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ID PMID Title PublicationDate abstract
8351867 [Sjögren's syndrome]. 1993 Apr The authors present an account on Sjögren's syndrome--clinical manifestations, diagnosis, pathogenetic aspects and treatment. The diagnosis was based on three findings: keratoconjunctivitis sicca, stomatodynia--xerostomia and other immunological, diseases. The authors used different immunological examinations, bioptic examination of the oral mucosa and parotid glands. As to gastroenterological examinations, the gastric secretion is examined according to Lambling's method, the gastroduodenum is subjected to radiographic examination, endoscopy is performed as well as a bioptic examination of the gastroduedenal mucosa.
8256628 Immunological aspects of juvenile rheumatoid arthritis. 1993 Oct This article reviews the evidence from recent studies on immunological abnormalities associated with pathophysiologic mechanisms operating in three clinical subtypes of juvenile rheumatoid arthritis (JRA) (polyarticular, pauciarticular and systemic). The main discussion is focused on three hallmarks of immunopathological studies. First, abnormalities in phenotype and function of lymphocytes from peripheral blood and inflamed synovium are discussed. The aberrations of lymphocytes are elucidated by T and B cells expressing phenotypic cell-markers such as CD20, CD21, CD4, CD8 and DR in association with different subtypes and disease activity. The functional imbalance and impairment of T and B cells are mainly observed by abnormal proliferation and/or in vitro Ig production in response to mitogens and alloantigens. Second, because the appearance of rheumatoid factors (RF) in serum indicates that the pathogenesis of JRA may be based on the autoimmune mechanism, the prevalence of RF including IgM, IgA and IgG isotype, hidden IgM RF and cross-reactive idiotype RF, and their characteristic properties are discussed. Moreover, specific auto-antibodies (antinuclear antibodies and others) for JRA are illustrated in this paper. Third, the production of various pro-inflammatory cytokines resulting in the release of tissue-damaging chemical mediators is also discussed. This may play a central role in the generation of systemic inflammation and joint involvement in JRA.
1326411 Cytokine abnormalities in inflammatory arthritis. 1992 Jun Cytokines are soluble molecules which control communication between cells of the immune and non-immune systems. Studies on their role in the pathogenesis of inflammatory arthritis have been increased with the discovery of new cytokines and the development of assays for their detection. Conditions such as rheumatoid arthritis are characterized by increased production of proinflammatory cytokines in association with reduced control by regulatory cytokines produced by T lymphocytes. The inadequate inhibition of proinflammatory cytokines by anti-inflammatory cytokines and other regulatory mechanisms contributes to this cytokine imbalance. This situation is responsible for the enhanced degradation, without sufficient repair activity. These results have provided the rationale for the use of cytokines as well as for drug targeting of the cytokine network in rheumatoid arthritis. This also includes the modulation of the cytokine network by targeting the level of the receptors as well as the effects and/or the responding cells.
1419506 Juvenile rheumatoid arthritis, juvenile chronic arthritis, and juvenile spondyloarthropath 1992 Oct Immunogenetics are supporting the marked heterogeneity of chronic arthritis in children. Thus DRw13-DRw18 and DQw6-DQw18 were associated with persistent pauciarticular disease in children with an early onset of disease. Several studies have shown DPw2 as an additional susceptibility factor in this subgroup. Standardization of diagnostic criteria for juvenile onset spondyloarthropathy and psoriatic arthritis is necessary; various studies are in progress, and although HLA-B27 provides the common marker, this may only apply to a small group of juvenile psoriatics who have spondyloarthropathy. In the management of juvenile rheumatoid arthritis, methotrexate in moderate doses has been shown to be superior to lower doses of methotrexate and placebo in controlling polyarthritis. Methotrexate may be of particular value in treating the polyarthritis that follows a pauciarticular onset. The possible value of sulfasalazine in a B27 group with persistent polyarthritis has been suggested. Highlights of corticosteroid therapy were intra-articular injections, particularly in pauciarticular disease, the suggestion that deflazacort has a calcium sparing effect, and the possible role of intravenous methylprednisone in the management of severe disease.
