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ID PMID Title PublicationDate abstract
10402042 Sjögren autoantibodies modify neonatal cardiac function via M1 muscarinic acetylcholine r 1999 Jul 1 Isolated congenital heart block may be associated with primary Sjögren syndrome. In this work we describe circulating antibodies in the sera of primary Sjögren syndrome patients that are able to interact with neonatal myocardium by activating muscarinic acetylcholine receptors of M1 subtype. We report on the presence of autoantibodies against the second extracellular loop of human M1 muscarinic acetylcholine receptors in primary Sjögren syndrome mothers whose children have congenital heart block using a synthetic peptide in indirect immunofluorescence technique. Autoantibodies from primary Sjögren syndrome patients gave positive image on neonatal atria but not on adult atria slices. The synthetic M1 peptide selectively abrogated indirect immunofluorescence recognition. The primary Sjögren syndrome-immunoglobulin G also displayed an 'agonist like' activity modifying the intracellular events associated with muscarinic acetylcholine receptor activation. The mechanism appears to occur secondarily to stimulation of phosphoinositides turnover via phospholipase C activation. This, in turn, triggers cascade reactions involving calcium/calmodulin and leads to activation of nitric oxide synthase and soluble guanylate cyclase. All of these effects were selectively blunted by pirenzepine and neutralized by M1 synthetic peptide. These biological effects were not obtained using adult instead of neonatal rat atria and neither occurred with the sera of normal healthy women of childbearing age. It could be concluded that antibodies against neonatal M1 muscarinic acetylcholine receptor may be another serum factor to be considered in the pathophysiology of the development of congenital heart block associated with primary Sjögren syndrome mothers.
10084034 Fluctuation of anti-Ro/SS-A antibody levels in patients with systemic lupus erythematosus 1999 Jan OBJECTIVE: To determine whether the titers of anti-Ro/SS-A (Ro) antibodies fluctuate during the course of SLE and Sjögren's syndrome (SS) in parallel with disease activity, and if such fluctuations could be used to predict disease flares. We also evaluated whether the anti-Ro profile (anti-Ro 52, anti-Ro 60) changes over time, since such information could provide new insights into the induction and regulation of anti-Ro autoimmunity. METHODS: Sixteen patients with SLE and 15 patients with SS, all anti-Ro/SS-A antibody positive, were followed up for two years at three-month intervals. Clinical and laboratory parameters of disease activity were examined. Determination of the anti-Ro/SS-A titer was performed by counterimmunoelectrophoresis and the fine anti-Ro antibody specificity was determined by immunoblotting. RESULTS: The titers of anti-Ro antibodies fluctuated during the course of the illness in both SLE and SS patients. In SLE patients these changes were not (except in one case) associated with disease activity nor were they predictive of disease flares. The same was true for the SS patients, with the exception of two patients with skin vasculitis in whom anti-Ro antibody titers fluctuated in parallel with the disease activity. The anti-Ro antibody (anti-Ro 60 kD, anti-Ro 52 kD) specificity did not change in any of the patients during the follow-up period. CONCLUSION: Anti-Ro antibodies could represent a valuable indicator of disease activity in SS patients with cutaneous disorders. They do not, on the other hand, reflect disease activity in patients with SLE. The stable antibody profile in both SLE and SS patients supports the hypothesis that autoantibody production is predominantly genetically regulated.
