Adult-Onset Still’s Disease: A Case Report

Abstract

Adult-onset Still's disease (AOSD) is a rare clinical entity with unknown etiology, characterized by arthritis, fever, evanescent rash and other systemic presentations. This report described a 45-year-old male who presented with sore throat, fever, arthritis, evanescent rash, raised liver enzymes and hyperferritinemia. He was diagnosed to have AOSD based on Yamuguchi criteria after the exclusion of other potential diagnoses. The patient partially responded to combined celecoxib and prednisolone. He was also prescribed methotrexate and his symptoms improved.

Paving a Shorter Path Towards Diagnosis: A Case on Adult Onset Still’s Disease from Pakistan

“…Three broad patterns of AOSD have been described: monocyclic with complete remission, which was seen in our case; polycyclic with recurrence of systemic and articular flares, separated by periods of remission; and a chronic pattern, most prone to joint destruction [ 12 ]. Alternately, the disease is classified into two subtypes—the rheumatoid arthritis (RA) subtype with severe articular involvement and the non-RA subtype, characterized by macrophage activation and systemic manifestations.…”

“…This disparity may very well be due to the fact that significantly lesser females seek medical care in the Third World countries in the region, thus counting for unreported cases and misrepresented statistics. The pathogenesis of the disease remains unclear, but its correlation with several cytokines like tumor necrosis factor-alpha (TNF-α), interleukin (IL)-6, and IL-18 has been established, along with the collective role of genetic, infectious, and environmental factors in its course—leading to its reclassification as a polygenic autoinflammatory disorder in the past decade [ 12 ].…”

“…Moreover, the most promising laboratory investigation to date—the glycosylated ferritin—has been useful but not definitive, as it is also a common feature of malignancy and infection and only 43.2% sensitive. The disproportionate increase in ferritin levels is thought to be due to cytokine secretion induced by the reticuloendothelial system or hepatic damage, although most cases present with increased levels, without any evident liver damage [ 12 ]. As research on AOSD continues, other novel biomarkers with diagnostic potential continue to emerge.…”

Atypical Skin Rash in a Patient with Adult-Onset Still's Disease: A Case Report

“…Several recent reports have described the successful use of disease-modifying anti-rheumatic drugs (DMARDs) in AOSD. 15,16 In the present study, we used methotrexate and prednisolone combination therapy, to which the patient responded well.…”

A Case of Adult-Onset Still's Disease with Positive Antinuclear Antibodies

“…The urticarial rash is a typical feature seen in ASOD, which was also present in this case. Apart from a sore throat, there was no lymphadenopathy, splenomegaly, or nervous system involvement, which might also be seen in case of ASOD [ 7 ].…”