10830004 Clinico-immunological profile in juvenile rheumatoid arthritis--an Indian experience. 1996 May From a Pediatric Rheumatology Clinic 361 children diagnosed as juvenile rheumatoid arthritis (JRA) according to American Rheumatism Association-JRA criteria were studied retrospectively for their clinico-immunological profile. The mean age of onset in systemic, pauciarticular and polyarticular onset, JRA subtypes were 5.2, 6.8 and 7.2 years respectively. There was male preponderance in systemic and pauciarticular JRA. In seropositive polyarticular JRA, girls outnumbered boys. The frequency of occurrence of systemic, pauciarticular and polyarticular disease was 87 (24%), 108 (30%) and 166 (46%) respectively. The systemic onset disease was dominated by extra-articular manifestations in terms of fever (100%), rash (57%), hepatomegaly (51%) and lymphadenopathy (25%). The pauci- and polyarticular illnesses were commonly dominated by joint involvement, morning stiffness, and in few patients, by extra-articular manifestations also. The joints were involved symmetrically. Most commonly involved joints in order of decreasing frequency were knee, ankle, wrist and elbow in all the subtypes. Anemia and leucocytosis were observed in majority with higher frequency in systemic onset JRA. The rheumatoid factor (RF) was present in 15% of polyarticular JRA. RF was also present in 7 and 9% of patients with pauciarticular and systemic subtypes respectively. The antinuclear antibody was positive in only 3 out of 66 patients in whom the test was carried out. The demographic profile and trends in clinical features were similar to the studies reported on caucasian population with difference in the actual frequency of various clinical features.
1389243 Pulmonary autograft failure after aortic root replacement in a patient with juvenile rheum 1992 We report a 12-year-old girl suffering from juvenile rheumatoid arthritis with severe aortic valve incompetence who died 5 months after an initially successful Ross procedure. Pulmonary autograft failure was a result of recurrence of aggressive valvulitis.
1290852 Bone marrow examination before steroids in thrombocytopenic purpura or arthritis. 1992 Dec Corticosteroids were used to treat two children with presumed idiopathic thrombocytopenic purpura and one with juvenile rheumatoid arthritis without examination of the bone marrow. Of the two with presumed idiopathic thrombocytopenic purpura, one had Fanconi's anaemia and the other may have had aplastic anaemia. The third child had acute lymphoblastic leukaemia. The diagnosis of Fanconi's anaemia was delayed. A diagnostic and therapeutic dilemma was caused in the second case. In the third, delayed diagnosis and, perhaps, compromised outlook resulted. These three cases re-emphasize the well aired caveats about the diagnosis of idiopathic thrombocytopenic purpura and juvenile rheumatoid arthritis and provide further support for the arguments of those who believe that if corticosteroids are to be used to treat such children, their bone marrow should be examined first.
1567496 Autoantibodies to the chromosomal protein HMG-17 in juvenile rheumatoid arthritis. 1992 Apr OBJECTIVE: To determine the antibody profiles in sera from patients with juvenile rheumatoid arthritis (JRA). METHODS: Immunoblotting using nuclear extracts and recombinant high-mobility group (HMG) nonhistone chromosomal proteins. RESULTS: Antibodies directed against HMG-17 were found in 47% of antinuclear antibody (ANA)-positive patients with pauciarticular-onset JRA and in 16% of ANA-positive patients with polyarticular-onset JRA. HMG-17 values of 6% and 8%, respectively, were detected in ANA-negative patients with JRA and in those with nonrheumatic diseases. CONCLUSION: There is evidence for a high prevalence of anti-HMG-17 antibodies in sera of patients with pauciarticular-onset JRA.
7572171 Childhood leukemia mimicking juvenile rheumatoid arthritis. 1995 Jul Arthritis is a known manifestation of childhood leukemia. When it is the sole clinical finding, diagnosis of juvenile rheumatoid arthritis (JRA) may be impressed initially and hence delay diagnosis of the underlying malignancy. This review analysed the clinical pictures of six such patients whose acute lymphoblastic leukemia (ALL) was diagnosed after a variable period of delay, ranging from 2 weeks to 44 months. In general, initial articular and extra-articular symptoms, and responses to conventional treatment, are not helpful in differentiating leukemic arthropathy from juvenile rheumatoid arthritis. However, the six ALL patients did have significantly less leukocytosis (6834 +/- 1586 vs 13365 +/- 8039/mm3, p < 0.05) and relative lymphocytosis (61 +/- 17% vs 30 +/- 13%, p < 0.05) on the initial hemograms when compared with JRA patient findings. JRA patients with initial hemograms showing less leukocytosis and relative lymphocytosis should be followed up with a high index of suspicion. Work-up for leukemia should be performed in any JRA patient with an evolving hemogram showing anemia, thrombocytopenia, leukopenia and lymphocytosis. Those who have an intractable clinical course necessitating immunosuppressive therapy should also receive bone marrow examination to obviate confusion in interpreting follow-up laboratory data. It can not be overemphasized that the differential diagnosis of acute leukemia should be made before JRA is impressed.
