Very early 'Rheumatoid' arthritis cohorts: limited by selection.
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Arthritis, Rheumatoid↗
Biomedical subjects
Murat Inanc
Publications and source records attributed to Murat Inanc.
Accuracy of anti-ribosomal P protein antibody testing for the diagnosis of neuropsychiatric systemic lupus erythematosus: an international meta-analysis.
OBJECTIVE: To quantitatively evaluate the diagnostic accuracy of antibodies to ribosomal P proteins (anti-P) for neuropsychiatric systemic lupus erythematosus (NPSLE) in general, for psychosis, mood disorder, or both, and for other diffuse manifestations. METHODS: This international meta-analysis combined standardized data from 1,537 lupus patients contributed by 14 research teams. Weighted estimation of sensitivity and specificity with fixed-effects and random-effects models, as well as summary receiver operating characteristic (SROC) curve analysis, was used to summarize test performance. The robustness of the overall estimates was examined in sensitivity analyses that included additional studies published up to November 1, 2004 in the Medline, EMBase, and Cochrane databases. RESULTS: Combining the data from the 14 teams, the weighted sensitivity and specificity estimates for the diagnosis of NPSLE were 26% (95% confidence interval [95% CI] 15-42%) and 80% (95% CI 74-85%), respectively. For psychosis, mood disorder, or both, the sensitivity and specificity were 27% (95% CI 14-47%) and 80% (95% CI 74-85%), respectively. For other diffuse manifestations, the sensitivity was 24% (95% CI 12-42%), and the specificity was 80% (95% CI 73-85%). The proportion of patients with anti-P antibodies did not vary markedly across different presentations of NPSLE. Between-study heterogeneity was substantial, but the SROC curves were consistent with the weighted estimates. In further analyses that included another 24 published studies, only the sensitivity for psychosis and/or mood disorder was slightly improved, but it was still suboptimal (42% [95% CI 30-53%]); the specificity remained essentially the same (81% [95% CI 76-85%]). CONCLUSION: Anti-P antibody testing has limited diagnostic value for NPSLE, and it is not helpful in differentiating among various disease phenotypes.
Antibodies↗
Anti-CCP and antikeratin antibodies in rheumatoid arthritis, primary Sjögren's syndrome, and Wegener's granulomatosis.
The objective of this study was to investigate the frequency of antibodies against cyclic citrullinated peptides (anti-CCP) and keratin (AKA) in patients with rheumatoid arthritis (RA) as well as in patients with primary Sjögren's syndrome (pSS) and Wegener's granulomatosis (WG), who may present with rheumatoid factor (RF)-positive arthritis. The study group consisted of 46 patients with RA (26 patients were negative for RF), 32 with pSS, 22 with WG, and 40 healthy controls. The RF, anti-CCP, and AKA were detected in serum using the latex agglutination test, enzyme-linked immunosorbent assay, and indirect immunofluorescence, respectively. The agreement between those tests was evaluated by kappa test. No positive result for AKA was detected in pSS and WG patients, and anti-CCP was found in only one patient with pSS. The results of kappa tests were low, varying between 0.25 (RF-anti-CCP) and 0.02 (RF-AKA). The sensitivity and specificity values were 43 and 44% for RF, 65 and 98% for anti-CCP, and 58 and 100% for AKA, respectively, in RA patients. In the RF-negative RA group, AKA was found to have a high frequency (55%) in comparison to anti-CCP (38%). Seropositivity was found to be 87% for any one of the three autoantibodies tested in RA patients. With a higher specificity, values for RA, anti-CCP, and AKA seem to be helpful for the differential diagnosis of patients with RF-positive arthritis, which may include patients with WG and pSS, and screening of all three antibodies may increase the diagnostic performance.
Adult↗
Assessment of cardiac structure and left atrial appendage functions in primary antiphospholipid syndrome: a transesophageal echocardiographic study.
