3Adult-onset Still's disease
Section snippets
Epidemiology and etiology
The mean age of patients with adult-onset Still's disease (AOSD) is 38.1 years, with 67% of the cases presenting over the age of 35. Women are more frequently affected (65–70%); incidence over the age of 16 among men is 0.22/10 ,000 and among women 0.34/100 000.1 The etiology and pathogenesis of the disease is not fully understood. Observations supporting the role of genetic predisposition, as well as of environmental influence, have both been published. The association with HLA types is much
Clinical signs
The frequency of individual symptoms is shown in Table 2. These include arthritis and arthralgia; however, their presence is often less evident at the onset than later during the course of the disease. It is rare that AOSD will present with arthritis prior to the onset of other systemic and extra-articular manifestations. Morning stiffness, myalgia and arthralgia dominate the early clinical picture. At first, synovitis may be fleeting, migratory or additive. Peripheral arthritis (including
Diagnosis
According to Cush, ‘AOSD remains a painstakingly difficult clinical diagnosis, largely because of its rarity, protean manifestations and a lack of pathognomonic features or diagnostic tests’.3 The diagnosis is established by diagnostic criteria. There are at least six classifications; Yamaguchi's criteria appear to be the most sensitive (93.5%), followed by those of Calabro and Londino. Medsger et al (80.6%), Kahn (69.3%), Reginato (55.2%), and Goldman (43.7%).7 Yamaguchi's criteria are shown
Risk factors
Only the role of stress has been proven; there is no correlation with smoking, alcohol drinking, vaccination, transfusion, surgical intervention, pregnancy or previous diet. No connection has been shown with tonsillectomy, adenoidectomy, appendectomy, asthma, rhinitis allergica or dust inhalation.22
Course
The course can be monocyclic (61.5%) or relapsing (38.5%); there is a chronic articular involvement in a third of the cases.3 If the proximal part of the extremity is inflamed, the course is generally chronic. Other isolated arthralgia might predict a relapsing course. Serositis is rare in the chronic forms. The inflamed wrists often herald systemic disease.14 Radiological alteration can be seen in 50% of the affected joints after one year.
The disease is coloured by several rare symptoms which
Treatment
Aspirin or non-steroid anti-inflammatory drugs (NSAIDs) are usually not effective enough in cases of high fever. In 88% of the cases corticosteroid administration is needed. Unfortunately, low doses of prednisone (i.e. <20 mg/day) and alternate-day therapy have proved ineffective in most patients. In order to reduce the minimal effective steroid dose, disease-modifying antirheumatic drug (DMARD) therapy is recommended: mainly methotrexate42 or sulphasalazine.43 The former is applied in a dose
Prognosis
The median disease duration is 10 years, i.e. half of the patients need treatment for more than 10 years. Somatic and psychosocial handicap and prolonged pain exist, but complaints are not as permanent as in other rheumatic diseases. There was no difference in education, work success, off-work days or financial income of the patients.50
Summary
We can conclude that in patients presenting with fever of unknown origin one has to consider AOSD more often (Table 8). The definition is like in ‘Le Petit Prince’ by Saint Exupery: scilicet, we seem to know better things we name. It is considered to be a different disease entity from the original conceptual category of juvenile rheumatoid arthritis. As a systemic disease, AOSD is closely related to rheumatoid arthritis3, and is likely to be part of a continuum of the better-known adult
References (50)
- et al.
A controlled study of the long-term prognosis of adult Still's disease
American Journal of Medicine
(1995) - et al.
Severe systemic inflammatory response syndrome with shock and ARDS resulting from Still's disease: clinical response with high-dose pulse methylprednisolone therapy
Chest
(1999) - et al.
A controlled study of the long-term prognosis of adult Still's disease
American Journal of Medicine
(1995) - et al.
Estimated prevalence and incidence of adult Still's disease: findings by a nationwide epidemiological survey in Japan
Journal of Epidemiology
(1997) - et al.
La maladie de Still de l'adulte II
Reviews of Rheumatology
(1995) - Cush JJ. Adult-onset Still's disease-a circadian syndrome?...
- et al.
Association between HLA-DR B1 and clinical features of adult onset Still's disease in Korea
Clinical Experimental Rheumatology
(2003) - et al.
La maladie de Still de l'adulte I
Reviews of Rhumatology
(1995) - et al.
Comparative study of 6 types of criteria in adult Still's disease
Journal of Rheumatology
(1996) - et al.
Preliminary criteria for classification of adult Still's disease
Journal of Rheumatology
(1992)
Bone marrow findings in patients with adult Still's disease
Scandinavian Journal of Rheumatology
Diagnostic value of ferritin and glycosylated ferritin in adult onset Still's disease
Journal of Rheumatology
Percentage of glycosylated serum ferritin remains low throughout the course of adult onset Still's disease
Annals of Rheumatic Diseases
Destructive lymphadenopathy and T-lymphocyte activation in adult-onset Still's disease
British Journal of Rheumatology
Lymph node lesion in adult-onset Still's disease resembling peripheral T-cell lymphoma: a report of three cases
International Journal of Surgical Pathology
Textbook of Rheumatology
Adult onset Still's disease associated hemophagocytosis
Journal of Rheumatology
Bone scintigraphy and magnetic resonance imaging in adult-onset Still's disease
Scandinavian Journal of Rheumatology
TCR gamma delta+T cells in peripheral blood of patients with adult Still's disease
Journal of Rheumatology
Different articular outcomes of Still's disease in Chinese children and adults
Clinical Rheumatology
Cytokines in juvenile rheumatoid arthritis
Current Opinion in Rheumatology
Levels of interleukin-18 and its binding inhibitors in the blood circulation of patients with adult-onset Still's disease
Arthritis et Rheumatism
Association between adult-onset Still's disease and interleukin-18 gene polymorphisms
Genes and Immunity
Risk factors for adult Still's disease
Journal of Rheumatology
Adult onset Still's disease with hemophagocytic syndrome and severe liver dysfunction
Hepatological Researches
Cited by (85)
Efficacy and safety of canakinumab in the treatment of adult-onset Still's disease: A systematic review
2021, Seminars in Arthritis and RheumatismCitation Excerpt :Previous studies have associated predisposition to AOSD with different polymorphisms in the genes encoding human leukocyte antigen (HLA), interleukin (IL)-6, IL-18, macrophage inhibitory factor (MIF), and serum amyloid A [13–21]. Likewise, it has been suggested that viral and bacterial infections could play an important role as triggers of this disease [22–27]. Regarding immunopathogenesis, different cell populations of both innate and adaptive immunity are involved, as well as their receptors and inflammatory mediators.
Adult-onset Still's disease
2016, Medicina ClinicaAdult-onset Still's disease: Advances in the treatment
2016, Best Practice and Research: Clinical RheumatologyPathophysiology, subtypes, and treatments of adult-onset Still's disease: An update
2015, Revue de Medecine InterneSeizing the clinical presentation in adult onset Still's disease. An extensive literature review
2015, Autoimmunity ReviewsAdult-onset Still's disease
2014, Autoimmunity Reviews