9582552 Absence of H(+)-ATPase in the intercalated cells of renal tissues in classic distal renal 1998 Apr Proton-secretory defect is thought to be a major pathophysiologic mechanism leading to classic distal renal tubular acidosis (dRTA). However, there have been only two case reports demonstrating the absence of proton pump in renal tissues of the patients with Sjögren's syndrome. This study presents two cases of classic dRTA in which the absence of intact H(+)-ATPase was shown in their renal biopsy tissues by immunohistochemistry using a rabbit polyclonal antibody against the 70 kDa catalytic subunit of H(+)-ATPase from bovine brain clathrin-coated vesicles; one of the cases is diagnosed as subclinical Sjögren's syndrome and the other is idiopathic dRTA. A normal human kidney (NC) and the renal biopsy tissues from a patient with chronic tubulointerstitial nephritus whose proton secretory capacity was intact (DC) were compared as controls. The first patient, a 26-year-old woman, presented with quadriparesis. Her serologic tests revealed positive autoantibodies (ANA, SSA; SSB & RF), and a lower lip biopsy confirmed the diagnosis of Sjögren's syndrome. The second patient, a 43-year-old woman, who initially presented with a pathologic fracture of both femoral necks was referred for an evaluation for hypokalemia by the Department of Orthopedic Surgery. Her renal ultrasonography showed medullary calcification, and no autoantibodies were positive. Serum electrolytes and blood gas analyses of the two patients indicated severe hypokalemia and metabolic acidosis, and proton secretory defects were shown by a failure to lower the urine pH during marked acidemia induced by NH4Cl loading and an abnormally low urine-blood pCO2 difference during bicarbonate administration. While stainings with the anti-H(+)-ATPase antibody in NC and DC were strongly positive in intercalated cells in the connecting tubules and collecting ducts, the tissues from both patients with dRTA were devoid of any anti-H(+)-ATPase staining in the intercalated cells. These results support that the pathophysiologic basis of impaired H+ secretion in idiopathic classic dRTA as well as Sjögren's syndrome is the absence of intact H(+)-ATPase pumps in the intercalated cells.
9566665 A longitudinal study of pulmonary involvement in primary Sjögren's syndrome: relationship 1998 Mar Eighteen non-smoking women suffering from primary Sjögren's syndrome (pSS) with previously documented alveolitis were re-examined, clinically and by pulmonary function tests (PFT), bronchoalveolar lavage (BAL), chest X-ray and high-resolution computed tomography (HRCT) after a 2 yr follow-up period. Longitudinal evaluation revealed unchanged PFT. The final BAL study showed a normal differential count in six of 14 patients with initial lymphocyte alveolitis, and a persistent alveolar lymphocytosis in the remaining eight patients, associated with an increased percentage of neutrophils in one of them. In four patients with initial mixed alveolitis, the BAL cell profile was unchanged 2 yr later. Five of 18 patients (28%) had abnormal HRCT, represented by isolated septal/subpleural lines in three patients, ground-glass opacities with irregular pleural margins in one patient, and ground-glass opacities associated with septal/subpleural lines in another. All these patients had abnormal BAL results with an increased proportion of both neutrophils and lymphocytes. The presence of alveolar neutrophils was associated with a significantly (P=0.005) greater mean rate of reduction of carbon monoxide diffusing capacity (DLCO) -- more than four times the normal rate of loss of DLCO. Chest X-ray, repeated at the end of the 2 yr follow-up period, showed parenchymal abnormalities in only one patient who had evidence of fibrosis on HRCT. This study provides evidence that lung involvement is not an uncommon extraglandular manifestation of pSS and that a BAL neutrophilia may play an important role in the pathogenesis of pulmonary disease in this autoimmune disorder.
9080300 Sjögren's syndrome salivary gland immunopathology: increased laminin expression precedes 1997 Feb Previous studies have demonstrated increased expression of a laminin-like protein in labial salivary glands from Sjögren's syndrome (SS) patients. The objective of the present study was to verify the identity of this protein and to compare lymphocytic infiltration and laminin expression in minor salivary gland ductal epithelium. This was carried out by comparing laminin protein and laminin mRNA expression in 13 SS patients, 11 normal controls, and 12 patients with non-specific sialoadenitis. Laminin protein and laminin mRNA expression was determined using immunoperoxidase (IP) and in situ hybridization (ISH) techniques, respectively. In addition, the relationship between lymphocytic infiltration and laminin expression was evaluated on adjacent serial salivary gland sections from SS patients, using routine histological methods and IP immunohistochemistry. Biopsies from SS patients showed significant increases in staining for both laminin protein and laminin mRNA compared to normal controls. On seven of the eight SS samples that showed significant laminin protein staining, the ductal epithelial staining occurred in the absence of periductal lymphocytic foci. Results of the ISH assay strongly suggest that the increased expression of the laminin-like protein is laminin, since the cDNA probe was specific for the B1 chain of laminin. In addition, this increased expression in ductal epithelial cells occurs without significant lymphocytic infiltration. These studies provide further evidence that altered laminin expression is an early event associated with salivary gland pathology in SS, since these data demonstrate a potential pathologic event prior to the arrival of lymphocytes. Further studies are underway to examine the relationship between laminin and lymphocytic infiltration in salivary gland pathology.
