Real-life experience of tofacitinib in patients with treatment-resistant rheumatoid arthritis: A 5-year follow-up: Monocentric experience.

OBJECTIVE: The present study aims to assess the short- and long-term effects of tofacitinib (TOFA) therapy on efficacy, safety, and drug retention rate patients with rheumatoid arthritis (RA) refractory to conventional synthetic disease-modifying anti-rheumatic drugs (csDMARDs) and/or biological disease-modifying anti-rheumatic drugs (bDMARDs). METHODS: Thirty-five patients with RA who received TOFA therapy for at least 3 months in rheumatology outpatient clinic between December 2015 and December 2020 were included in the study. The prospectively follow-up results of the patients obtained on the 6th month and 5th year are presented. Demographic characteristics of the patients, the disease activity score-28 for RA with erythrocyte sedimentation rate (DAS 28-4 [ESR]), change in DAS-28, health assessment questionnaire score, patient visual analog scale score, and laboratory parameters were recorded. The data at 6 months and 5 years of treatment were compared with baseline data. All side effects were recorded at each follow-up visit. Wilcoxon signed-rank tests were used for analysis. RESULTS: Of the 35 patients, 23 received TOFA treatment after receiving ≥1 bDMARDs, while the remaining 12 patients received TOFA therapy were biologic naive. On the 6-month follow-up, DAS 28-4 (ESR) score and DAS28 improvement significantly decreased at the 6th months from baseline (p<0.001 and p<0.001, respectively), and moderate disease activity was achieved in 13 patients. High disease activity persisted in four patients. DAS28 improvement according to the EULAR response criteria was good response in 86% of the patients. DAS 28-4 (ESR) score and DAS28 improvement significantly decreased at 5 years from baseline (p<0.01 and p<0.001, respectively), and the moderate disease activity was achieved in 10 patients. High disease activity persisted in two patients. Drug retention rate at 5-year follow-up was 54% and the daily glucocorticoid therapy could be discontinued in 9 patients (47%). Three patients (15%) were tested positive for COVID-19. None of them required hospitalization and no deaths were occurred due to COVID-19. CONCLUSION: TOFA is effective and well-tolerated treatment options that reduce the need for steroids in patients with RA.

Prevalence of Malnutrition According to the Global Leadership Initiative on Malnutrition Criteria in Community-dwelling Older Adults in Turkey.

Objective: This study aimed to investigate the prevalence of malnutrition according to the Global Leadership Initiative on Malnutrition (GLIM) criteria in community-dwelling older adults in Turkey. Methods: Malnutrition was assessed based on the GLIM criteria, and Mini-Nutritional Assessment-Short Form was used for screening. The severity of malnutrition was determined as severe or moderate based on the phenotypic criteria of GLIM. Results: Five hundred sixty-nine (69% female, mean age 74.42±6.58 years) community-dwelling older adults participated in this study. The educational statuses of the participants were as follows: 17.2%, illiterate; 13.3%, literate; 35.3%, primary school; 7.0%, secondary school; 11.1%, high school; and 16.2%, university graduates. Among the participants, 16.4% were living alone, whereas 43.9% of them were living with their spouses. The rest were living with their extended families. According to the GLIM criteria, 24.5% (n=139) of the participants had malnutrition, and 13.9% of the total population had severe malnutrition. Age was significantly associated with malnutrition [odds ratio 1.064, 95% confidence interval (CI) 1.034-1.096, p<0.0001]. No significant difference was found between genders (p=0.207), education groups (p=0.323), and living status (p=0.434) in terms of malnutrition. However, women had higher malnutrition rates than men (26.0% vs. 21.0%) (risk ratio 0.757, 95% CI 0.494-1.160, p=0.207). Conclusions: The prevalence of malnutrition was 24.5%, whereas the rate of severe malnutrition was 13.9% in community-dwelling older adults. Women had higher rates of malnutrition, and age was associated with malnutrition. We recommend for researchers and clinicians to integrate the GLIM criteria into their practices to create a common language in malnutrition assessment.

Clinical outcomes of patients with COVID-19 and inflammatory rheumatic diseases receiving biological/targeted therapy.

