Adult-onset Still’s Disease as a Differential Diagnosis in Prolonged Fever: Diagnosis and Treatment Experience

Authors

  • Felix F Widjaja Department of Internal Medicine, Faculty of Medicine Universitas Indonesia – Cipto Mangunkusumo Hospital, Jakarta, Indonesia
  • Diah Martina Department of Internal Medicine, Faculty of Medicine Universitas Indonesia – Cipto Mangunkusumo Hospital, Jakarta, Indonesia
  • Soroy Lardo Department of Internal Medicine, Indonesia Army Central Hospital Gatot Soebroto, Jakarta, Indonesia
  • Suryo Anggoro K Wibowo Department of Internal Medicine, Faculty of Medicine Universitas Indonesia – Cipto Mangunkusumo Hospital, Jakarta, Indonesia

Keywords:

adult onset Still's disease, prolonged fever, diagnosis, treatment

Abstract

Adult onset Still’s disease is a rare systemic disease that may involve many organs and may mimick many disease such as infection, autoimmune disease, and also malignancy. The diagnostic approach and treatment strategies have not been well established due to its rarity; however, there are some diagnostic criteria that may help. We present a case of 36-year old man who experienced high prolonged fever which firstly thought as infection. He also had unilateral wrist and knee joint pain and maculopapular rash. Laboratory examination showed high leukocytes count with elevated polymorphonuclear neutrophil count, high platelet count, high ferritin level, and negative results of many infection markers (typhoid antibody, procalcitonin, malaria test, blood culture, urine culture, IgM pneumonia, ASTO, syphilis test, antiHIV, HBsAg, antiHCV, etc). Chest X-ray, joint X-ray, ultrasonography, and echocardiography showed normal result. The patient was then diagnosed with Adult-onset Still’s disease and received intravenous methylprednisolone and the fever was disappeared in 3 days. Six months later the arthralgia appeared again, methotrexate was administered and the pain was then relieved.

References

Ruscitti P, Cipriani P, Di Benedetto P, et al. Increased level of H-ferritin and its imbalance with L-ferritin, in bone marrow and liver of patients with adult onset Still’s disease, developing macrophage activation syndrome, correlate with the severity of the disease. Autoimmun Rev. 2015;14:429-37.

Hersch EC, Oh RC. Prolonged febrile illness and fever of unknown origin in adults. Am Fam Physician. 2014;90:91-6.

Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Seve P. Adult-onset Still’s disease. Autoimmun Rev. 2014;13:708-22.

Maria AT, Le Quellec A, Jorgensen C, Touitou I, Riviere 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;13:1149-59.

Narula N, Narula T, Abril A. Seizing the clinical presentation in adult onset Still’s disease. An extensive literature review. Autoimmun Rev. 2015;14:472-7.

Fautrel B. Adult-onset Still disease. Best Pract Res Clin Rheumatol. 2008;22:773-92.

Cush JJ, Medsger TA, Jr., Christy WC, Herbert DC, Cooperstein LA. Adult-onset Still’s disease. Clinical course and outcome. Arthritis Rheum. 1987;30:186-94.

Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol. 1992;19:424-30.

Fautrel B, Zing E, Golmard JL, et al. Proposal for a new set of classification criteria for adult-onset still disease. Medicine (Baltimore). 2002;81:194-200.

Bywaters EG. Still’s disease in the adult. Ann Rheum Dis. 1971;30:121-33.

Mahroum N, Mahagna H, Amital H. Diagnosis and classification of adult Still’s disease. J Autoimmun. 2014;48-49:34-7.

Gewitz MH, Baltimore RS, Tani LY, et al. Revision of the Jones criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation. 2015;131:1806-18.

Oba Y, Watanabe H, Nishimura Y, et al. A Case of Adult-Onset Acute Rheumatic Fever With Long-Lasting Atrioventricular Block Requiring Permanent Pacemaker Implantation. Int Heart J. 2015;56:664-7.

Delevaux I, Andre M, Colombier M, et al. Can procalcitonin measurement help in differentiating between bacterial infection and other kinds of inflammatory processes? Ann Rheum Dis. 2003;62:337-40.

Kalyoncu U, Buyukasik Y, Akdogan A, et al. Increased serum vitamin B12 levels are associated with adult-onset Still’s disease with reactive macrophage activation syndrome. Joint Bone Spine. 2010;77:131-4.

Colina M, Zucchini W, Ciancio G, Orzincolo C, Trotta F, Govoni M. The evolution of adult-onset Still disease: an observational and comparative study in a cohort of 76 Italian patients. Semin Arthritis Rheum. 2011;41:279-85.

Efthimiou P, Georgy S. Pathogenesis and management of adult-onset Still’s disease. Semin Arthritis Rheum. 2006;36:144-52.

Roy SG, Karunanithi S, Dhull VS, Bal C, Kumar R. (1)(8)F-FDG PET/CT aids the diagnosis of adult onset Still’s disease in a patient with fever of unknown origin. Rev Esp Med Nucl Imagen Mol. 2014;33:392-3.

Pouchot J, Arlet JB. Biological treatment in adult-onset Still’s disease. Best Pract Res Clin Rheumatol. 2012;26:477-87.

Downloads

Published

2019-08-05

How to Cite

Widjaja, F. F., Martina, D., Lardo, S., & Wibowo, S. A. K. (2019). Adult-onset Still’s Disease as a Differential Diagnosis in Prolonged Fever: Diagnosis and Treatment Experience. Acta Medica Indonesiana, 51(2), 158. Retrieved from https://www.actamedindones.org/index.php/ijim/article/view/190

Issue

Vol. 51 No. 2 (2019): Acta Medica Indonesiana

Section

CASE REPORT

License

Copyright

The authors who publish in this journal agree to the following requirements:

  1. Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a  Creative Commons Attribution 4.0 International License (CC BY 4.0) that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
  2. Authors can enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
  3. Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) before and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work. (See The Effect of Open Access)

Privacy Statement

The names and email addresses entered in this journal site will be used exclusively for the stated purposes of this journal and will not be made available for any other purpose or to any other party.