9046742 Asymptomatic primary Sjögren's syndrome in a patient with penicillin drug eruption. 1997 Jan A 20-year-old woman visited our clinic because of acral numbness and skin eruptions after administration of oral penicillin. Serological tests revealed an increase of immunoglobulins (G & M), positive rheumatoid factor, anti-nuclear factor, anti-SSA and -SSB antibodies. Ophthalmologic and otolaryngological studies were consistent with those of Sjögren's syndrome. However, a skin biopsy specimen failed to show any specific changes for Sjögren's syndrome and the diagnosis of penicillin drug eruption was considered to be more preferable. We briefly discussed the coexistence of a drug allergy and asymptomatic connective tissue disease.
11434479 Remission of the renal involvement in a patient with primary Sjögren's syndrome (SS) afte 2001 We report the case of a young female patient with primary Sjögren's syndrome (SS). In addition to sicca symptoms she also suffered from progressive renal insufficiency and renal tubular acidosis (RTA). She was treated with three sets of pulse high-dose corticosteroid infusion and subsequent low-dose corticosteroid oral administration. When the efficacy was evaluated about 6 months after the start of the therapy, dramatic improvements were seen with no adverse effects, not only in laboratory tests but also histopathologically, as indicated by the repeat kidney biopsy. This suggests that renal involvements of SS might be reversible in some cases, and that there might be a clinical benefit of pulse high-dose corticosteroid infusion therapy in SS with progressive renal involvement.
10805354 Clinical evolution, and morbidity and mortality of primary Sjögren's syndrome. 2000 Apr OBJECTIVES: To study the clinical and laboratory profile evolution, as well as morbidity and mortality impact, of primary Sjögren's syndrome (pSS), in a large cohort of patients followed-up longitudinally. METHODS: We studied the evolution of the clinical picture and laboratory profile of pSS, the incidence and predictors for systemic sequelae, and the impact of pSS on overall survival in a prospective cohort study of 261 patients with pSS. Analyses included calculation of incidence rates, Cox proportional hazards predictive models, and estimation of standardized mortality ratios (SMRs) compared with the general Greek population, adjusting for age and sex. RESULTS: Glandular manifestations of the syndrome were typically present at the time of diagnosis. Systemic manifestations such as arthritis, Raynaud's phenomenon, purpura, interstitial nephritis, and liver involvement, as well as the serological profile, also did not change substantially during subsequent follow-up. Incidence rates for peripheral neuropathy, glomerulonephritis, and lymphoproliferative disorders were 3.3, 6.6, and 12.2 per 1,000 person-years, respectively. Glomerulonephritis and lymphoma tended to co-exist in the same patients (relative risk, 34.0; P < .0001). The development of lymphoproliferative disorders was associated with low levels of C4 complement (relative risk, 7.5; P = .0016), the presence of mixed monoclonal cryoglobulins (relative risk, 7.9; P = .0012), and purpura (relative risk, 3.9; P = .037). Low levels of C4 was the strongest predictor for mortality after adjusting for age (relative risk, 6.5; P =.0041). Patients with pSS had an SMR of 2.07 (95% CI, 1.03 to 3.71). However, when patients with adverse predictors were excluded, the mortality rate was identical to that of the general population (SMR 1.02). CONCLUSIONS: The initial presentation of pSS determines subsequent outcome. Purpura, decreased C4 complement levels, and mixed monoclonal cryoglobulinemia are adverse prognostic factors. The overall mortality of patients with pSS compared with the general population is increased only in patients with adverse predictors.
10690174 Possible involvement of IL-12 expression by Epstein-Barr virus in Sjögren syndrome. 1999 Nov AIM: To determine the correlation between interleukin 12 (IL-12) expression and Epstein-Barr virus (EBV) in Sjögren syndrome. METHODS: Indirect immunohistochemical technique, enzyme linked immunosorbent assay (ELISA), and immunoblot analysis were used to investigate IL-12 expression by EBV activation, using 13 surgical specimens and four B cell lines. RESULTS: Marked expression of IL-12 was found in the epithelial cells and the infiltrating B cells of salivary gland tissues from patients with Sjögren syndrome (six of 10 cases), but not in those from normal individuals (none of three cases). A striking topographic correlation between IL-12 and EBV was found. In addition, levels of IL-12 production by B cell lines were clearly enhanced by EBV activation in vitro. CONCLUSIONS: IL-12 expression closely reflects the intracellular event of EBV activation in Sjögren syndrome, and may contribute to the T helper cell type 1 (Th1) cytokine overexpression seen in this disease.