8216396 Effects of methotrexate on radiologic progression in juvenile rheumatoid arthritis. 1993 Oct OBJECTIVE: To assess the effects of methotrexate (MTX) therapy on radiologic progression in juvenile rheumatoid arthritis (JRA). METHODS: We evaluated serial wrist radiographs for carpal length in 23 JRA patients with bilateral wrist involvement, before and during MTX treatment. These carpal length measurements were compared with established norms for carpal length in a healthy pediatric population. RESULTS: Both clinical responders to MTX (17 of 23 patients) and nonresponders (6 of 23) had decreasing carpal length prior to initiation of the treatment. Eleven of the 17 clinical responders had improved carpal length after a mean of 2.5 years of MTX treatment. All 6 clinical nonresponders had progressive loss of carpal length. CONCLUSION: MTX treatment resulted in radiologic improvement, as measured by carpal length, in the majority of children with JRA who had a clinical response to MTX.
8827866 The seronegative spondyloarthropathies. 1996 Jul The four seronegative spondyloarthropathies can be divided into two main groups by their pattern of sacroiliitis and spondylitis (Table 1). The axial skeletal changes of ankylosing spondylitis and enteropathic arthropathy are often indistinguishable, as are those of psoriatic arthritis and Reiter's syndrome. Early proximal appendicular joint involvement in ankylosing spondylitis is a poor prognostic sign except in women where peripheral arthritis is more common, but has a more benign course. Peripheral joint destruction in enteropathic arthropathy is rare because treatment of the bowel disease also treats the arthritis. Distal appendicular involvement is characteristic of psoriatic arthritis and Reiter's syndrome. Proliferative erosions and enthesitis, periostitis, and normal mineralization aid in differentiating psoriatic arthritis and Reiter's syndrome from rheumatoid arthritis. The distribution of arthritis also differs from that seen in classic rheumatoid arthritis, with asymmetry and involvement of the distal interphalangeal joints more common in psoriatic disease and Reiter's syndrome.
1605877 [Pneumopathy in children with juvenile rheumatoid arthritis]. 1992 May In the National Institute of Pediatric were studied 35 patients with juvenile rheumatoid arthritis, in a prospective form, in order to asses pulmonary manifestations. Arthritis had between 5 months to five years of clinical evolution. The radiologic patterns of the lung correlated well with respiratory tests, without correlation with pulmonary gammagram which was normal in all the cases. Symptoms were not characteristic of any type of pulmonary lesion.
8441140 Complement activation and immune complexes in juvenile rheumatoid arthritis. 1993 Jan We investigated the potential role of the complement system in juvenile rheumatoid arthritis (JRA) by examining plasma for levels of complement activation fragments C4d and Bb. We correlated findings with both disease activity and laboratory abnormalities, including immune complex (IC) levels. While there was a strong correlation between active disease and both C4d and Bb plasma levels in JRA, no strong correlations could be made between IC levels and complement activation products; weak associations were seen between complement activation fragments and erythrocyte sedimentation rate. Our data suggest that plasma complement activation is a concomitant of active disease in JRA; however, its role in the pathophysiology of JRA remains uncertain.
8068484 Case report: immunoglobulin A deficiency in patients with juvenile rheumatoid arthritis tr 1993 In three patients with juvenile rheumatoid arthritis, serum IgA concentrations were within the normal limit at the onset of disease and before aspirin administration. After aspirin administration, their serum IgA levels gradually decreased. After discontinuation of aspirin, their serum IgA levels gradually increased. These results suggest that IgA deficiency may be due to aspirin administration in such patients. The IgA production in vitro of peripheral blood mononuclear cells from a patient taken 3 months of discontinuation of aspirin was markedly inhibited by preincubation with aspirin. Since the patients' serum IgG and IgM levels hardly changed the heavy chain class switching may be influenced by aspirin through some undefined mechanisms.
8871845 Vitamin E, thiobarbituric acid reactive substance concentrations and superoxide dismutase 1996 Jul OBJECTIVE: To study the role of active oxygen species in tissue injury in rheumatoid arthritis. METHODS: We examined the levels of thiobarbituric acid reactive substances (TBARS) and antioxidants of the first line antioxidative defence of the organism, i.e. vitamin E (VE) and superoxide dismutase (SOD) in the blood of 74 young patients with juvenile rheumatoid arthritis (JRA) and in 138 healthy children, all aged 3-15. RESULTS: A statistically significant increase of TBARS was found in the blood plasma of the children with JRA compared with the control group. In the whole group of patients and in the patients over 6 years of age, the VE concentration was in the red blood cells (RBC) was significantly lower in children who had suffered from JRA for more than one year and in those with the systemic form of the disease. The type of treatment also affected the values for the plasma VE and SOD in the RBC. CONCLUSION: Our results seem to confirm the supposition of increased oxidative stress in children with JRA and low antioxidant levels in terms of SOD activity and vitamin E concentrations.
