STILL’S DISEASE IN A DULTS (CLINICAL CASES)

Objective: to analyse and describe clinical cases of Still’s disease in adults and peculiarities of therapy in patient with a monocyclic course of the disease who had at the beginning of the observation a high systemic account (12) and in patient with a recurrent course of the disease with a positive result for 25 and 10 years of a dynamic observation.

Materials and methods. Patient L., a female, 35 years old, was taken to the therapeutic department of the clinic with fever of 38–39 °С, chills and sweating, resistant not itching rash, lymphadenopathy, signs of myocarditis, loss of 20 kg of weight, exudative pleurisy, pronounced orthopnea with respiration rate of 40–48, tachycardia up to 130 beats per minute, myalgia, hepatosplenomegaly and abdominal pains. In past medical history for 2 months of the disease the patient was examined, and the sings of disease progression were present. The clinical constellation in patient L. allowed us to formulate a diagnosis of Still»s disease. Some investigations were conducted repeatedly to track the changes. The following therapy by Indomethacin in a daily dose of 150–200 mg/day for 1 year and for 6 months in combination with D-penicillamine in a dose of 450 mg/day induced remission. Till the end of 2016 the disease recurrence was not revealed. In another patient B., a male of 57 years old, the disease manifested with sub-febrile temperature, pain in a testicular area, thrombocytosis, and the symptoms increased in 2 weeks and included resistant febrile temperature, lymphadenopathy, changes of laboratory tests, signs of nephropathy and encephalitis. The patient was examined to exclude oncology.

Results. Still’s disease was diagnosed and the following therapy was administrated: diclofenac and methotrexate for 4 years with episodes of disease recurrence in case of decreasing the doses of the medications, but in future the therapy managed to cancel, and there was no recurrence observed.

Conclusion. These clinical cases have demonstrated peculiarities of course, diagnostic and management of patients with Still’s disease, the possibility of prescribing non-steroidal anti-inflammatory drugs and synthetic basic anti-inflammatory drugs for achievement of persistent disease remission.

prolonged fever, differential diagnosis, orphan diseases, Still’s disease in adults, therapy of Still’s disease in adults, prospective observation for 25 years associated with remission, prospective observation for 10 years with a recurrent course, monocyclic variant of clinical course of Still’s disease, recurrent variant of clinical course of Still’s disease

1. Lebedev A. A. Rare diseases: problems and perspectives of solution. Zemskiy vrach = County Doctor 2011;6(10):5–9. (In Russ.).

2. Bywaters E. G. Still’s disease in the adult. Ann Rheum Dis 1971;30(2):121–33.

3. Ohta A., Yamaguchi M., Kaneoka H. et al. Adult Still’s disease: review of 228 cases from literature. J Rheumatol 1987;14(6):1139–46.

4. Bannatyne G. A., Wohlmann A. S. Rheumatoid arthritis: its clinical history, etiology and pathology. Lancet 1896;(1):1120–5.

5. Magadur-Joly G., Billaud E., Barrier J. H. 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.

6. Wakai K., Ohta A., Tamakoshi A. et al. Estimated prevalence and incidence of adult Still’s disease; finding by a nationwide epidemiological survey in Japan. J Epidemiological 1997;7(4):221–5.

7. Ohta A., Yamaguchi M., Tsunematsu T. et al. Adult Still’s disease: a multicenter survey of Japanese patients. J Rheumatol 1990;17(8):1058–63.

8. Wouters J. M., Reekers P., van de Putte L. B. Adult-onset Still’s disease. Disease course and HLA associations. Arthr Rheumat 1986;29(3):415–8.

9. Wouters J. M., van de Putte L. B. Adultonset Still’s disease; clinical and laboratory features, treatment and progress of 45 cases. Quart J Med 1986;61(235):1055–65.

10. Pouchot J., Sampalis J. S., Beaudet F. et al. Adult Still’s Disease: Manifestations, Disease Course, and Outcome in 62 patients. Medicine (Baltimore) 1991;70(2):118–36.

11. Yamaguchi M., Ohta A., Tsunematsu T. et al. Preliminary criteria for classification of adul Still’s disease. J Rheumatol 1992;19(3):424–30.

12. Reginato A. J., Schumacher H. R., Baker D. G. et al. Adult onset Still’s disease: experience in 23 patients and literature review with emphasis on organ failure. Semin Arthritis Rheum 1987;17(1):39–57.

13. Fautrel B., Zing E., Goldmart J. L. et al. Proposal for a new set of classification criteria for adult-onset Still disease. Medicine (Baltimore) 2002;81(3):194–200.