10388393 s-IgA and cytokine levels in whole saliva of Sjögren's syndrome patients before and after 1998 Dec Previous investigations have found elevated levels of s-IgA in the parotid saliva and normal levels in submandibular saliva of patients with Sjögren's syndrome (SS). Fox et al. also found elevated levels of cytokines (i.e., IL-2 and IL-6) in serum, salivary epithelial cells and parotid saliva of patients with SS. The oral administration of pilocarpine hydrochloride stimulates whole and parotid salivary flow. The purpose of this study was to determine the levels of s-IgA and IL-2 and IL-6 in whole saliva before and after administration of pilocarpine hydrochloride in SS subjects. Ten definitively diagnosed SS subjects were enrolled in the study, as were ten controls (C). The mean age was 57.2 years and all subjects were female. Whole unstimulated saliva (WUS) was collected by standard techniques for 5 min, after which the volume and flow rate were determined (mean WUS: SS = 0.047 vs C = 0.480 ml/min). Samples were centrifuged and the immunoglobulin analysis performed on the supernatants by immunoreactivity in a double-sandwich technique as previously described by Rudney et al. Cytokine analysis was performed similarly utilizing commercially available kits from R&D Systems. The results as analyzed by pairwise t-tests revealed comparable levels of s-IgA in the saliva of the SS patients, as compared to controls at baseline (means +/- SEM: SS-IgA = 348.1 +/- 82.0 vs C-IgA = 284.0 +/- 65.1 micrograms/ml; NS). Whole salivary flow was significantly increased (328%) in the SS subject group 60 min after the administration of 5 mg pilocarpine hydrochloride (means +/- SEM: 0.0472 +/- 0.017 vs 0.1546 +/- 0.054 ml/min; P < 0.01). There was no significant change in the concentration of s-IgA in the SS subject group following the pilocarpine dose (means +/- SEM: SS-IgA = 439.9 +/- 121.2 microliters/ml; P = NS). There were elevated levels of IL-2 in the saliva of four out of the ten and IL-6 in two out of the ten SS patients, as compared to controls (means +/- SEM: SS-IL-2 = 127.8 +/- 11.4 vs C-IL-2 = 30.8 +/- 1.6 pg/ml and SS-IL-6 = 41.4 +/- 7.1 vs C-11.6 +/- 2.8 pg/ml). There was also a significant decrease in the concentration of IL-2 in the same four out of ten SS subjects following the pilocarpine dose (means +/- SEM: SS-IL-2 = 32.4 +/- 10.3; P < 0.01). These preliminary results indicate that s-IgA levels do not change with increased salivary flow following the administration of pilocarpine hydrochloride in patients with Sjögren's syndrome. While cytokines are elevated in the whole saliva of some SS patients, a decrease in IL-2 concentration may occur with increased salivary flow.
10364906 Hydroxychloroquine treatment for primary Sjögren's syndrome: its effect on salivary and s 1999 Apr OBJECTIVE: To evaluate the effect of hydroxychloroquine treatment on interleukin 6 (IL6), hyaluronic acid (HA), and soluble interleukin 2 receptor (sIL2R) concentrations in the saliva and serum of patients with primary Sjögren's syndrome (SS). METHODS: Fourteen SS patients treated with hydroxychloroquine 200 mg/day for 12 months were investigated in an open prospective study. Clinical parameters of efficacy and routine biochemical and haematological data to assess drug safety and tolerability were determined every three months. Salivary and serum IL6, sIL2R, and HA values were determined at study entry, 6 and 12 months, using ELISA and radiometric assays. RESULTS: After hydroxychloroquine treatment, salivary IL6 concentrations decreased from 13.2 (1.2) to 7.3 (1.1) pg/ml (mean (SEM)) (p < 0.0001). Similarly, salivary HA concentrations were also reduced from 577.8 (120) to 200 (34) ng/ml (mean (SEM) (p < 0.003). Serum IL6 concentrations decreased from 5.4 (0.6) to 2.9 (0.2) pg/ml (mean (SEM) (p < 0.001), while serum HA concentrations remained unchanged. No change has been detected in salivary or serum sIL2R concentrations after 12 months of treatment with hydroxychloroquine. Treatment also resulted in significant reduction in erythrocyte sedimentation rate, serum gamma globulin, and C reactive protein values while only partial clinical improvement was noted in some patients. A more pronounced decrease of salivary IL6 and HA levels was found in the two patients in whom a reduction in the swelling of the parotid gland was noted. CONCLUSION: In this open label study of hydroxychloroquine treatment for SS a significant reduction of some salivary inflammatory markers was seen at the end of 12 months. Although during the treatment period only a partial clinical effect could be noted, the findings suggest that a double blind controlled study of hydroxychloroquine in SS is indicated.