BACKGROUND AND PURPOSE: Although thromboembolic events are the major complication of primary antiphospholipid syndrome (PAPS), cardiac involvement is commonly present. Left atrial appendage (LAA) is recognized as an important source for thrombus formation and thromboembolism. The purpose of the study was to assess the structure and function of LAA with transesophageal echocardiography (TEE) in PAPS patients. METHODS: Thirty-one PAPS patients (22 women, mean age 36+/-9 years) in sinus rhythm and 31 (17 women, mean age 37+/-7 years) controls with normal TEE examination were investigated. RESULTS: Eighty-four percent of the PAPS patients had functional and structural valvular defect predominantly in the mitral valve. Valvular lesions were especially frequent in PAPS patients with a history of cerebrovascular events, patients with history of arterial thrombosis (91.6%), and patients with high titers of IgG anticardiolipin antibodies (100%). Intracardiac thrombus was present in 5 patients and in 1 of them it was located in LAA. The structure of LAA was similar between groups. Left atrial appendix ejection fraction (51.8+/-4 versus 48.6+/-5.5%; P<0.05) and LAA peak outflow velocity (87+/-10.9 versus 80.6+/-10.3 cm/s; P=0.02) was significantly higher in PAPS group compared with controls. In PAPS patients with mitral regurgitation (MR), LAA outflow peak velocity (84.3+/-10 versus 98.6+/-6.5 cm/s; P=0.002) and LAA inflow peak velocity (67.8+/-10.5 versus 80.8+/-8.6 cm/s; P=0.009) were significantly lower compared with PAPS patients without MR. CONCLUSIONS: It was concluded that disease process in PAPS frequently involved cardiac valves especially mitral valve but spared LAA function. LAA function was normal, but intracardiac thrombus was present in 5 patients and 1 of them was located in LAA. MR in PAPS patients seems to impair LAA function.
Adolescent↗
Efficacy of infliximab in the treatment of uveitis that is resistant to treatment with the combination of azathioprine, cyclosporine, and corticosteroids in Behçet's disease: an open-label trial.
OBJECTIVE: To investigate the effect of infliximab on the frequency of uveitis attacks and the visual prognosis in male patients with Behçet's disease in whom uveitis was resistant to combination therapy with corticosteroids, azathioprine, and cyclosporine. METHODS: The study group comprised patients who were receiving combination therapy but experienced at least 2 attacks of posterior uveitis/panuveitis or retinal vasculitis during the 6-month period prior to enrollment. Infliximab infusions (5 mg/kg) were administered at weeks 0, 2, 6, and 14. Weeks 0-22 were defined as the infusion period, and weeks 23-54 were defined as the observation period. Patients continued to receive azathioprine and corticosteroids, but cyclosporine was discontinued after the screening visit. The primary outcome measures were the absence of uveitis attacks during the infusion period (remission), and the absence of uveitis attacks throughout the study period (sustained remission). RESULTS: Thirteen patients were enrolled in the study. Thirty-two uveitis attacks involving the posterior segment occurred during the previous-treatment period. During the infusion period, 4 patients (30.8%) remained attack-free, and 9 patients had a total of 13 uveitis attacks. Ten of these attacks (76.9%) occurred at either week 14 or week 22. One of 13 patients fulfilled the definition of sustained remission, and the remaining 12 patients had a total of 36 uveitis attacks during the observation period. The mean number of uveitis attacks and daily corticosteroid doses were significantly lower during the infusion period than during the previous-treatment period or the observation period. Although potential visual acuity was regained following infliximab infusion, this beneficial effect was not preserved until week 54. None of the patients experienced a serious adverse event. CONCLUSION: The results of this trial suggest that infliximab is effective in suppressing the occurrence of uveitis attacks, has a corticosteroid-sparing effect, and has favorable implications for the visual prognosis of patients with resistant Behçet's uveitis.
Adrenal Cortex Hormones↗
Bilateral acquired Brown syndrome in systemic scleroderma.
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Diplopia↗
Systemic necrotizing vasculitides in Turkey: a comparative analysis of 40 consecutive patients.
OBJECTIVE: The aim of this study was to analyze and compare the demographic and clinical features and prognosis of patients with different systemic necrotizing vasculitides (SNV) in Turkey. PATIENTS AND METHODS: Twenty-three patients with Wegener's granulomatosis (WG), 15 with polyarteritis nodosa (PAN), and two with Churg-Strauss syndrome were included in the study. The clinical and laboratory features of patients with WG and PAN were compared, and survival analysis was performed for the WG patients. RESULTS: Twenty-one patients with WG had systemic disease involving kidneys, and two had localized disease. Fifteen patients were placed in the PAN group, 12 of whom were classified as having classic PAN and three with microscopic polyangiitis. Median follow-up time was 37 months (range 1-81) for WG patients and 41 months (range 5-132) for the PAN group. Upper respiratory tract, pulmonary, and renal involvement were significantly more frequent in the WG group than in PAN. Peripheral nervous system involvement was more frequent in the PAN group. In WG, survival was calculated as 59% at 35 months. High initial vasculitis damage index scores were found to be predictive for mortality. CONCLUSION: This study revealed that the most frequent type of SNV was WG in a tertiary rheumatology setting in Turkey. There was initial organ damage in most of the patients, frequently caused by severe renal involvement. In contrast to other published series, overt cardiovascular and gastrointestinal involvement were not observed in our patients with SNV.