BACKGROUND: Anti-cytokine treatments are used in the treatment of severe COVID-19. Other studies have shown statistical significance with TNF inhibitors but not with other biological/targeted synthetic disease-modifying anti-rheumatic drugs (b/tsDMARD). OBJECTIVES: Compare the rate of severe acute respiratory syndrome coronavirus-2 (SARS-COV-2) infection and the course and incidence of COVID-19 infection in patients who received b/tsDMARD with control patients. DESIGN: Analytical cross-sectional SETTINGS: Tertiary care hospital PATIENTS AND METHODS: All patients who applied to the rheumatology outpatient clinic between June 2020-March 2021 and received b/tsDMARD were included in the study. All patients with rheumatoid arthritis, psoriatic arthritis and spondyloarthritis who applied to the rheumatology outpatient clinic in the three months before March 2021 and did not receive b/tsDMARD were included as the control group. History of COVID-19 infection and treatments were recorded. Multivariate analysis was performed to assess factors associated with use of tumor necrosis factor (TNF) inhibitors and differences between specific biologic drugs. MAIN OUTCOME MEASURES: Rate of COVID-19 disease among patients using biological/targeted synthetic therapy and non-biological/targeted synthetic therapy. COVID-19 clinical outcomes (hospitalization, intensive care admission, mechanical ventilation and death). SAMPLE SIZE: 533 in total; 341 received b/tsDMARD, 212 in the control group that did not receive b/tsDMARD. RESULTS: One hundred patients (18%) had been infected with SARS-COV-2. The difference in SARS-COV-2 infection between b/tsDMARD and the control was statistically significant (13, 2% vs. 25, 9%, respectively) (P<.001). The hospital stays were longer in the controls (P<.001). Multinomial regression analysis revealed that COVID-19 negative patients were more likely to use tumor necrosis factor (TNF) inhibitors (OR: 2, 911; 95% CI: 1.727-4.908; P<.001) compared to COVID-19 positive participants. Multinomial logistic regression analysis indicated that hospitalized patients were more likely to use TNF inhibitors (OR: 11, 006; 95% CI: 3.447-35.138; P<.001) and there was no significant difference between b/tsDMARDs other than TNF inhibitors in frequency of hospitalization. CONCLUSIONS: Patients who were medicated with b/tsDMARD were less likely to be infected with COVID-19 and be hospitalized due to the infection. We have found that this effect was particularly dependent on the use of TNF inhibitors. LIMITATIONS: Conducted in a single center and unable to provide a homogeneous study population. CONFLICT OF INTEREST: None.

A Case of IgG4-related Mesenteritis and Ankylosing Spondylitis.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disease of unknown etiology and pathogenesis, which can affect all organs. The most commonly involved organs are the pancreas, hepatobiliary system, salivary glands, orbits and lymph nodes. Rarely, the thyroid, pituitary, aorta, lung and kidney may also be involved; mesenteric involvement is rare. The association of IgG4-RD with some rheumatological diseases is observed; while there are data in the literature about rheumatoid arthritis and Sjögren syndrome, but association with ankylosing spondylitis is rare. Anti-tumor necrosis factor alpha (anti-TNFα) treatment has been tried in refractory IgG4-RD cases; but this case is of great interest in terms of progressive presentation under treatment with etanercept, an anti-TNFα agent, which is also used for the treatment. But, IgG4-related mesenteritis with ankylosing spondylitis receiving anti-TNFα therapy, which has also been tried in the treatment of IgG4 disease, has not been reported in the literature. Key Words: IgG4-related disease, Mesenteritis, Ankylosing spondylitis.

Evaluation of the relationship between acute kidney injury and renin angiotensin system inhibition in COVID-19 patients.

