Adult-Onset Still's Disease: A Review

Abstract

Adult-onset Still's disease (AOSD) is an uncommon inflammatory condition characterised by high fever, leukocytosis with neutrophilia, arthralgia and skin rash. Diagnosis is often clinical, and it is always necessary to exclude common disease mimics such as infections, malignancies and other rheumatic diseases. Ferritin and glycosylated ferritin are useful adjuncts to aid in the diagnosis of AOSD. Important complications to consider include fulminant hepatitis, disseminated intravascular coagulopathy and hemophagocytic syndrome. Mainstay treatment of AOSD is often with glucocorticoids. Other agents that can also be used include methotrexate, and biologic therapy, such as tumor necrosis factor-α (TNF-α) blockers and interleukin-1 (IL-1) inhibitors. A retrospective review of patients diagnosed with AOSD admitted to Singapore General Hospital (SGH) was performed, and their disease characteristics and laboratory findings were compared with that of previous published cohorts.

Adult-Onset Still’s Disease in an Adult Nigerian Male - A Case Report

“…9 Several infectious agents have been noted to be involved in the aetiology of AOSD; these include viruses like parvovirus, cytomegalovirus, human immunodeficiency virus, rubella virus, Epstein-Barr virus, Hepatitis B and C viruses, and bacteria like Yersinia enterocolitica, Chlamydia pneumoniae, and Mycoplasma pneumoniae. 3,10,11 It has been associated with Miller-Fisher syndrome and reported to occur as a paraneoplastic syndrome. 12,13 The pathogenesis of AOSD is still unclear.…”

“…14 Regarding the pathogenesis of AOSD, the possible role of T-helper (Th) cells has been postulated and no familial trend has been described. 3,15 The hallmark of the pathogenesis of AOSD is macrophage and neutrophil activation. 16 This is mediated by cytokines, mainly tumour necrosis factor-alpha (TNF-α), interleukin (IL) 1 family (IL-1β and IL-18) IL-6, and IL-8.…”

“…Airenakho Emorinken 1 , Folasade Ojuolape Agbebaku 2 , Mercy Ofunami Dic-Ijiewere 3 , Blessyn Omoye Akpasubi 4 , Asuwemhe Johnson Ugheoke 5 1, 2, 3, 4, 5 Department of Medicine, Irrua Specialist Teaching Hospital, Irrua, Edo State, Nigeria.…”

“…1 Bywaters in 1971 was the first to describe this clinical entity. 2,3 Its estimated annual incidence is 0.16 per 100,000 persons. 4 AOSD has a bimodal pattern of age distribution with peaks at 15 -25 and 36 -46 years.…”

Cardiovascular Disease Risk Associated to Chronic Inflammatory Rheumatic Diseases in Patients Seen in Rheumatology Unit in Yaounde, Cameroon

“…CIRD patients enrolled in this cross-sectional study are members of a large cohort designed for all aspects of research in the domain of CIRD at the Yaounde Central Hospital rheumatology unit. The cohort comprises a large array of CIRD that were all included in this research: 1) RA diagnosed according to the American College of Rheumatology (ACR)/European League against Rheumatism (EULAR) criteria [10] [11], 2) systemic lupus erythematosus (SLE) and 3) systemic sclerosis both diagnosed with respect to their respective ACR criteria [12] [13], 4) mixed connective tissue disease fulfilled the Sharp criteria [14], 5) Sjögren's syndrome (SS) diagnosed as recommended by the American-European consensus group criteria [15], 6) autoimmune polymyositis fulfilled the Hoogendjick et al criteria [16], 7) spondyloarthropathies diagnosed according to the Amor et al criteria [17], 8) and adult onset Still's disease diagnosed according to the Fautrel et al criteria [18]. Individuals of both sexes aged ≥ 18 years all members of the cohort were recruited consecutively based on a non-probabilistic sampling and included in the CIRD group.…”