Anti-Inflammatory Agents↗
A comparison of clinical findings of familial Mediterranean fever patients with and without amyloidosis.
OBJECTIVE: This study investigates the clinical and demographic characteristics of familial Mediterranean fever (FMF) patients with and without amyloidosis. PATIENTS AND METHODS: The clinical data of 503 patients with FMF (females:males 250:253) were reviewed. Fifty of these patients had amyloidosis (f:m 23:27). RESULTS: The ages of attack onset in patients with and without amyloidosis were 7.8+/-6.2 and 11.1+/-8.5, respectively (P<0.05). The time between disease onset and diagnosis was longer in patients with amyloidosis than those without (187.6+/-99.4 months and 132.5+/-110.2 months, respectively, P<0.001). More patients in the amyloidosis group had positive family histories of FMF (68% vs 54%, P<0.05). The frequencies of chest pain (78% vs 51%, P<0.001), arthritis ( 80% vs 60%, P<0.01), and erysipelas-like erythema (44% vs 16%, P<0.001) were higher in the amyloidosis group. CONCLUSION: In the amyloidosis group, FMF-related manifestations of chest pain, arthritis, and erysipelas-like erythema are more frequent. Our results also support that long periods between disease onset and diagnosis are associated with a high risk of developing amyloidosis.
Adolescent↗
An unusual overdose of leflunomide in a patient with rheumatoid arthritis.
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Arthritis, Rheumatoid↗
Experience with monthly, high-dose, intravenous immunoglobulin therapy in patients with different connective tissue diseases.
We aimed to investigate the efficacy and safety profile of high-dose intravenous immunoglobulin (HD-IVIG) therapy in patients with severe systemic lupus erythematosus (SLE), inflammatory muscle disease (IMD), Wegener's granulomatosis (WG), and/or concurrent infection who failed to respond to standard therapies. We evaluated the records of eight patients with SLE, eight with IMD, and four with WG who were treated with HD-IVIG (2 g/kg per month for 1-12 months) for active disease in 19 patients and concurrent infection in three (mycobacterial in two with SLE and cytomegaloviral in one with WG). Systemic lupus erythematosus disease activity index (SLEDAI) scores before and after HD-IVIG were statistically analysed. Remission was achieved in 14 cases (70%). The SLEDAI scores significantly decreased in patients with SLE (P=0.02). No serious side effect was observed. High-dose IVIG may be used as an adjunctive treatment in connective tissue diseases that do not respond to standard therapies or as alternative treatment for patients with concurrent severe infections or for whom immunosuppressives are contraindicated.
Adolescent↗
Thrombotic risk in patients submitted to splenectomy for systemic lupus erythematosus and antiphospholipid antibody syndrome-related thrombocytopenia.
Background: Splenectomy has been performed to treat refractory autoimmune thrombocytopenia. However, some reports have suggested that an increased risk of thrombosis could be present in splenectomized patients. This study aims to evaluate the possibility of an increased risk of thrombosis after splenectomy in patients with systemic lupus and antiphospholipid syndrome. Methods: Thrombotic-related events in patients with systemic lupus erythematosus (SLE) and/or primary antiphospholipid syndrome (PAPS), before and after splenectomy for severe thrombocytopenia, were compared. Clinical data, laboratory investigations, and anticoagulation or antiaggregation treatment data were collected from the notes of outpatients attending three European centers. Results: Twenty patients who had had a splenectomy were identified: eight with SLE, five with PAPS, and seven with SLE and APS. The mean time between diagnosis and splenectomy was 3.1 years and mean follow-up was 6.5 years. There were no differences in anticardiolipin antibody titers, lupus anticoagulant, anti-DNA or anti-nuclear antibodies before and after surgery. The incidence of venous events before and after splenectomy was not significantly different. There was a trend towards an increase in the total number of arterial events post-splenectomy. In aCL-positive patients, and in the pre-splenectomy period, the total number of miscarriages was higher (p=0.017), as was the number of patients who had had a miscarriage (p=0.025). Conclusions: The total risk of thrombosis in patients with PAPS and SLE was not increased after splenectomy, but there was a trend towards an increase in the number of arterial events. Splenectomy induced long-term remission of thrombocytopenia (partial or complete) in all patients.
