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      Adult-Onset Still’s Disease: Typical Presentation, Delayed Diagnosis

      case-report
      1 , , 1 , 1 , 1 , 1
      ,
      Cureus
      Cureus
      adult-onset still’s disease, fever, rash, arthritis

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          Abstract

          Adult-onset Still’s disease (AOSD) is an uncommon auto-inflammatory disease of unknown etiology, with a classical triad of fever, arthritis, and evanescent rash. Its low prevalence and lack of specific guidelines contribute to frequent delays in diagnosis and treatment. Clinical manifestations vary greatly between mainly systemic or articular symptoms and the clinical pattern between monocyclic, polycyclic, or chronic illness. Treatment options include non-steroid anti-inflammatory drugs (NSAIDs), systemic corticoids, disease-modifying anti-rheumatic drugs (DMARDs), and, more recently, biological agents directed at identified immune pathological pathways like anti-interleukin-1 (IL-1) or anti-tumor necrosis factor-alpha (TNF-alpha).

          We report a case of a 40-year-old male with persistent fever, polyarthralgia, sore throat, and rash for two weeks despite antibiotic treatment for suspected bacterial pharyngitis. During hospitalization and after extensive diagnostic workup, an AOSD diagnosis was made according to Yamaguchi’s criteria and successfully managed with systemic corticoids.

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          Most cited references11

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          Still's disease in the adult.

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            Epidemiology of adult Still's disease: estimate of the incidence by a retrospective study in west France.

            To estimate the incidence of adult Still's disease (ASD) and to specify, if possible, associated factors. A retrospective study of the populations of the Brittany and Loire regions in west France was made from 1 January 1982 to 31 December 1991. All internal medicine and rheumatology practitioners of these regions were consulted. Sixty-two (62) cases were reported (93% response). The disease incidence calculated over five years was 0.16 per 100,000 inhabitants in the study population. There was no sex bias (sex ratio 1.06 in ASD v 1.05 in the overall population. The mean age of the study population was 36 years, with two peaks of distribution at 15-25 and 36-45 years. A history of allergy was present in 23% of patients (n = 14). In two patients, it was possible to correlate an environmental allergen to exacerbation of ASD. The yearly incidence of ASD was estimated to be 0.16 per 100,000 inhabitants. However, it was not possible to incriminate any infectious, toxic, or genetic factors in exacerbation of the disease.
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              Diagnostic value of ferritin and glycosylated ferritin in adult onset Still's disease.

              To determine the usefulness of serum ferritin and glycosylated ferritin (GF) levels in diagnosing adult onset Still's disease (AOSD). We performed a retrospective multicenter study of 205 patients who had ferritin and GF assays in one hospital laboratory. Records of all patients were reviewed, and a standardized questionnaire used to extract all data available at the time of the assay. The clinicians' final diagnosis was also recorded. Patients were classified as having "certain AOSD" (based on Yamaguchi's criteria) or a control disease. The concordance of ferritin and GF levels with final diagnosis was evaluated. In total 49 AOSD and 120 control patients were eligible. The mean ferritin value was significantly higher in the AOSD group (4,752 +/- 9,599 microg/l) than in the control group (1,571 +/- 3,807 microg/l), p = 0.029. GF was significantly lower in AOSD patients (15.9 +/- 11.9%) than in the control group (31.5 +/- 18.7%), p < 0.001. The combination of a GF level of < or = 20% with ferritin above the upper limit of normal yielded a sensitivity of 70.5% and specificity of 83.2%. The combination of a GF level < or = 20% with ferritin 5 times normal produced a sensitivity of 43.2% and specificity of 92.9%. This latter combination allowed an AOSD diagnosis to be ruled out for 6 of the 8 control patients who met Yamaguchi's positive criteria. Ferritin and GF levels are powerful diagnostic markers of AOSD. They may be helpful in clinical practice for excluding differential diagnoses.
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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                8 June 2020
                June 2020
                : 12
                : 6
                : e8510
                Affiliations
                [1 ] Internal Medicine, Hospital Senhora Da Oliveira, Guimarães, PRT
                Author notes
                Article
                10.7759/cureus.8510
                7346332
                312574cd-b8f9-45a2-bd34-f9959a805ffd
                Copyright © 2020, Seco et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 5 May 2020
                : 8 June 2020
                Categories
                Internal Medicine
                Allergy/Immunology

                adult-onset still’s disease,fever,rash,arthritis
                adult-onset still’s disease, fever, rash, arthritis

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