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Keywords:

  • adult-onset Still's disease;
  • DMARD ;
  • methotrexate

Abstract

Background

Adult-onset Still's disease (AOSD) is a rare chronic inflammatory disorder presenting with prolonged fever and polyarthritis.

Methods

Retrospective study of patients with AOSD, seen between 1992 and 2009 at a large tertiary care hospital.

Results

Twenty-nine patients (18 female) with median age at onset of 28 (17–58) years were seen. The clinical features included fever in 29, inflammatory polyarthritis in 26, sore throat in eight and typical rash in 13. Lymphadenopathy was present in 15, hepatomegaly in 15, splenomegaly in 13 and serositis in five patients. Anemia was present in 22, neutrophilic leukocytosis in 28 and thrombocytosis in 13 patients. Acute phase reactants were elevated in all. Fifteen patients had transaminitis. Low titer antinuclear antibodies were present in 6/28 patients. On median follow-up (25 patients) of 23.7 months (range: 3–84) one patient had self-limited or monocyclic pattern, eight had polycyclic and 16 had chronic articular pattern. All patients received non-steroidal anti-inflammatory drugs and 25 received methotrexate and/or prednisolone. During the course 14 patients had remission and of these six were in remission on drugs at last follow-up. One patient received tociliziumab and was in clinical remission. One patient developed macrophage activation syndrome and one had atlanto-axial dislocation. Three patients developed tuberculosis and two died of infection associated with immunosuppression.

Conclusion

AOSD is an uncommon disorder with 1–2 patients seen at a large tertiary care rheumatology unit. Overall AOSD has a fair outcome with significant morbidity and most needing long-term therapy with steroids and methotrexate.