Journal Article↗
Fc receptor gamma subunit polymorphisms and systemic lupus erythematosus.
OBJECTIVE: To investigate the possible association between Fc receptor (FcR) gamma polymorphisms and systemic lupus erythematosus (SLE). METHODS: We have investigated the full FcR gamma gene for polymorphisms using polymerase chain reaction (PCR)-single strand conformational polymorphism and DNA sequencing. The polymorphisms identified were genotype using PCR-restriction fragment length polymorphism. Systemic lupus erythematosus cases and controls were available from 3 ethnic groups: Turkish, Spanish and Caucasian. The study was conducted in the year 2001 at the Arthritis Research Campaign, Epidemiology Unit, Manchester University Medical School, Manchester, United Kingdom. RESULTS: Five single nucleotide polymorphisms were identified, 2 in the promoter, one in intron 4 and, 2 in the 3' UTR. Four of the 5 single nucleotide polymorphisms (SNPs) were relatively common and investigated in the 3 populations. Allele and genotype frequencies of all 4 investigated SNPs were not statistically different between cases and controls. CONCLUSION: Fc receptor gamma gene does not appear to contribute to SLE susceptibility. The identified polymorphisms may be useful in investigating other diseases where receptors containing the FcR gamma subunit contribute to the pathology.
Alleles↗
A patient with classical polyarteritis nodosa evolving into end stage renal failure.
Classical polyarteritis nodosa (c-PAN) is a form of systemic necrotizing vasculitis mainly affecting medium-size arteries, is not associated with renal glomerular disease and acute renal failure. Perirenal hematoma can be seen in up to fifty percent of c-PAN patients and minority of them develop mild renal impairment. Herein, we describe a 34-year-old male with c-PAN who presented with rapidly progressive renal failure and evolved into end stage renal disease.
Acute Kidney Injury↗
Coexistence of homozygous factor V Leiden mutation and antiphospholipid antibodies in two patients presented with Budd-Chiari syndrome.
Two female patients are reported, who presented with Budd-Chiari syndrome (hepatic vein thrombosis), and were found to have both, antiphospholipid antibodies and homozygous factor V Leiden mutation. Both patients also had recurrent fetal losses, as well as splenic and portal vein thrombosis. The coexistence of homozygous factor V Leiden mutation and antiphospholipid antibodies in patients with Budd-Chiari syndrome is extremely rare. The interaction of antiphospholipid antibodies and factor V Leiden mutation in the pathogenesis of antiphospholipid syndrome and their contribution to Budd-Chiari syndrome are discussed.
Abortion, Habitual↗
Transforming growth factor-12 polymorphism and systemic lupus erythematosus.
OBJECTIVE: To determine whether transforming growth factor-beta2 (TGF-beta2) gene polymorphism is associated with systemic lupus erythematosus (SLE) susceptibility. TGF-beta is a multifunctional family of cytokines important in tissue repair, inflammation and immunoregulation. SLE is thought to be a T cell dependent autoimmune disorder with T cell dysfunction. Due to its known suppressive effects on interleukin 2 dependent T cell growth, TGF-beta2 is considered to be a candidate SLE susceptibility gene. Furthermore, SLE has been linked with a region to which the TGF-beta2 gene has been mapped. METHODS: Association studies were performed in 3 case-control populations, from Spain. Turkey, and UK, using a TGF-beta2 5'-untranslated region (5'-UTR) 4 base pair (bp) insertion polymorphism. Genotyping was performed using fluorescent labeled polymerase chain reaction product sizing. Results. No significant differences were detected in TGF-beta2 5'-UTR polymorphism allele frequencies between SLE patients and matched controls in the 3 populations studied. CONCLUSION: The 4 bp insertion polymorphism within the TGF-beta2 gene does not appear to be associated with SLE. However, this does not rule out the possible involvement of TGF-beta2 in the disease pathogenesis.
Alleles↗