OBJECTIVE: In patients with coronavirus disease 2019 (COVID-19), acute kidney injury (AKI) may alter the clinical course and outcome of the disease. In this study, the association of AKI with renin angiotensin system (RAS) inhibitor treatment and its clinical consequences were examined in COVID-19 patients admitted to our hospital during the initial stages of the pandemic. METHODS: A total of 407 patients between 18 and 85 years of age (202 male and 205 female) admitted to the Umraniye Research And Training Hospital between May 2020 and August 2020 with a diagnosis of COVID-19 were included in the study. Patients were categorized as follows: Group 1, subjects with no chronic conditions (n=150); and Group 2, subjects with comorbid conditions (n=257). Group 2 was subdivided into Group 2A (receiving angiotensin receptor blocker [ARB])/(angiotensin converting enzyme inhibitor [ACEI], n=81), and Group 2B (not receiving ARB/ACEI, n=176). RESULTS: Hypertension was the most frequent comorbid condition (36.4%). There was no difference in survival rates between the patients who used RAS inhibitor and the ones who did not based on log rank test (p=0.342). Fifty-four patients (13.4%) had developed AKI during the time frame of the disease. In patients with chronic diseases such as hypertension, the use of RAS inhibitory medication was not associated with developing AKI (OR 95% CI: 0.317-1.358; p=0.256). The survival rate of the patients with AKI was significantly lower than patients without AKI (p<0.0001). CONCLUSION: COVID-19 may cause renal injury represents a risk factor for mortality. Therefore, detection of renal injury has a particular prognostic importance.

Evaluation of factors affecting the frequency and clinical course of COVID-19 in patients using anti-TNF-alpha agents.

OBJECTIVES: Patients being treated with anti-tumor necrosis factor-alpha (anti-TNF-alpha) agents were reported to have better prognosis related to COVID-19. We evaluated the factors affecting the frequency, clinical course, and outcome of COVID-19 in patients treated with anti-TNF-alpha agents. METHODS: Patients with rheumatoid diseases and chronic inflammatory bowel diseases treated with anti-TNF-alpha agents were evaluated retrospectively. The laboratory data in routine visits, frequency of COVID-19, pneumonia, hospitalization and/or intensive care unit (ICU) follow-up and, mortality were recorded. The factors related to COVID-19 frequency and clinical outcome were evaluated. RESULTS: A total of 324 patients (177 males [54.6%] and 147 females [45.4%], mean age: 45.3±12.16 years) was included in the study. In all, 44 (13.6%) patients had COVID-19; of these, 11 (25%) developed pneumonia, 7 (15.9%) were hospitalized, and 1 (2.3%) was followed up in ICU. There was no mortality. The patients with COVID-19 pneumonia were older (mean age: 52±11 years versus 41±12 years, p=0.01), had hypertension and coronary artery disease more frequently (5 cases [55.6%] versus 4 cases [44.4], p=0.02 and 2 cases [100%] versus 0 cases [0%], p=0.014, respectively), and lower eosinophil % (1.35±1.79% versus 2.3±1.45%, p=0.016). The diabetes mellitus was more frequent (66.7 versus 33.3%, p=0.013), and mean eosinophil % was lower among inpatients compared with outpatients (1.29±2.22% versus 2.19±1.37%, p=0.02). CONCLUSIONS: We concluded that the patients treated with anti-TNF-alpha agents having COVID-19 might have mild clinical course and better prognosis.

Can Monocytopenia Be a New Indicator in Determining Survival in Covid-19 Disease?

BACKGROUND: Monocytes play a central role in Covid-19 infection. Monocytopenia is especially observed in patients with severe infection. In our study, we aimed to investigate the effects of monocytopenia on survival in patients presenting with the suspicion of Covid-19. METHODS: Patients diagnosed with Covid-19 who received inpatient or outpatient treatment in the pandemic clinic of Umraniye Training and Research Hospital between April 2020 and July 2020 were included in our retrospective cohort study. Patients were divided into two groups, severe and non-severe. Demographic data, laboratory parameters, those who were monocytopenic at presentation, those who developed monocytopenia during follow-up, and those with persistent monocytopenia were noted. RESULTS: The study included 471 patients with a mean age of 50.4 ± 18.2 years. Sixty-seven (14.2%) patients had severe and 404 (85.8%) had non-severe disease. The minimum value of monocytes detected during the follow-up in severe patients was significantly lower (p < 0.001). As patients were grouped into those with monocytopenia at the time of admission, those with monocytopenia developing during their follow-up, and those whose monocytopenia at admission persisted, they were compared with regards to the presence or absence of severe disease. Newly developing monocytopenia and persistent monocytopenia were significant in patients with severe disease (p < 0.001, p = 0,001 respectively). Also, among non-surviving patients, there was a significant difference in survival times in the monocytopenia group (p = 0.029). CONCLUSIONS: In our study, we found more developing and persistent monocytopenia in the severe group, and the survival time was low, especially in those with newly developing monocytopenia. The development of monocytopenia in the daily hemogram follow-up of the patients may increase the possibility of mortality, which suggests that monocytopenia may be a new marker in determining survival.

