Clinical case of a patient with adult-onset Still’s disease – a diagnostic challenge or a late diagnosis?
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Keywords

Still's syndrome
autoinflammatory syndrome
classification criteria

How to Cite

[1]
Emin, S., Boyadzhieva, V., Stoilov, N. and Stoilov, R. 2021. Clinical case of a patient with adult-onset Still’s disease – a diagnostic challenge or a late diagnosis?. Revmatologiia (Bulgaria). 28, 4 (Feb. 2021), 72-80. DOI:https://doi.org/10.35465/28.4.2020.pp72-80.

Abstract

Abstract. Adult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory disease with poorly understood etiology, that features quotidian spiking fevers, polyarthritis and distinctive salmon-colored maculopapular rash. Extremely high levels of ferritin are present in most patients, making it an important laboratory test for diagnosis. AOSD is a crucial diagnostic and therapeutic problem, which in many cases remains unrecognized due to the rarity in the population and the diverse clinical features. We present the clinical case of a 34-yearold woman with a first manifestation – maculopapular rash on the dorsal surface of the wrist joints and on the knee joints. At this time the patient has first-time pregnancy in the 6th gestational week. During a gynecological examination, intrauterine fetal death was found, which necessitated premature termination of pregnancy in the 8th gestational week. At the same time the patient was subfebrile and a couple days later she reported fever, reaching up to 39°C. The patient was hospitalized in the Clinic of Rheumatology with a fever of unknown origin up to 39°C, polyarthralgia, polymyalgia, maculopapular rash on the chest, leukocytosis, thrombocytosis, increased LDH-, ferritin-, GGT-acute phase reactant levels, microcytic hypochromic anemia, negative immunological findings (anti-MCV, anti- CCP, ANA-screen, ANA-blot, anti-dsDNA, ANCA, anti-MPO, anti-PR3, aCL, anti-B2GPI, anti-prothrombin), negative Quantiferon test. The patient was diagnosed with AOSD and started therapy with Methylprednisolone 60mg/daily, when she got a persistent afebrile condition and significant improvement in the clinical status and laboratory parameters.

https://doi.org/10.35465/28.4.2020.pp72-80
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References

  1. Gopalarathinam R, Orlowsky E, Kesavalu R, Yelaminchili Sл Adult Onset Still’s Disease: A Review on Diagnostic Workup and Treatment Options. Case Reports in Rheumatology, 2016. https://doi.org/10.1155/2016/6502373
  2. Gündüz E, Güllü MN, Zengin Y, et al. Adult-onset Still’s disease: Case report. Am J Emerg Med, 2014, 5:120-122.
  3. Bywaters EGL. Still’s disease in the adult. Ann Rheum Dis. 1971;30:121-133.
  4. Still G. On a form of chronic joint disease in children. Med Chir Trans. 1897;80:47-60.
  5. Efthimiou P, Georgy S. Pathogenesis and management of adult-onset Still's disease. Semin Arthritis Rheum. 2006, 36:144-152. 10.1016/j.semarthrit.2006.07.001
  6. Magadur-Joly G, Billaud E, Barrier JH 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):587-90.
  7. Masson C, Le Loet X, Liote Fet al. Comparative study of 6 types of criteria in adult Still’s disease. J Rheumatol 1996. Mar;23(3):495-497.
  8. Fautrel B. et al. Proposal for a new set of classification criteria for adult-onset still disease. Medicine (Baltimore) 81:194-200, 2002.
  9. Golchin N, Sharifzadeh M, Fransawy Alkomos M, et al. Adult-onset Still’s Disease in a Female Patient with Schizophrenia: A Case Report and Literature Review. Cureus 2018,10(7): e3019. doi:10.7759/cureus.3019
  10. Mehta B, Efthimiou P. Ferritin in Adult-Onset Still’s Disease: Just a Useful Innocent Bystander?, Int J Inflammation, 2012, https://doi.org/10.1155/2012/298405
  11. Yamaguchi M, Ohta A, Tsunematsu T et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol 1992;19:424-430.
  12. Gerfaud-Valentin M, Maucort-Boulch D, Hot A, et al. Adult-onset still disease: Manifestations, treatment, outcome, and prognostic factors in 57 patients. Medicine. 2014;93:91-99.
  13. Pak S, Pham C, Delay in the Diagnosis of Adult-Onset Still’s Disease. Int Med, University of Toledo Medical Center, 2017; 9(6): e1321. https://dx.doi.org/10.7759%2Fcureus.1321
  14. Wouters JM, Van der Veen J, Van de Putte LB, de Rooij DJ. Adult onset Still’s disease and viral infections. Ann Rheum Dis. 1988;47:764-767.
  15. Perez C, Artola V. Adult Still’s disease associated with Mycoplasma pneumoniae infection. Clin Infect Dis. 2001;32:105-106.
  16. Mavragani CP, Spyridakis EG, Koutsilieris M. Adult-onset Still’s disease: from pathophysiology to targeted therapies. Jun; 2012.
  17. Gordon SC, Lauter CB. Mumps arthritis: unusual presentation as adult Still's disease. Ann Intern Med. 1982 Jul;97(1):45-47.
  18. Dold U, Haelbig C. Mykoplasmose und Still-Syndrom. Gibt es einen ätiologischen zusammenhang? MMW Munch
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