9742868 [Primary Sjögren's syndrome with pulmonary hypertension]. 1998 May A 50-year-old woman was admitted to our hospital because of dyspnea on exertion. A chest X-ray film showed cardiomegaly and echocardiography revealed right-heart overload. Right heart catheterization studies revealed pulmonary hypertension and no signs of pulmonary embolism or vasculitis. The patient had complained of Raynaud's phenomenon since 6 years before admission. She did not notice eye or mouth dryness, but examination of a lip-biopsy specimen showed infiltration of lymphocytes into the salivary glands. Laboratory findings showed hypergammaglobulinemia and hemolytic anemia. Primary Sjögren's syndrome with pulmonary hypertension was diagnosed. Her general condition improved with steroid pulse therapy followed by oral administration of corticosteroids and cyclophosphamide.
9226178 Characterization of overt B-cell lymphomas in patients with hepatitis C virus infection. 1997 Jul 15 A pathogenetic role of the hepatitis C virus (HCV) has been hypothesized for a subset of B-cell non-Hodgkin's lymphomas (NHLs). However, the preliminary characterization of B-cell NHLs in HCV-infected individuals has been poorly addressed. In the present study, we report detailed information on 35 consecutive patients with overt B-cell NHL of recent onset and HCV infection; all patients referred to a single oncological center in Northeast Italy. Histopathologic evaluation was performed by a single reference hemopathologist, and the link with the two relevant autoimmune diseases predisposing to B-cell NHL and in which HCV has been implied, ie, "essential" mixed cryoglobulinemia (EMC) and Sjogren's syndrome, was investigated. Control groups included 122 consecutive HCV-negative patients with B-cell NHL and 464 consecutive histopathologic cases of B-cell NHL referred to the same center, as well as 127 consecutive patients with HCV infection and without lymphoma referred to a different center in the same geographical area. B-cell NHLs in HCV-infected patients frequently presented at onset (1) an extranodal localization with peculiar target organs of HCV infection (ie, the liver and major salivary glands) being significantly overrepresented; (2) a diffuse large cell histotype without any prior history of low-grade B-cell malignancy or bone marrow involvement; and (3) a weak association with a full-blown predisposing autoimmune disease, although serum autoimmune features were common and cryoglobulins were always present. Therefore, the HCV-related B-cell NHLs in this oncological series presented distinctive features compared with B-cell NHLs in HCV-negative patients, and they differed from bone marrow low-grade NHLs frequently diagnosed in HCV-positive patients with EMC. Such novel information may be relevant for future research aimed at clarifying the possible link between HCV infection, autoimmunity, nonmalignant B-cell lymphoproliferation, and overt B-cell malignancy.
9166971 Biclonal immunoglobulin M dysglobulinaemia: evolving aspects in a case of primary Sjögren 1997 Apr The observation of suggestive clinical symptoms in a patient suffering from a Gougerot-Sjögren syndrome led to a search for a cryoglobulin. Unusual physico-chemical features of this cryoglobulin were discovered, using standard electrophoresis, immunoelectrophoresis, immunofixation and electroimmunotransfer. The main unusual finding was that the cryoprecipitate was made up of a biclonal IgM kappa associated to polyclonal IgG. Therefore, we suggest that this new form of cryoglobulin be classified as a subtype IIb, thus distinguishing two subtypes in the usual classification.
