Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0003864 (arthritis)
 69,039 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To investigate the genetics of susceptibility to early onset pauciarticular juvenile chronic arthritis (JCA), 158 unrelated ethnic British patients with a mean disease onset of 3.2 years, together with controls, were tested for HLA-A, B, C, and DR antigens. Additionally, 117 patients were also investigated for complement Bf and C4 markers. New observations included an increased frequency of the C4B 2 allotype (p corrected (pc) less than 0.02) and C4A 4,B 2 phenotype (p less than 0.0005). Findings suggested a unique increase of the haplotype HLA-DRw8, Bf*S, C4A*4, C4B*2, HLA-B39, possibly predisposing to more severe disease. Strong positive associations were confirmed with HLA antigens A2 (pc = 2.5 X 10(-8)), DRw8 (pc = 3.5 X 10(-14)), DR5 (pc less than 0.02), DRw52 (pc = 2.8 X 10(-6)) and DR5, w8 phenotype (pc = 3.9 X 10(-6)), and negative associations with DR7 (pc = 5.8 X 10(-7)), DR4 (pc less than 0.002), and DRw53 (pc = 0.004). Antinuclear antibody (ANA) seropositivity correlated with DR5 (p less than 0.02), and in children with chronic iridocyclitis (CIR) Bw62 incidence was raised (p less than 0.03) and B44 reduced (p less than 0.03). HLA-A2 was found in 88% of ANA+, CIR+ patients (p less than 0.01). A significant excess of DR5, w8 heterozygotes was present (relative risk = 41.1) and a lack of corresponding homozygotes. Results are inconsistent with a recessive, dominant, or intermediate mode of inheritance of susceptibility, and favour the existence of at least two DR linked 'disease' genes. Moreover, there may be an interaction in heterozygotes of combinatorial factors associated with DR5 and DRw8 in enhancing susceptibility. Possible immunogenetic mechanisms underlying the observed associations with three antigen classes are discussed. Evidence here suggests a role for the HLA-DQ locus in determining susceptibility to this disease.
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PMID:Genetic susceptibility to early onset pauciarticular juvenile chronic arthritis: a study of HLA and complement markers in 158 British patients. 348 35

The distribution of the recently described HLA-DP antigens was examined in a population of patients with pauciarticular juvenile rheumatoid arthritis and iridocyclitis, in an attempt to further characterize the immunogenetically determined susceptibility to this disease. There was a significantly increased frequency of the HLA-DPw2 antigen in the patients compared with the controls (67% versus 34%; odds ratio 3.9, P = 0.003 by Fisher's exact test). Population studies and family studies showed that this association with HLA-DPw2 was not secondary to linkage disequilibrium with the previously defined HLA-D region markers of disease (HLA-DR5 and HLA-DRw8) in these patients. These data raise the possibility that susceptibility to this form of juvenile rheumatoid arthritis may be regulated by more than one HLA-linked gene.
Arthritis Rheum 1986 Sep
PMID:HLA-DP antigens in patients with pauciarticular juvenile rheumatoid arthritis. 348 65

71 patients with juvenile spondarthritis were studied retrospectively. Age at onset of disease varied between 4 and 16 years. 94% of patients were HLA-B27 positive. Relatives with symptoms of spondarthritis were found in the families of 23 children. All patients developed radiographic sacroiliitis. Prominent disease symptom was a peripheral arthritis, which was mostly pauciarticular and asymmetric, involving big joints of the lower limbs. 41 patients suffered from heel pains, 33 complained of back or sacroiliac pains. An acute iridocyclitis with one or several relapses was seen in 10 patients. The results led to a basic concept of main and side criteria for diagnosis of juvenile spondarthritis. They enable a better classification for suspected or probable disease. Diagnosis is verified by manifestation of radiologic sacroiliitis.
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PMID:[Juvenile spondarthritis. Retrospective study of 71 patients]. 349 95