8248215 Dominant T-cell-receptor beta chain variable region V beta 14+ clones in juvenile rheumato 1993 Dec 1 The characteristic histopathology and major histocompatibility complex associations in juvenile rheumatoid arthritis suggest an oligoclonal antigen-specific T-cell population may be critical to pathogenesis. To test this, we analyzed the T-cell repertoire of a polyarticular HLA-DR4+ juvenile rheumatoid arthritis patient with an aggressive form of disease that required arthrocentesis of the knee joints and early replacement of both hip joints. A comparison of T-cell-receptor beta chain variable region (V beta) gene expression in peripheral blood and synovial fluid performed by semiquantitation of cDNA samples amplified by the PCR revealed overexpression of the T-cell-receptor V beta 14 gene family. To determine the nature of V beta 14 overexpression, we sequenced randomly cloned amplification products derived from two synovial fluid, two synovial tissue, and three peripheral blood samples by using a V beta 14/beta chain constant region primer pair. Sequence data showed that the T-cell response in the synovia was oligoclonal. Of four clones found, one was present in all joints examined and persisted over time. This clone accounted for 67% and 74% of all V beta 14+ clones sequenced in two synovial fluid samples and 75% and 40% in two synovial tissue samples. This clone was also found at a lesser frequency in peripheral blood samples. Further studies provided evidence for the presence of oligoclonally expanded populations of T cells utilizing the V beta 14 T-cell receptor in 6 of 27 patients examined. In contrast to the remaining patients studied, 3 with a late onset polyarticular course who exhibited especially marked clonality were characterized by features typical of adult rheumatoid arthritis (IgM rheumatoid factor-positive and HLA-DR4+). These data suggest a role for V beta 14+ T cells in a group of juvenile rheumatoid arthritis patients.
7932426 Complement activation and immune complexes in children with polyarticular juvenile rheumat 1994 Jun OBJECTIVE: To determine whether levels of either circulating immune complexes (IC) or complement activation fragments change as disease activity changes in children with polyarticular juvenile rheumatoid arthritis (JRA). METHODS: Twenty-six children with polyarticular JRA were examined over a time course of 4-21 months. Plasma complement activation fragments and IC were measured by commercially available enzyme linked immunoabsorbant assays. RESULTS: Both complement activation fragments and IC levels were lower in individual children when their articular disease was inactive. Children with persistently active disease had no change in any of these measures. CONCLUSION: Elevated levels of plasma IC and complement activation fragments are features of active polyarticular JRA. We believe our data support a role for the complement system in the pathophysiology of polyarticular JRA.
8441143 Glycosylation of alpha 1-acid glycoprotein in systemic onset juvenile rheumatoid arthritis 1993 Jan Agarose based affinity immunoelectrophoresis with free concanavalin A (Con-A) as ligand was used to examine the microheterogeneity of alpha 1-acid glycoprotein in sera of patients with systemic onset juvenile rheumatoid arthritis (JRA) and acute bacterial infections. In JRA, a decreased proportion of Con-A reactive alpha 1-acid glycoprotein variants was found when compared to healthy control. In contrast, acute bacterial infection showed an increased reactivity. Investigation of glycosylation may be useful in the differential diagnosis of systemic onset JRA and acute bacterial infection.
8701483 Juvenile rheumatoid arthritis: cervical spine involvement and MRI in early diagnosis. 1996 Apr Magnetic resonance imaging (MRI) of the cervical spine was performed on 20 patients (mean age 10 years) with a preliminary diagnosis of juvenile rheumatoid arthritis (JRA). In all patients conventional x-rays of the cervical spine were obtained, and the relationship between clinical status and MRI findings were evaluated. Two patients with clinical manifestations, including neck pain and diminished range of motion, exhibited significant pathologic features on radiogram and MRI, the latter providing more detailed information. Among 18 patients who had no complaints about their cervical spines, 3 patients (65%) had either soft tissue involvement, pannus formation or erosions on the surface of atlantoaxial joints; only four patients (20%) had erosions on plain x-ray views. Since the early diagnostic ability of MRI in JRA allows early therapeutic intervention, every patient with a probable diagnosis of JRA would benefit from MRI.
23194870 Special extract of BOSWELLIA serrata (H 15) in the treatment of rheumatoid arthritis. 1996 May H15, a special extract of the gum resin of Boswellia serrata (BS) is effective in the treatment of rheumatoid arthritis (RA). These findings were obtained in more than 260 patients by using a range of different clinical approaches for evaluation. The criteria for assessment were mainly joint swelling, pain, erytrocyte sedimentation rate (ESR), stiffness, additional use of NSAID, side effects and tolerance. The therapeutic action was mainly proven by comparing H15 with a placebo standard therapy. H15 is: