Identification of susceptibility loci for Takayasu arteritis through a large multi-ancestral genome-wide association study.

Takayasu arteritis is a rare inflammatory disease of large arteries. We performed a genetic study in Takayasu arteritis comprising 6,670 individuals (1,226 affected individuals) from five different populations. We discovered HLA risk factors and four non-HLA susceptibility loci in VPS8, SVEP1, CFL2, and chr13q21 and reinforced IL12B, PTK2B, and chr21q22 as robust susceptibility loci shared across ancestries. Functional analysis proposed plausible underlying disease mechanisms and pinpointed ETS2 as a potential causal gene for chr21q22 association. We also identified >60 candidate loci with suggestive association (p < 5 × 10-5) and devised a genetic risk score for Takayasu arteritis. Takayasu arteritis was compared to hundreds of other traits, revealing the closest genetic relatedness to inflammatory bowel disease. Epigenetic patterns within risk loci suggest roles for monocytes and B cells in Takayasu arteritis. This work enhances understanding of the genetic basis and pathophysiology of Takayasu arteritis and provides clues for potential new therapeutic targets.

A shared motif of hla-dpb1 affecting the susceptibility to pr3-anca positive granulomatosis with polyangiitis: comparative analysis of a Turkish cohort with matched healthy controls.

We aimed to analyse the distribution of HLA Class 2 genotypes which were reported among the genetic risk factors for ANCA-associated vasculitis (AAV) among Turkish patients in comparison with healthy subjects and previously reported data of AAV cohorts. Ninety-eight patients (F/M: 47/51 and mean age: 49 ± 1.14) were enrolled in the study and records of gender and birthplace-matched 196 healthy kidney donors were used as the control group. Patients were classified according to the clinical subgroups and ANCA serotypes (MPO-AAV, PR3-AAV). DNA was isolated from venous blood from all patients, and high-resolution HLA Class 2 genotyping was carried out by using NGS-Omixon Holotype HLA Kit. The frequencies of HLA-DQB1*03:03, - *06:04, and -DPB1*13:01, -*16:01 and -*66:01:00 alleles were significantly higher, and the frequencies of HLA-DQB1*02:02, -DPB1*02:01 and -*04:01 alleles were lower in the PR3-AAV subgroup (n = 53) compared to the controls. Comparison of amino acid sequences of the associated HLA-DPB1 alleles revealed the sequence of D-E-A-V in risk alleles replaced with the G-G-P-M sequence in protective alleles between 84 and 87th positions. Structural analysis of the HLA-DPB1*02:01 showed that this shared position is in the contact area between HLA-DP α and ß chains and within pocket 1 of the antigen-binding groove. First HLA genotyping analysis in Turkish AAV patients revealed a negative correlation between PR3-ANCA positivity and certain HLA-DPB1 alleles contradictory to the results reported from European cohorts. Known functional effects of D-E-A-V sequence on HLA-DPB1 support the importance of our finding, but further studies are needed to reveal its pathogenic mechanisms.

Which joints and why do rheumatologists scan in rheumatoid arthritis by ultrasonography? A real life experience.

OBJECTIVES: Ultrasonography (US) has been demonstrated to improve assessment of synovitis and disease activity in rheumatoid arthritis (RA). However, the utility and feasibility of US in RA in clinical practice in real life is not known. We aimed to investigate: i) the indications for performing US in RA in daily practice; and ii) whether the number of scanned joints varies according to the purpose. METHODS: Consecutive patients who had a US scan either for diagnosis or follow-up for RA from 5 centres were recruited. The sonographers were asked to mark the joints that had a US scan and grade their findings. Descriptive analysis was applied to find out the sites and the number of joints scanned and compared according to the indications of US. RESULTS: Two hundred consecutive patients were recruited. The most common indication was assessing disease activity (48.5%) followed by diagnosis (45.5 %). Wrists (66%) and MCPs (63.5) were the most frequently scanned joints followed by knees (26%), PIPs (20%). The number of joints scanned by US was significantly higher when performed for diagnostic purposes as compared to assessing disease activity and guidance for injections (p=0.001). CONCLUSIONS: The current data highlight differences between the numbers of joints for which that the clinician feels necessary to perform US in real life. This observation may be a guide when providing recommendations regarding which joints need to be scanned according to the indication.

Remarkable damage along with poor quality of life in Takayasu arteritis: cross-sectional results of a long-term followed-up multicentre cohort.

OBJECTIVES: We aimed to assess the outcome of a large Takayasu arteritis (TAK) cohort using the vasculitis damage index (VDI) and quality of life (QoL) scale, tools which have been validated for vasculitis. METHODS: Disease activity, damage and QoL were cross-sectionally evaluated in 165 TAK patients from 6 centres. SF-36 were applied to 51 age-matched healthy controls (HC). Persistent activity for ≥6 months was considered as treatment resistance (r-TAK). The correlation between VDI, clinical characteristics and mental (MCS)/physical (PCS) component scores of SF-36 were analysed. SF-36 and VDI scores were compared between TAK subgroups and HC. RESULTS: The median age, follow-up time and disease duration were 40 (17-68), 60 (6-384), and 72 (6-396) months, respectively. 35% of them were r-TAK. VDI scores (VDIs) in TAK 4 (1-12) were mainly due to the disease itself [4 (1-10)]. VDIs in r-TAK were significantly higher than nr-TAK [5 (2-12) vs. 3 (2-10), p<0.001)]. In the TAK patients, MCS and PCS were found as 43±10 and 38±11, respectively. A high proportion of poor MCS (70%) and PCS (80%) were demonstrated in TAK. A significantly negative but weak correlation was observed between VDI and MCS (p=0.003, r=-0.23), PCS (p<0.001, r=-0.34). Higher VDIs were detected in patients with PCS <50 [5 (1-12) vs. 2 (1-6) p<0.001)]. SF-36 score was significantly lower in TAK than HC. CONCLUSIONS: Disease-related damage mainly caused by peripheral vascular involvement was more predominant than treatment-related damage without reaching the level of severe damage scores, but contributing to poor QoL, in the TAK cohort.

Criteria sets for primary Sjogren's syndrome are not adequate for those presenting with extraglandular organ involvements as their dominant clinical features.

Patients with primary Sjogren's syndrome (pSS) may go undiagnosed or be misclassified due to the insidious nature and wide spectrum of the disease. The available several classification criteria emphasize glandular findings. We aimed to analyze the efficiency of various classification criteria sets in patients diagnosed on the clinical basis by expert opinion and to compare those pSS patients who fulfilled these criteria with those who did not. This is a multicenter study in which 834 patients from 22 university-based rheumatology clinics are included. Diagnosis of pSS was made on the clinical basis by the expert opinion. In this study, we only interviewed patients once and collected available data from the medical records. The European criteria, American-European Consensus Group (AECG) and American College of Rheumatology (ACR) Sjogren's criteria were applied. Majority of the patients were women (F/M was 20/1). The median duration from the first pSS-related symptom to diagnosis was significantly shorter in men (2.5 ± 2.3 vs 4.3 ± 5.9 years) (p = 0 < 0.016). When the European, AECG and ACR Sjogren's criteria were applied, 666 patients (79.9%) satisfied at least one of them. In total, 539 patients (64.4%) satisfied the European, 439 (52.6%) satisfied the AECG, and 359 (43%) satisfied the ACR criteria. Among the entire group, 250 patients (29.9%) satisfied all and 168 (20.1%) met none of the criteria. The rates of extraglandular organ involvements were not different between patients who met at least one of the criteria sets and those who met none. There is an urgent need for the modification of the pSS criteria sets to prevent exclusion of patients with extraglandular involvements as the dominant clinical features.

Identification of multiple genetic susceptibility loci in Takayasu arteritis.

Takayasu arteritis is a rare inflammatory disease of large arteries. The etiology of Takayasu arteritis remains poorly understood, but genetic contribution to the disease pathogenesis is supported by the genetic association with HLA-B*52. We genotyped ~200,000 genetic variants in two ethnically divergent Takayasu arteritis cohorts from Turkey and North America by using a custom-designed genotyping platform (Immunochip). Additional genetic variants and the classical HLA alleles were imputed and analyzed. We identified and confirmed two independent susceptibility loci within the HLA region (r(2) < 0.2): HLA-B/MICA (rs12524487, OR = 3.29, p = 5.57 × 10(-16)) and HLA-DQB1/HLA-DRB1 (rs113452171, OR = 2.34, p = 3.74 × 10(-9); and rs189754752, OR = 2.47, p = 4.22 × 10(-9)). In addition, we identified and confirmed a genetic association between Takayasu arteritis and the FCGR2A/FCGR3A locus on chromosome 1 (rs10919543, OR = 1.81, p = 5.89 × 10(-12)). The risk allele in this locus results in increased mRNA expression of FCGR2A. We also established the genetic association between IL12B and Takayasu arteritis (rs56167332, OR = 1.54, p = 2.18 × 10(-8)).

Capillaroscopic findings and vascular biomarkers in systemic sclerosis: Association of low CD40L levels with late scleroderma pattern.

OBJECTIVE: To determine the relationship between vascular biomarkers reflecting the vascular injury and neoangiogenesis with capillaroscopic changes in systemic sclerosis (SSc). METHODS: Nailfold video-capillaroscopy (NVC) was performed qualitatively (early, active and late scleroderma patterns) in 72 SSc patients (66 female) fulfilling ACR/EULAR (2013) criteria. Serum samples of patients were collected and analysed by flow cytometer with multiplex kits of sCD40L, tPA, MCP-1, sE-selectin, IL-8, IL-6, VEGF, sP-selectin, TGF-ß1 and VCAM at the same time with NVC. RESULTS: Compared to healthy subjects; tPA (p=0.02), MCP-1 (p=0.001), sE-selectin (p=0.008) and TGF-ß1 (p=0.001) levels were significantly higher, however sP-selectin (p=0.011) and IL-8 (p=0.001) levels were lower in SSc patients. SSc patients were defined according to NVC patterns as 'early' (n=10), 'active' (n=37) and 'late' (n=25). According to NVC patterns of SSc patients, only sCD40L levels were significantly lower in the 'late' group (p=0.039). The other markers were similar among NVC groups. CONCLUSIONS: NVC is a useful method for investigating the vascular pathogenesis and severity of SSc. Although the levels were similar to healthy controls in patients with early/active NVC patterns, there were lower sCD40L serum levels in patients with late NVC pattern. CD40L may have a role in the early/active phase of vascular involvement.

The relationship between vascular biomarkers and disease characteristics in systemic sclerosis: elevated MCP-1 is predominantly associated with fibrotic manifestations.

OBJECTIVES: To determine the relationship between vascular biomarkers reflecting the vascular injury and organ involvement in systemic sclerosis (SSc). METHODS: Seventy-two SSc patients (66 female) fulfilling 2013 ACR/EULAR Criteria were evaluated. Serum samples of patients were collected for flow-cytometric analysis of sCD40L, tPA, MCP-1, sE-selectin, IL-8, IL-6, VEGF, sP-selectin, TGF-ß1 and VCAM levels (Bender MedSystems) in SSc patients and 20 healthy controls. Results were compared with Pearson chi-square/Fisher's and Mann Whitney tests. RESULTS: Levels of MCP-1 were found to be elevated in patients with diffuse cutaneous SSc, flexion contractures, FVC<80%, DLCO<80%, pulmonary fibrosis and high acute phase response (p=0.002, p=0.005, p=0.045, p=0.005, p=0.036, p=0.006, respectively), TGF-ß1 in patients receiving immunosupressives (p=0.001), sE-selectin in patients with high acute phase response (p=0.028), sCD40L in patients with lcSSc (p=0.011) and smoking habitus (p=0.032). MCP-1 and sE-selectin levels were correlated with disease activity score (r=0.243, p=0.040 and r=0.303, p=0.010), MCP-1 and TGF-ß1 were correlated with severity of pulmonary involvement (r=0.323, p=0.006 and r=0.312, p=0.008). CONCLUSIONS: MCP-1 was the prominent biomarker correlated with the manifestations related to fibrosis, disease activity score and severity of pulmonary involvement. Treatment and smoking may have an effect on cytokine profile. Vascular biomarkers can be used to predict the characteristics and severity of SSc warranting prospective studies.

The relationship between nailfold capillaroscopic assessment and telangiectasia score with severity of peripheral vascular involvement in systemic sclerosis.

OBJECTIVES: To determine the association of nailfold video-capillaroscopy (NVC) findings and telangiectasia score with digital ulcer (DU) history and severity of peripheral vascular involvement (PVI) in systemic sclerosis (SSc). METHODS: Fifty-nine SSc patients fulfilling Leroy & Medsger criteria were evaluated including telangiectasia score, disease activity and severity scores. NVC was performed according to qualitative (early, active and late patterns) and semi-quantitative assessments. RESULTS: When DU+ and DU- groups were compared; the mean score of capillary number (CN) was 2.0±0.5 vs. 1.4±0.7 (p<0.001), irregularly enlarged capillaries (IEC) was 1.8±0.6 vs. 1.4±0.7 (p<0.05), microangiopathy evolution score (MES) was 2.5±1.5 vs. 1.8±1.0 (p<0.05) and 'early' pattern was significantly less frequent in DU+ patients (1 vs. 9, p=0.016). The frequency of severe-PVI (Medsger severity score of 2-4) was 22% in females (12/54) and 80% in males (4/5). When severe and non-severe groups were compared; the mean score of CN was 2.1±0.4 vs. 1.5±0.7 (p<0.001), MES was 2.8±1.6 vs. 1.8±1.1 (p<0.05) and 'early' pattern was significantly less frequent in patients with severe PVI (0 vs. 9, p=0.049). The mean values of telangiectasia score were similar between groups. CONCLUSIONS: DU history and severe PVI in SSc were associated with capillary loss and microangiopathy. 'Early' NVC pattern was very rare in patients with DU history and was not found in severe PVI. Severe PVI in males was more frequent than females. Telangiectasia scores were not found to be related to PVI. NVC may be a helpful method in the assessment of SSc patients for PVI prognosis, warranting prospective studies.

Small bowel mucosal damage in familial Mediterranean fever: results of capsule endoscopy screening.

OBJECTIVE: Familial Mediterranean fever (FMF) is the most common form of autoinflammatory diseases. We aimed to evaluate the small bowel mucosa by capsule endoscopy (CE) in FMF patients for investigation of other possible causes of abdominal pain. MATERIAL AND METHODS: The study group consisted of 41 patients with FMF. A standard questionnaire was used to record the gastrointestinal symptoms, other clinical findings, Mediterranean fever gene (MEFV) mutations, and history of medications including non-steroidal anti-inflammatory drugs (NSAIDs). Gastroscopy, colonoscopy and small bowel CE were performed in all patients, and biopsies were taken from terminal ileum and duodenum. RESULTS: The mean age of the patients was 34 ± 11 years, 63% of them were female, and 76.5% of them were carrying MEFV exon 10 mutations. Only one patient used NSAIDs in addition to colchicine. In endoscopic investigations, gastric erosion was detected in only one patient, and no significant findings were detected in colonoscopy. CE showed small bowel mucosal defects in 44% (erosions in 26.8%, ulcer in 17.1%) and edema in 29.3% of the patients. Most (64%) of the ulcer and erosions were localized to jejunum, and only 24% were in ileum. Mitotic changes as an indirect finding of colchicine toxicity were not different from the changes observed in samples of independent group of patients with irritable bowel syndrome. CONCLUSION: Mucosal defect was observed in half of the FMF patients, which may be associated with underlying inflammation or chronic colchicine exposure. Detection of nonspecific chronic inflammation without mitotic changes supports that mucosal defects may be associated with the autoinflammatory process.

PDCD1 polymorphisms are not associated with Takayasu's arteritis in Turkey.

OBJECTIVES: Takayasu's arteritis (TA) is a chronic arterial inflammation of unknown etiology involving mainly the aorta and its major branches. Based on the associations of programmed death-1 (PD-1) protein encoding gene (PDCD1) with connective tissue diseases and vasculitides, PDCD1 polymorphisms are studied for susceptibility to TA in this study. METHODS: The study group is made up of TA patients (n=229) fulfilling the 1990 ACR classification criteria and compared to 193 healthy controls (HC). PD-1.3, PD-1.5 and PD-1.6 single nucleotide polymorphisms of PDCD1 gene are genotyped by polymerase chain reaction and restriction analysis (PCR-RFLP). RESULTS: The distribution of PD-1.5 polymorphism in TA patients and HC revealed a similar presence of TT genotype in patients and controls (13.3% vs. 11.4%). PD-1.3 and PD-1.6 were less polymorphic and did not differ between the groups. Rare AA genotype of PD-1.3 (1.4% vs. 1.0%) and AG genotype of PD-1.6 was again similarly (22.4% vs. 19.2%) present in TA and HC. CONCLUSIONS: PD-1.3, 1.5 and 1.6 polymorphisms of PDCD1 gene, which were shown to be associated with various autoimmune disorders and vasculitides, are not associated with a susceptibility to TA in Turkish population.

Serologic response to Epstein-Barr virus antigens in patients with systemic lupus erythematosus: a controlled study.

Previous studies showed a link between systemic lupus erythematosus (SLE) and Epstein-Barr virus (EBV) infection. We sought to determine the features of serologic response to EBV in SLE patients and whether this response differs from those of systemic sclerosis (SSc) and primary antiphospholipid syndrome (PAPS) patients as well as healthy individuals. Sera from 198 consecutive SLE patients have been tested to detect IgG antibodies to EA/D, EBNA-1, VCA P18 and for comparison, cytomegalovirus (CMV) using commercially available ELISA kits (Trinity Biotech, USA). Forty-six SSc patients and 38 PAPS patients were enrolled as diseased control groups and sixty-five individuals as healthy controls. Significantly more SLE (54%, P = 0.001, OR 5.77, 95% CI 2.8-11.6), SSc (41.3%, P = 0.005, OR 3.4, 95% CI 1.4-8.2) and PAPS sera (36.8%, P = 0.023, OR 2.86, 95% CI 1.14-7.22) reacted against EA/D than healthy controls (16.9%). The mean age of anti-EA/D-positive SLE patients was significantly higher, and their disease duration was longer compared to anti-EA/D-negative SLE patients (41 ± 14 vs. 33.8 ± 10.8 years, P < 0.001 and 100 ± 73 vs. 71 ± 62 months, P = 0.003). In SLE patients, EA/D reactivity was associated with Raynaud's phenomenon and the presence of any anti-ENA antibodies. Although it did not reach a statistical significance, anti-EBNA-1 reactivity was slightly lower in patients with SLE. The frequency of anti-CMV Ig G positivity was found significantly higher in SLE patients (100%) when compared to patients with SSc (95.7%), PAPS (94.7%) and healthy controls (95.4%) (P = 0.035, P = 0.025 and P = 0.015 respectively). Our results support the proposed link between EBV and SLE. The finding that SSc and PAPS patients also have increased frequency of anti-EA/D response has revealed that this immune interaction may not be unique to patients with SLE, and there may be a common mechanism involving EBV in these autoimmune diseases.

Risk factors for avascular bone necrosis in patients with systemic lupus erythematosus.

The objective was to investigate the predictive factors for avascular necrosis (AVN) of bone in patients with systemic lupus erythematosus (SLE). The records of 868 patients with SLE from four centers were reviewed retrospectively. Forty-nine patients with AVN were identified. A total of 154 patients with SLE who did not have clinically apparent AVN during the follow-up were evaluated as a control group. The demographic, clinical, laboratory and management characteristics of these two groups of patients were recorded according to predefined protocol and compared. The prevalence of AVN was detected 6% in our SLE population. The highest dose corticosteroid administered within 4 months and total cumulative prednisolone dose were significantly higher in the SLE patients with AVN. The use of cytotoxic agent significantly higher proportion of patients with AVN. AVN tends to develop more frequently in male gender and younger patients. Oral ulcer, pleuritis, Raynaud's phenomenon, cutaneous vasculitis, lymphadenopathy, autoimmune thyroiditis, peripheral neuropathy and Sjögren's syndrome were higher incidence in SLE patients with AVN. The bilateral femoral heads were the commonest site of involvement of AVN in our patients with SLE.

Rituximab as a new therapeutic option in granulomatosis with polyangiitis: a report of two cases.

Findings of several reports suggest that rituximab, a chimeric monoclonal anti-CD20 antibody causing B-lymphocyte depletion, might represent a treatment option for people with granulomatosis with polyangiitis (GPA) (former Wegener's granulomatosis). This study presents the results of rituximab treatment in two patients with treatment-refractory GPA. First patient received rituximab for a granulomatous posterior orbital mass lesion, and eye symptoms were resolved after three courses of treatment. The second patient had eye and paranasal sinus involvement and benefited from two courses of rituximab treatment, with significant clinical improvement. Rituximab may represent an effective novel treatment for remission induction in GPA.

Metabolic syndrome is associated with increased cardiovascular risk and disease damage in patients with Takayasu arteritis.

OBJECTIVE: Metabolic syndrome (MetS) is one of the preventable risk factors for cardiovascular disease (CVD). The aim of this study was to investigate the effect of MetS on CVD and cumulative organ damage in a multi-center, large cohort of patients with Takayasu arteritis (TAK). METHODS: This is a cross-sectional study involving 192 consecutive TAK patients from seven tertiary rheumatology centers in Turkey. Clinical data of TAK patients fulfilling the 1990 American College of Rheumatology classification criteria were collected from medical records. They were evaluated for risk factors of CVD, disease activity, damage, and MetS at their last visits. RESULTS: A total of 192 consecutive TAK patients were included in this study. One hundred and fifty-eight (82%) were female, the mean age was 43.3 ± 13 years, and mean disease duration was 13.5 ± 9.3 years. MetS was detected in 50 (26%) of the patients and CVD was detected in 28 (14.6%). The presence of MetS was detected as an independent risk factor for CVD (P < 0.001). In addition, the mean vasculitis damage index of the group with MetS was significantly higher than in the other patients (4.5 ± 3.3 vs 3.2 ± 2.2, respectively, P = 0.004). CONCLUSION: The presence of MetS in TAK is associated with increased CVD and disease damage. Awareness and management of MetS can improve disease prognosis in patients with TAK.

TNF-alpha antagonist therapy modify the tuberculin skin test response.

Tumour necrosis factor-alpha (TNF-α) antagonist drugs have been associated with increased risk of tuberculosis (TB). Tuberculin skin test (TST) is the most frequently used tool for identification of latent TB infection. We herein aimed to analyse the effect of TNF-α antagonists on the TST responses in a prospective study. The study group consisted of 182 patients (99 female, 83 male) who received TNF-α antagonists for various rheumatic disorders. All patients were evaluated with TST along with other parameters on the day of referral and on the 12th month visit. For those patients with a response of <5 mm induration at the initial evaluation, the TST was repeated to observe the booster effect. Out of 182 patients, 87 patients (48%) had a negative (0-4 mm) and 95 (52%) had a positive (≥ 5 mm) TST response at initial evaluation. The TST responses were converted from negative at initial visit to positive at 1-year repeat in 26 (30%) patients. A significant increase was observed in the diameters of TST that were repeated on the first year of TNF-α antagonist treatment (9.15 ± 0.55) compared to their initial diameters (6.60 ± 0.51) (P < 0.001). Increased TST responses in patients receiving TNF-α antagonists may be associated with the restoration of suppressed immune reactivity against TB antigens with the decreased disease activity. The meaning of TST conversion in the definition of latent TB infection and the need for chemoprophylaxis in these patients remains to be answered by further studies.

Pulmonary hypertension in systemic lupus erythematosus: relationship with antiphospholipid antibodies and severe disease outcome.