Risk factors for significant visual loss were investigated in 51 patients with iridocyclitis associated with juvenile rheumatoid arthritis (JRA). Average follow-up was 12.7 years. Of 89 eyes with uveitis, 22% had visual loss to 20/200 or worse, 46% had cataracts, 30% had band keratopathy, and 27% had glaucoma. Severity of visual loss and complications correlated with the degree of inflammation found on initial ocular examination. Of 58 eyes that were initially normal or had signs of mild inflammation (cells, flare, keratitic precipitates), 3% had final vision of 20/200 or worse, 28% had cataracts, 5% had band keratopathy, and 17% had glaucoma. Of 31 eyes with posterior synechiae on initial examination, 58% had final vision of 20/200 or worse, 81% had cataracts, 77% had band keratopathy, and 45% had glaucoma. When arthritis clearly preceded uveitis, 6% of patients had a poor visual outcome compared to 67% of patients whose initial manifestation of JRA was uveitis. Systemic corticosteroid administration used primarily for arthritis correlated with cataract formation.
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PMID:Prognostic factors in the uveitis of juvenile rheumatoid arthritis. 368 2

One hundred and fifty children with unsuspected juvenile chronic arthritis (JCA) and a disease duration of 3-6 months entered a prospective study. Diagnosis of JCA could be verified in 107 patients according to the WHO-EULAR criteria. After 5.0 +/- 0.9 years 66 of the 107 patients showed no disease activity, 24 of them for more than 2 years, 23 for less than 2 years without any drug therapy. Nineteen patients were still on NSAIDs and/or long acting drugs, 14 patients with unfavourable morphologic outcome (greater than stage II) and 17 patients with severe functional impairment (greater than or equal to stage III) all belonged to the 41 children with still active disease after 5 years follow-up. The disease course was polyarticular in 10 of the 14 patients with severe radiologic changes and in 14 of the 17 with unfavourable functional results. The wrist joint proved especially vulnerable since 46% of the severe radiologic changes concerned this joint. All 4 children with positive rheumatoid factor followed a polyarticular course with severe radiologic changes (stage greater than II), 3 of them together with unfavourable functional outcome. Risk factors for morphologically and functionally unfavourable course are therefore seen in a still active disease after 5 years, a polyarticular joint involvement and a positive rheumatoid factor. Extra-articular complications concerned chronic iridocyclitis in 7 patients, acute iridocyclitis in 2, amyloidosis in 1 and growth retardation in 2 children. -The prospective study is to be continued.
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PMID:Five year follow-up of a prospective cohort of juvenile chronic arthritis with recent onset. 369 Sep 88

Presence and titer of antinuclear antibodies (ANA) were determined in 217 juvenile rheumatoid arthritis (JRA) patients, by indirect immunofluorescence using HEp-2 cells as substrate. Positive ANA titers (greater than or equal to 1:40) were present in 131 (60%) of the JRA patients. All 3 JRA onset types demonstrated increased percentages of ANA positivity compared with healthy children. Sixty-seven percent of the patients in the polyarticular onset group had positive titers; titers were positive in 62% of the pauciarticular onset group and in 32% of the systemic onset group. ANA were also found in 45% of control patients with other connective tissue diseases. In JRA patients, the speckled pattern occurred most commonly (72%). Fourteen patients (8 with pauciarticular onset and 6 with polyarticular onset) had iridocyclitis; all of them had high titers (greater than or equal to 1:80) of ANA. The use of HEp-2 cells provided a sensitive substrate for detecting ANA in JRA. It proved to be of value in differentiating JRA patients from healthy controls, but not from patients with other connective tissue diseases.
Arthritis Rheum 1984 Nov
PMID:Use of the HEp-2 cell substrate in the detection of antinuclear antibodies in juvenile rheumatoid arthritis. 633 75

An assessment was undertaken of the value of plasmapheresis in patients with circulating immune complexes and/or extraintestinal manifestations of Crohn's disease (arthritis, iridocyclitis, erythema nodosum, pyoderma gangrenosum, stomatitis, fever). In 17 patients aged 20 to 50 years (median 34 years) 46 plasmaphereses were performed using a continuous flow-cell separator. Before and after plasmaphereses the concentrations of circulating immune complexes, of complement C4, of the immunoglobulins IgG and IgM and of the proteins alpha-1-antitrypsin, CRP, prealbumin and beta-lipoprotein were determined. 71% of patients given plasmaphereses showed clinical remission of the extraintestinal manifestations and 85% demonstrated a reduction of circulating immune complexes. Clinical follow-up of the patients was documented by the Crohn's Disease Activity Index (CDAI), which was reduced by plasmaphereses to a highly significant degree (p less than 0.005). For patients displaying an acute phase of Crohn's disease with extraintestinal manifestations and/or circulating immune complexes plasmapheresis represents an effective mode of therapy, which, moreover, lacks serious side effects.
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PMID:[Plasmapheresis in extra-intestinal manifestations of Crohn's disease]. 633 22