9884333 Decreased angiogenesis and arthritic disease in rabbits treated with an alphavbeta3 antago 1999 Jan Rheumatoid arthritis (RA) is an inflammatory disease associated with intense angiogenesis and vascular expression of integrin alphavbeta3. Intra-articular administration of a cyclic peptide antagonist of integrin alphavbeta3 to rabbits with antigen-induced arthritis early in disease resulted in inhibition of synovial angiogenesis and reduced synovial cell infiltrate, pannus formation, and cartilage erosions. These effects were not associated with lymphopenia or impairment of leukocyte function. Furthermore, when administered in chronic, preexisting disease, the alphavbeta3 antagonist effectively diminished arthritis severity and was associated with a quantitative increase in apoptosis of the angiogenic blood vessels. Therefore, angiogenesis appears to be a central factor in the initiation and persistence of arthritic disease, and antagonists of integrin alphavbeta3 may represent a novel therapeutic strategy for RA.
9858422 Expression of stem cell factor (SCF) and SCF receptor (c-kit) in synovial membrane in arth 1998 Dec OBJECTIVE: Stem cell factor (SCF), the ligand for the SCF receptor (c-kit) expressed on precursors and mature mast cells (MC), is a major agonist for human MC (e.g., SCF induces MC development, chemotaxis, activation, proliferation of MC precursors, mediates MC adhesion, and changes MC releasability). We investigated expression of SCF and c-kit in synovial membrane with particular reference to the mechanism of local MC hyperplasia and inflammation in arthritis. METHODS: We conducted single and double labeling immunohistochemistry (ABC, APAAP, indirect immunofluorescence techniques) with antibodies to SCF, c-kit, MC tryptase, Ki-67 antigen (marker for proliferating cells), and CD68 (monocyte/macrophage marker). Synovial specimens analyzed were from 31 patients: traumatic arthritis (TrA, n=9), osteoarthritis (OA, n=12), and rheumatoid arthritis (RA, n=10). Control experiments were performed on human lung, skin, and buccal mucosa tissues, on the HMC-1 mast cell line, and isolated lung MC. Morphometry was performed by computerized image analysis. RESULTS: Synovial c-kit expression was found to be restricted to MC, whereas SCF is detected in synovial lining cells, stromal fibroblasts, monocyte/macrophages, endothelial cells, and in vascular basement membranes. SCF staining was localized to MC as well, but it was not possible to specify whether this represents SCF produced by or bound (via c-kit) to MC. In inflamed synovial membranes/areas, SCF was found to be redistributed into the extracellular matrix. Redistribution of SCF was accompanied by degranulation and/or accumulation of c-kit+ MC, the hyperplasia of which correlated positively with histologic inflammation/inflammatory cell densities, but did not appear to involve MC proliferation in situ. These findings appeared to be common for all the conditions (TrA, OA, RA) studied. CONCLUSION: In addition to the demonstration/characterization of SCF and c-kit protein expression in human synovium, results of this study suggest the hypothesis that, in arthritis, local mobilization of SCF may play a role in the development of synovial MC hyperplasia without inducing in situ proliferation of MC, and that the synovial SCF/MC c-kit system may contribute to the local nonspecific inflammatory response/arthritic flares in TrA, OA, and RA.
10955346 High dose, alternate day corticosteroids for systemic onset juvenile rheumatoid arthritis. 2000 Aug OBJECTIVE: To determine the safety and efficacy of high dose alternate day (qod) prednisone as therapy in acute systemic onset JRA (SOJRA). METHODS: A retrospective chart review was performed of all active patients with SOJRA at our institutions who began high dose qod prednisone (n = 20; 9 male, 11 female; mean age of onset 6.5 yrs, range 1.2-18.6). Patients were followed for at least one year after initiation of high dose qod prednisone. Disease activity (fever, rash, active joint count, complete blood cell count, erythrocyte sedimentation rate, ESR) and possible side effects of treatment were assessed at each visit. RESULTS: Within a mean of 2.1 months (range 1-5), systemic features (fever, rash, serositis, coagulopathy) in all patients had resolved and there was significant improvement in laboratory indices of disease activity. Doses ranged from 1 to 5.8 mg/kg qod (actual doses: 50-400 mg qod), with a mean of 3.2 mg/kg. No patient had to restart daily prednisone. The only major side effect was the development of mild cataracts in one patient. Height standard deviation scores (SDS) remained within normal range in all but 2 patients. Clinical improvement was maintained in all patients, as measured by lack of systemic symptoms, and decreased active joint count. Repeated measures of analysis of variance revealed significant improvement in all laboratory tests (white blood cell count, hemoglobin, platelet count, ESR) measured at 0, 6, and 12 months (p < 0.001). Nine of the 20 patients continued qod prednisone more than 12 months beyond the study period (mean 5.2 yrs, range 3-8). The only additional side effects were a vertebral crush fracture in one patient and possible avascular necrosis in another. Height SDS did not change significantly over the 3 year period after the initiation of qod prednisone (p > 0.05). CONCLUSION: High dose qod prednisone appears to be effective in controlling the systemic features of SOJRA and was well tolerated. Side effects attributable to corticosteroids, including growth suppression, were minimal.
