Polyarticular juvenile idiopathic arthritis: Clinical manifestations and diagnosis
- Pamela F Weiss, MD, MSCE
Pamela F Weiss, MD, MSCE
- Assistant Professor
- Department of Pediatrics
- University of Pennsylvania
- Children's Hospital of Philadelphia
Polyarticular juvenile idiopathic arthritis (formerly called polyarticular onset juvenile rheumatoid arthritis [JRA]) is a subset of juvenile idiopathic arthritis (JIA) that is defined by the presence of more than four affected joints during the first six months of illness . In the revised nomenclature, this disease, which comprises 20 to 30 percent of patients with JIA, is included in the group termed "childhood polyarthritis." (See "Classification of juvenile arthritis".)
The clinical manifestations, diagnosis, course, complications, and prognosis of polyarticular JIA are reviewed here. The treatment of polyarticular JIA is discussed separately. (See "Polyarticular juvenile idiopathic arthritis: Treatment".)
Polyarticular juvenile idiopathic arthritis (JIA) is more frequent in females than males. There is a bimodal distribution of the age at onset. The first peak is between the ages of two and five years, and the second is between 10 and 14 years. This age distribution suggests that two or more distinct diseases may be included in this classification. The classification, epidemiology, and immunopathogenesis of JIA are reviewed in greater detail separately. (See "Classification of juvenile arthritis" and "Juvenile idiopathic arthritis: Epidemiology and immunopathogenesis".)
The clinical presentation of polyarticular juvenile idiopathic arthritis (JIA) is varied, although it tends to fall into patterns based upon the age of onset.
Younger children — In children less than 10 years of age, polyarticular JIA often begins similarly to oligoarticular disease, with one or two joints affected . The disease is relentlessly progressing, spreading to involve five or more joints during the first six months after disease onset. There are periods of apparent response to therapy followed by relapses with an increasing number of involved joints. The development of the disease is often indolent. Thus, it may go unrecognized until an intercurrent infection precipitates a dramatic increase in symptoms. This failure to recognize the initial symptoms may make it appear that the disease had a sudden onset and rapid progression.
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