The early diagnosis of juvenile chronic polyarthritis rests on the recognition of 3 district modes of onset that are important in preventing deformities, blindness, and even death. Systemic onset is characterised by typical systemic features, including high spiking fever and rheumatoid rash; polyarticular onset is characterised by arthritis of more than 4 joints; and pauciarticular onset by involvement of 4 joints or less, most often a knee initially. Management must be individualised, including the use of non-steroidal anti-inflammatory drugs of which aspirin remains the drug of choice. The course of progressive polyarthritis, found in 15% of children, necessitates the additional use of slow-acting agents, such as intramuscular gold. Supportive measures include rest, splinting and exercise. Regular slit-lamp examination is mandatory to screen for asymptomatic iridocyclitis, which if undetected and untreated may result in blindness.
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PMID:Optimum Management of juvenile chronic polyarthritis. 641 7

Juvenile rheumatoid arthritis (JRA) is best defined as the condition of chronic synovitis in children. Such chronic childhood arthritis probably includes several distinct disease processes. Recognizable subgroups are systemic-onset disease (20%), rheumatoid factor-negative polyarthritis (25%), rheumatoid factor-positive polyarthritis (5%), pauciarthritis associated with antinuclear antibodies and chronic iridocyclitis (30%-35%), and pauciarthritis associated with sacroiliitis and HLA-B27 (10%-15%). Rheumatoid factor-positive polyarthritis appears to be the childhood equivalent of classic adult rheumatoid arthritis; the pauciarthritis associated with HLA B27 appears to be closely related to the spondyloarthropic diseases. Although there are no diagnostic laboratory tests, various subgroups differ in immunogenetic findings as well as in clinical appearance and prognosis. A wide variety of conditions (infectious diseases, childhood malignancies, genetic and congenital conditions, and noninflammatory musculoskeletal lesions) can mimic JRA and must be considered in the differential diagnosis. The outlook for most children with JRA is good; fewer than 20% have progressive destructive disease (generally those with rheumatoid factor-positive or systemic-onset disease). Therapy rests on the conservative use of antirheumatic drugs, active physical therapy programs, maintenance of activities, and attention to the psychosocial development of the whole child. Orthopedic surgery can be helpful, particularly in the rehabilitation of children who have suffered severe joint destruction or deformity. Combined orthodontic and oral surgery therapy can restore function and appearance for young people with the micrognathia of temporomandibular joint involvement.
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PMID:Chronic arthritis in children. Juvenile rheumatoid arthritis. 660 49

Sera from 104 children with JA with different onset-types of disease were evaluated for 19S IgM RF by the LFT , hidden 19S IgM RF by the hemolytic assay, ANA by HEp-2 cell substrate, and levels of IC by the C1qSPA . Their relationship to active disease was determined. Classical 19S IgM RF were detected by the LFT in only seven patients. All were late-onset polyarticular females. Hidden 19S IgM RF were detected by the hemolytic assay in the separated IgM-containing fraction in 55 patients of all onset-types. Clinical activity correlated with the presence of hidden 19S IgM RF in 82% of cases. ANA, using the HEp-2 cell substrate, were found in 61 patients, the majority showing a speckled, immunofluorescent pattern. ANA were noted in all RF positive patients and in nine of 10 patients with iridocyclitis. IC were found in 39 patients, and correlation with clinical activity occurred in 54% of cases. A search for positive associations among the four parameters showed no statistically significant correlations except for the concordance of ANA positivity in all seven RF positive patients. The presence of hidden RF correlated more closely with disease activity (P less than 0.001) than did that of ANA or IC. The significance of these data and previous studies remains to be determined. We have demonstrated that in the average JA population 7% have 19S IgM RF and about 60% have hidden RF, ANA, or elevated levels of IC. The present findings of 98 of 104 patients with at least one of the abnormal immunoproteins , the association of ANA in patients with iridocyclitis or with RF positivity, of hidden RF with disease activity, and the presence of 19S IgM RF in isolated IC suggest a possible immunologic etiology for JA.
Semin Arthritis Rheum 1984 May
PMID:Autoantibodies in juvenile arthritis. 661 Feb 17


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