s disease: unbiased analysis using hierarchical clustering. Clin Exp Rheumatol. 2018 Nov-Dec;36(6 Suppl 115):74-9.

9. Mollaeian A, Chen J, Chan NN, Nizialek GA, Haas CJ. Adult onset Still’s disease in the elderly: a case-based literature review. BMC Rheumatol. 2021 Apr 20;5(1):12. doi: 10.1186/s41927-021-00183-6.
10. Vural AT, Özcan D, Tepeoğlu M. Adult Still’s disease: An unusual case with atypical skin and histopathological findings. Turk Arch Dermatol Venereol. 2020;54:111-3. doi: 10.4274/turkderm.galenos.2020.87522
11. Gündüz E, Güllü MN, Zengin Y, Dursun R, İçer M, Özhasenekler A, Karakoç Y, Orak M. Adult-Onset Still’s Disease: Case Report. JAEMCR. 2014; 5: 120-2. doi: 10.5152/jaemcr.2014.70707.
12. Narváez Garcia FJ, Pascual M, López de Recalde M, Juarez P, Morales-Ivorra I, Notario J, Jucglà A, Nolla JM. Adult-onset Still’s disease with atypical cutaneous manifestations. Medicine (Baltimore). 2017 Mar;96(11):e6318. doi: 10.1097/MD.0000000000006318.
13. Sen K, Ghosh UC, Mukherjee AK, Ghosal A, Pandey UK. A case of adult onset Still’s disease with flare of arthritis. J Indian Med Assoc. 2013 Feb;111(2):132-134.
14. Guerber A, Garneret E, El Jammal T, Zaepfel S, Gerfaud-Valentin M, Sève P, Jamilloux Y. Evaluation of glycosylated ferritin in adult-onset Still’s disease and differential diagnoses. J Clin Med. 2022 Aug 26;11(17):5012. doi: 10.3390/jcm11175012.