9576010 Detection of a soluble form of B7-1 (CD80) in synovial fluid from patients with arthritis 1998 Apr The costimulatory molecule B7-1 (CD80) has been shown to be an important component for T cell immune responses. We have generated several monoclonal antibodies (PSRM-1, -2, -3, -6, and -7) against B7-1 using a human glycosylphosphatidylinositol-anchored B7-1 (GPI-B7-1) as an antigen. These monoclonal antibodies are able to detect B7-1 by flow cytometry, ELISA, and Western blotting. One antibody in particular, PSRM-3, blocks the CD28/CTLA-4 interaction with B7-1 and consequently blocks costimulation of T cells. The other PSRM monoclonal antibodies did not compete with PSRM-3 for recognition of B7-1 and also failed to block B7-1 interaction with CTLA-4 and CD28, indicating that these antibodies bind to different epitopes. PSRM-3 and -7 detect phosphatidylinositol-specific phospholipase C-released soluble GPI-B7-1 in a sandwich ELISA. We used this sandwich ELISA to assay for the presence of a soluble form of B7-1 in synovial fluids of arthritis patients. By sandwich ELISA, B7-1 was detected in the synovial fluid of 5/11 patients with rheumatoid arthritis, 5/5 patients with osteoarthritis, and 2/6 patients with other forms, including crystalline-induced arthritis. The presence of soluble B7-1 was confirmed by immunoprecipitation using PSRM-3-coupled Sepharose beads. The source and function of soluble B7-1 are unknown at present; it is possible, however, that the soluble form of B7-1 molecule may play a local immunoregulatory role which may suppress or induce inflammation depending upon whether it interacts with the T cell costimulatory CD28 molecule or the negative signaling CTLA-4 molecule.
11246684 Hypocomplementemic urticarial vasculitis, jaccoud's arthropathy, valvular heart disease, a 2001 Feb We describe a patient who, during 29 years of observation, manifested polyarthralgia and polyarthritis leading to progressive deformity of the joints of hands and feet (without loss of cartilage or erosion of bone); persistent urticaria made worse by cold and accompanied by hypocomplementemia; and progressive cardiac valvular disease with mitral and aortic stenosis and regurgitation. In 1996, she developed subglottic tracheal stenosis that resolved by the end of 1997 without a change in treatment, which has consisted of low dose azathioprine, glucocorticoid, and nonsteroidal antiinflammatory drugs. Tests for cryoprecipitable protein, antineutrophil cytoplasmic antibodies, antinuclear antibody, and rheumatoid factor were negative. Skin biopsy was consistent with "leukocytoclastic vasculitis." The pathogenesis of this remarkable combination of syndromes is unknown.
11848326 Outbreak of polyarthritis with pyrexia in Western Rajasthan. 2001 Oct AIM: To establish the etiology of recent out break of polyarthritis which occurred in Kanvari village of Churu district of Rajasthan in August, 1999. METHODOLOGY: Forty eight patients of polyarthritis were studied by Hb, TDLC, ESR, CRP, throat swab Gram's stain and culture, blood culture, ASO titer, rheumatoid factor, Rose Bengal plate agglutination test, standard tube agglutination test for brucellosis, widal test, urine examination, X-ray chest, ECG and X-ray of the affected joint. RESULTS: Forty eight patients presented with acute polyarthritis with low grade fever of 1-2 week duration. Most common joint involved was sacroiliac joint (52.08%). Most of patients had multiple joint involvement (93.75%). The Rose Bengal plate agglutination test and standard tube agglutination test for brucella were positive in high titres in 44 (91.60%) patients. All the patients were treated with therapy for brucellosis and followed up for 12 weeks and responded well without complications. CONCLUSION: In case of polyarthritis possibility of brucellosis should always be kept in mind.