14. Franchini S., Dagna L., Salvo F. et al. Adult onset Still’s disease: clinical presentation in a large cohort of Italian patients. Clin Exp Rheumatol 2010;28(1):41–8. PMID: 20346237.

15. Lee S. W., Рark Y. B., Song J. S., Lee S. K. The mid-range of the adjusted level of ferritin can predict the chronic course in patients with adult onset Still’s disease. J Rheumatol 2009;36(1):156–62.

16. Ruscitti P., Cipriani P., Masedu F. et al. Adult-onset Still’s disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers. BMC Medicine 2016;14(1):194. DOI: 10.1186/s12916‑016‑0738‑8.

17. Cush J. J. Adult-onset Still’s disease. Bull Rheum Dis 2000;49(6):1–4.

18. Nemcinov E. N., Kanevskaya M. Z., Yankin V.V. Adultonset Still’s disease: diagnostic criteria, variants of progression, clinical observations. In: Current problems of internal medicine and teaching. Moscow: MMA imeni I. M. Sechenova, 2002: 214–221. (In Russ.).

19. Kong X. D., Zhang W., Zhao Y., Zhang F. Clinical features in adult-onset Still’s disease: a study of 104 cases. Clin Rheumatol 2010;29(9):105–9. DOI: 10.1007/s10067‑010‑1516‑1.

20. Guilpain P., Le Quelles A. About the complexity of adult onset Still’s disease and advances still required for its management. BMC Med 2017;15(1):5. DOI: 10.1186/s12916‑016‑0769‑1.

21. Masson C., Le Loët X., Liote F. et al. Adult Still’s disease: part I. Manifestations and complications in sixty-five cases in France. Rev Rhum Engl Ed 1995;62(11):748–57.

22. Evensen K. J., Nossent H. C. Epidemiology and outcome of adult Still’s disease in northern Norway. Scand J Rheumatol 2006;35(1):48–51.

23. Tristano A. G. Macrophage activation syndrome: a frequent in patients with adult Still’s disease. Med Sci Monit 2008;14(3):RA27–36.

24. Masyama A., Kobayashi H., Kobayach Y. et al. A case of adult-onset Still’s disease complicated by thrombotic thrombocytopenic purpura with retinal microangiopathy and rapidly fatal cerebral edema. Mod Rheumatol 2013;23(2):379–85.

25. Calabro J. J., Cruess R. L., Levinson J. E. et al. Juvenile rheumatoid arthritis. Patient Care 1977;(11):70–116.

26. Bujak J. S., Aptekar R. G., Decker J. L., Wolff S. M. Juvenile rheumatoid arthritis presenting in the adult as fever of unknown origin. Medicine(Baltimore) 1973; 52(5):431–44.

27. Shojania K., Chalmers A., Rangno K. Cyclosporin A in treatment of adult Still’s disease. J Rheumatol 1995;22(7):1391–2.

28. Cefle A. Leflunomide and azathioprine combination in refractory adult Still’s disease. Ann Pharmacother 2005;39(4):764–7.

29. Jamilloux Y., Gerfaud-Valentin M., Henry T., Seve P. Treatment of adult-onset Still’s disease: a review. Ther Clin Risk Manag 2014;11:33–43. DOI: 10.2147/TCRM.S64951.

30. Elkon K. B., Hughes G. R., Bywaters E. G. et al. Adult onset Still’s disease: Twentyyear follow-up and further studies of patients with active disease. Arthr Rheum 1982;25(6):647–54.

31. Muravjev Yu. V. Adult-onset Still’s disease. Russian clinical guidelines. Rheumatology. Ed.: acad. E. L. Nasonov. Moscow: GEOTARMedia, 2017. 464 р. (In Russ.).

32. Hochino T., Ohta A., Yang D. et al. Elevated serum interleukin 6, interferongamma, and tumor necrosis factor-alpha levels in patients with adult Still’s disease. J Rheumatol 1998;25(2):396–8.

33. Kotter I., Wacker A., Koch S. et al. Anakinra in patients with treatmentresistant adult-onset Still’s disease: four case report with serial cytokine measurements and a review of the literature. Semin Arthr Rheum 2007;37(3):189–97.

34. Ogata A., Kitano M., Yamanaka J. et al. Interleukin 18 and hepatocyte grown factor in fulminant hepatic failure of adult onset Still’s disease. J Rheumatol 2003;30(5):1093–6.

35. Kontzias A., Efthimiou P. Adult-onset Still’s disease pathogenesis, clinical manifestations and therapeutic advances. Drugs 2008;68(3):319–37.

36. Muravjev Y. V., Nasonov E. V. Adult-onset Still’s disease. Nauchno-prakticheskaya revmatologiya = Scientific and Practical Rheumatology 2010;2:58–65 (In Russ.).