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Adult-onset Still's disease

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Abstract

Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology and pathogenesis. AOSD is a rare condition, usually presenting with high fever accompanied by systemic manifestations. The disease is a heterogeneous pathological entity with a range of etiologies, manifestations and prognosis.

There is no single diagnostic test for AOSD; rather, the diagnosis is based upon clinical criteria such as arthralgia, fever, skin rash, lymphadenopathy, and hepatosplenomegaly. Determination of the procalcitonin level and the biological response to empirical corticosteroid therapy generally helps the diagnosis, while immune-serology, as a ‘screening’ test, will not add meaningful information in most cases. Treatment consists of anti-inflammatory medications. Non-steroid anti-inflammatory drugs have limited efficacy, corticosteroid therapy and disease-modifying antirheumatic drugs are usually required. Novel therapeutic approaches, such as anti-tumor necrosis factor blockade and stem cell transplantation, are promising. In this chapter we present clinical and laboratory parameters of 18 patients diagnosed with AOSD at our institution between 1997 and 2003, and review the literature.

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

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