Acute Febrile Juvenile Rheumatoid Arthritis in Adults

Goldman, John A.; Beard, Marvin; Casey, Helen

doi:10.1097/00007611-198005000-00005

Cited by 45 publications

(14 citation statements)

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“…Reported neurologic complications of AOSD include peripheral neuropathy and central nervous system (CNS) abnormalities with seizures, aseptic meningoencephalitis, and brainstem hemorrhage (8). A previous case of fatal status epilepticus has been described (33). Neurologic manifestations are considered rare in SJIA, and some cases have been attributed to drug toxicity (34).…”

Section: Discussionmentioning

confidence: 99%

A Pruritic Linear Urticarial Rash, Fever, and Systemic Inflammatory Disease in Five Adolescents: Adult‐Onset Still Disease or Systemic Juvenile Idiopathic Arthritis sine Arthritis?

Prendiville

Tucker

Cabral

et al. 2004

Pediatric Dermatology

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The characteristic rash of systemic juvenile idiopathic arthritis is a transient erythematous eruption associated with a quotidian spiking fever. Usually asymptomatic, it can be pruritic, with dermatographism at sites of scratching or pressure. An illness similar to this entity in adults is designated adult-onset Still disease. The relationship between the pediatric and adult disease is uncertain and differences in case definition have evolved. Specifically, a sustained arthritis for at least 6 weeks is required for a diagnosis of systemic juvenile idiopathic arthritis, whereas transient arthritis and arthralgia are accepted criteria in adult-onset Still disease. We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption. Intense pruritus and linear erythematous lesions flared with a spiking fever, usually in the late afternoon and evening. Periorbital edema/erythema and nonlinear urticarial lesions were also seen. Two children had splinter hemorrhages of the nail beds and one girl developed a fixed, scaling, pigmented, linear eruption. Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient. Other systemic manifestations included sore throat, transient arthritis, abdominal pain, lymphadenopathy, hepatomegaly, splenomegaly, hyperferritinemia, and hepatic dysfunction. No patient had a sustained arthritis. The course of the disease was variable. One patient, diagnosed with macrophage activation syndrome, recovered on oral naproxen. Two patients responded to systemic corticosteroid therapy. One girl developed status epilepticus and died from aspiration and asphyxia. A boy with severe hepatitis developed renal failure and thrombotic thrombocytopenic purpura and was treated with plasmapheresis, dialysis, and systemic corticosteroids; he had recurrent episodes of rash and fever into adult life. These children did not fulfill the case definition of systemic juvenile idiopathic arthritis because they lacked a persistent arthritis. Adolescent and adult patients with the same clinical and laboratory findings are described under the rubric of adult-onset Still disease. Recognition of the distinctive urticarial skin eruption and spiking fever is important in the diagnosis of a disease with severe morbidity and potentially life-threatening complications.

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Section: Discussionmentioning

confidence: 99%

A Pruritic Linear Urticarial Rash, Fever, and Systemic Inflammatory Disease in Five Adolescents: Adult‐Onset Still Disease or Systemic Juvenile Idiopathic Arthritis sine Arthritis?

Prendiville

Tucker

Cabral

et al. 2004

Pediatric Dermatology

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“…While the presenting features and clinical manifestations of adult-onset Still's disease (AOSD) have been elucidated, deficiencies still exist in our knowledge of diagnosis, therapy, clinical course, and eventual prognosis. Although early reports (1)(2)(3)(4) attested to the overall benign course of AOSD, some authors (5-8) have questioned this claim. Elkon et a1 (8) recently termed AOSD a "chronic and relapsing disorder with greater morbidity than originally described."…”

mentioning

confidence: 99%

Adult‐onset still's disease

Cush

Medsger

Christy

et al. 1987

Arthritis & Rheumatism

306

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We reviewed the long-term natural history of 21 adult-onset Still's disease patients. Patient subsets were identified according to clinical course patterns. These included monocyclic systemic disease in 4, polycyclic systemic disease in 2, chronic articular monocyclic systemic disease in 10, and chronic articular polycyclic systemic disease in the remaining 5 patients. Functional outcome differed according to course patterns and the extent of articular involvement. Systemic manifestations, per se, did not contribute to poor functional prognosis. Chronic articular disease had the worst outcome: 27% evolved to functional class 111 status, compared with none in the cyclic systemic groups. Those patients who had a chronic articular pattern or a polyariticular onset and course were at higher risk to develop disabling arthritis. An aggressive approach to therapy, including the early use of remittive agents, should be considered in these patient subsets. _____

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“…Several sets of classification criteria have been developed from retrospectively analyzed data 3,4,5,6,7,8,9,10 (see also Table 1), which points to the difficulty of identifying unambiguous criteria.…”

Section: Scoring Adult Onset Still's Diseasementioning

confidence: 99%