Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome: a review of the literature and a report of three cases.

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome is a disease that commonly produces symmetrical synovitis and swelling of both the upper and lower extremities. It generally involves the wrists, hands, feet, and ankles of the affected individual. This syndrome most often resembles that of polymyalgia rheumatica and rheumatoid arthritis and usually affects elderly Caucasian males. Serological testing is typically negative except for a mild to moderate elevation of the erythrocyte sedimentation rate. The HLA-B7 phenotype is present in approximately 50% of patients with this syndrome. Treatment of RS3PE syndrome is heralded by the predictable response to low-dose corticosteroid or hydroxychloroquine therapy. There has been no previous mention of this condition in the podiatric literature. Presented below is a review of this syndrome and three case studies.

Subspecialty referrals for pauciarticular juvenile rheumatoid arthritis.

OBJECTIVES: To examine referral patterns from primary care physicians for children with pauciarticular juvenile rheumatoid arthritis (JRA) and to determine whether children with pauciarticular JRA referred to pediatric rheumatologists differ in clinical presentation from children referred to other specialists. DESIGN: A retrospective records review of 49 patients with pauciarticular jRA was performed. Records were reviewed to determine the specialty of the referring physician and whether the children referred had symptoms and signs compatible with a synovitis at the time primary care was sought. SETTING: Inner-city tertiary pediatric rheumatology referral center. PARTICIPANTS: Children with pauciarticular JRA. MAIN OUTCOME MEASURES: Identification of referral patterns of primary care physicians. Associated morbidity owing to JRA was ascertained at the time of referral. RESULTS: Most children with pauciarticular JRA (62%) were referred to orthopedic surgeons prior to referral for pediatric rheumatology care. No differences in clinical symptoms were seen between children referred to pediatric rheumatologists and those referred to orthopedic surgeons. Children referred initially to orthopedic surgeons were younger than those referred to pediatric rheumatologists. CONCLUSION: A notable number of children with pauciarticular JRA are referred to orthopedic surgeons prior to the establishment of that diagnosis, even when such children present with unequivocal signs of synovitis. This may be owing to the misconception that arthritis is rare in preschool-aged children or to the difficulty of ascertaining the presence of synovitis in younger children.

Blau syndrome (familial granulomatous arthritis, iritis, and rash) in an african-american family.

Blau syndrome (familial granulomatous arthritis, iritis, and rash) was originally described in 1985, in 11 members of a family of Dutch ancestry. Inheritance is autosomal dominant. Several more Caucasian families have been described since. Skin and synovial biopsy specimens show noncaseating sarcoid like granulomas, but the lung is not involved as in classic sarcoidosis. This report describes 3 members of an African American family with Blau syndrome. It is important to differentiate this genetic disorder from other childhood arthritides, such as, juvenile rheumatoid arthritis, juvenile spondyloarthropathies, and early-onset sarcoidosis, because of the need for genetic counseling, treatment and differing potential for selective involvement of other organs (eye, skin, and tendons/joints). All children of an affected individual have a 50% chance of inheriting the disease. Unaffected children do not have to be concerned about subsequent generations being affected. The response to conventional treatments used in juvenile rheumatoid arthritis and to etanercept in our patients has not been satisfactory. Joint disease responds to corticosteroids, but these agents are not suitable for a disease that is lifelong. The eye involvement is aggressive and can lead to blindness. These patients need close follow-up by an ophthalmologist.

T cell receptor (V beta) bias in the response of rheumatoid arthritis synovial fluid T cells to connective tissue antigens.

T cell receptor (V beta) use in the response to type II collagen and cartilage proteoglycans was analysed in peripheral blood and synovial fluid T cells from RA patients. T cells from RA patients with an immune response to connective tissue antigens, and paired PB and SF samples were stimulated in vitro with type II collagen, high density aggrecan proteoglycans (PG), and the T cell mitogen concanavalin A. After short term culture, mRNA was extracted from cells and a reverse transcription-polymerase chain reaction was performed, using primers specific for eight TCR V beta determinants. Blood cells stimulated with ConA generated strong bands with virtually all the V beta primers tested, but the TCR (V beta) expression by SF T cells stimulated with mitogen was biased, suggesting a selection process during joint infiltration. The V beta phenotypes of cells responding to PG was restricted in individual RA patients, but the pattern of V beta use in the the RA population was not consistent. In contrast, the V beta phenotypes of SF cells responding to CII was highly biased in both individual patients and the RA population, with V beta 14, V beta 17, and V beta 8 phenotypes predominant. We conclude that the T cell response to connective tissue antigens is restricted compared with mitogen stimulation, with the highest degree of TCR bias seen in the response of SF T cells to stimulation with type II collagen.