Pulmonary hypertension (PH) in systemic lupus erythematosus (SLE) is associated with an unfavorable prognosis. We investigated the characteristics of SLE patients with PH. The patients with a pulmonary artery systolic pressure more than 30 mmHg at rest on echocardiogram were diagnosed with PH. Echocardiography was done only in patients with clinical or radiological evidence suggesting PH. Right heart catheterization was not performed. We identified 10 SLE patients with PH between 1980 and 2000. We compared their clinical and laboratory parameters with those of 97 consecutive SLE patients without PH. Nine of the ten patients with PH were females. The mean age at the time of SLE onset was 25.2 ± 6.9 years; the mean duration of follow-up was 93.4 ± 52.8 months, and the interval between the onset of SLE and PH diagnosis was 9.0 ± 4.6 (5-21) years. Antiphospholipid antibody positivity was significantly higher in the PH group (80 vs. 36%; p < 0.05), but there was no significant difference between two groups in regard to secondary antiphospholipid syndrome. The frequency of Raynaud's phenomenon was higher in PH group (60 vs. 27%; p < 0.05). Renal involvement (80 vs. 43%; p < 0.05), neuropsychiatric involvement (40 vs. 7.2%; p < 0.005) and serositis (70 vs. 14.4%; p < 0.001) were significantly more frequent in the PH group. The mean damage score in patients with and without PH were 4.0 ± 2.4 and 0.4 ± 1.0, respectively (p < 0.001). Four patients with PH died during the follow-up. This study reveals that the presence of PH defines a subgroup of patients with a severe disease and increased mortality. Antiphospholipid antibodies and Raynaud's phenomenon may contribute to the pathogenesis of PH. We recommend that all patients with SLE, especially those positive for antiphospholipid antibodies and/or with signs of Raynaud's phenomenon should be regularly evaluated for the development of PH.

Assessment of disease activity and progression in Takayasu's arteritis with Disease Extent Index-Takayasu.

OBJECTIVE: Disease Extent Index-Takayasu (DEI.Tak) is a new index developed for the follow-up of Takayasu's arteritis (TA), assessing only clinical findings without the requirement of imaging. We investigated the effectiveness of DEI.Tak in assessing disease activity and progression by comparing with physician's global assessment (PGA) and active disease criteria defined by Kerr et al. METHODS: The initial DEI.Tak forms were filled in for 145 TA patients cross-sectionally to detect the baseline damage and after 29.8 (31) months (n = 105, 144 visits) only by including the new/worsening symptoms within the past 6 months. RESULTS: At baseline, all patients had a DEI.Tak >0 [mean (s.d.): 7.6 (4.2)]. At this evaluation, 62% of the patients had active, 16.2% had persistent and 21.8% had inactive disease according to the PGA. At follow-up, in 69% of the patients the DEI.Tak score was 0. However, 14% of them were accepted as having active and 17% persistent disease according to PGA. In contrast, 18% with a DEI.Tak ≥ 1 were inactive according to PGA. Patients with active or persistent disease with PGA had higher DEI.Tak compared with inactives [1.3 (1.9), 1 (1.3) vs 0.2 (0.6), respectively; P < 0.001]. According to Kerr's criteria 27% were active. The total agreement between DEI.Tak and Kerr's criteria was 94% (κ = 0.85). Compared with PGA, Kerr's criteria had a total agreement of 74% and DEI.Tak 68%. CONCLUSION: During follow-up, most TA patients showed no clinical activity with DEI-Tak. Although the agreement between Kerr's criteria and DEI.Tak seemed very good, using Kerr's criteria instead of DEI.Tak increased the consistency with PGA, which could be explained by the influence of imaging data and acute-phase reactant levels on the physician's decisions.

Recurrent anterior uveitis in Henoch Schonlein's vasculitis.

Uveitis is an important component of many rheumatic diseases. The main causes of recurrent uveitis are seronegative spondylarthropathies and Behçet's disease. We describe a rare case of Henoch Schönlein vasculitis (HSV) along with multiple recurrences of acute anterior uveitis. In cases of skin rash and recurrent anterior uveitis, HSV should be considered in the differential diagnosis.

Clinical evaluation and comparison of different criteria for classification in Turkish patients with psoriatic arthritis.

Several criteria are being used for the classification of psoriatic arthritis (PsA) and there is a lack of consensus about PsA as a separate entity. Our aim is to investigate the clinical features of our patients with a clinical diagnosis of PsA, compare the sensitivities of different classification criteria, agreement between the criteria. In this study 86 PsA patients were investigated (48 females, mean age 44). Moll and Wright criteria was fulfilled by 91%, Vasey and Espinoza criteria by 94% and modified European SpA study group criteria by 59%, classification of PsA study group criteria by 86%, modified McGonagle criteria by 96%, Fournie et al. criteria by 84% and Gladman criteria by 95%. Significant agreement was present between criteria but generally kappa-values were less than 0.5. The pattern of PsA can differ with time and the implementation of the available classification criteria showed considerable differences.