Evaluation of factors affecting the frequency and clinical course of COVID-19 in patients using anti-TNF-alpha agents

SUMMARY OBJECTIVES: Patients being treated with anti-tumor necrosis factor-alpha (anti-TNF-alpha) agents were reported to have better prognosis related to COVID-19. We evaluated the factors affecting the frequency, clinical course, and outcome of COVID-19 in patients treated with anti-TNF-alpha agents. METHODS: Patients with rheumatoid diseases and chronic inflammatory bowel diseases treated with anti-TNF-alpha agents were evaluated retrospectively. The laboratory data in routine visits, frequency of COVID-19, pneumonia, hospitalization and/or intensive care unit (ICU) follow-up and, mortality were recorded. The factors related to COVID-19 frequency and clinical outcome were evaluated. RESULTS: A total of 324 patients (177 males [54.6%] and 147 females [45.4%], mean age: 45.3±12.16 years) was included in the study. In all, 44 (13.6%) patients had COVID-19; of these, 11 (25%) developed pneumonia, 7 (15.9%) were hospitalized, and 1 (2.3%) was followed up in ICU. There was no mortality. The patients with COVID-19 pneumonia were older (mean age: 52±11 years versus 41±12 years, p=0.01), had hypertension and coronary artery disease more frequently (5 cases [55.6%] versus 4 cases [44.4], p=0.02 and 2 cases [100%] versus 0 cases [0%], p=0.014, respectively), and lower eosinophil % (1.35±1.79% versus 2.3±1.45%, p=0.016). The diabetes mellitus was more frequent (66.7 versus 33.3%, p=0.013), and mean eosinophil % was lower among inpatients compared with outpatients (1.29±2.22% versus 2.19±1.37%, p=0.02). CONCLUSIONS: We concluded that the patients treated with anti-TNF-alpha agents having COVID-19 might have mild clinical course and better prognosis.

COVID-19 Among Patients With Inflammatory Rheumatic Diseases.

BACKGROUND: The course of novel coronavirus disease 2019 (COVID-19) has been of special concern in patients with inflammatory rheumatic diseases (IRDs) due to the immune dysregulation that may be associated with these diseases and the medications used for IRDs, that may affect innate immune responses. OBJECTIVE: In this cohort study, we aimed to report the disease characteristics and variables associated with COVID-19 outcome among Turkish patients with IRDs. METHODS: Between April and June, 2020, 167 adult IRD patients with COVID-19 were registered from 31 centers in 14 cities in Turkey. Disease outcome was classified in 4 categories; (i) outpatient management, (ii) hospitalization without oxygen requirement, (iii) hospitalization with oxygen requirement, and (iv) intensive care unit (ICU) admission or death. Multivariable ordinal logistic regression analysis was conducted to determine variables associated with a worse outcome. RESULTS: 165 patients (mean age: 50 ± 15.6 years, 58.2% female) were included. Twenty-four patients (14.5%) recovered under outpatient management, 141 (85.5%) were hospitalized, 49 (30%) required inpatient oxygen support, 22 (13%) were treated in the ICU (17 received invasive mechanic ventilation) and 16 (10%) died. Glucocorticoid use (OR: 4.53, 95%CI 1.65-12.76), chronic kidney disease (OR: 12.8, 95%CI 2.25-103.5), pulmonary disease (OR: 2.66, 95%CI 1.08-6.61) and obesity (OR: 3.7, 95%CI 1.01-13.87) were associated with a worse outcome. Biologic disease-modifying antirheumatic drugs (DMARDs) do not seem to affect COVID-19 outcome while conventional synthetic DMARDs may have a protective effect (OR: 0.36, 95%CI 0.17-0.75). Estimates for the associations between IRD diagnoses and outcome were inconclusive. CONCLUSIONS: Among IRD patients with COVID-19, comorbidities and glucocorticoid use were associated with a worse outcome, while biologic DMARDs do not seem to be associated with a worse outcome.

The Relationship between Vitamin D Level and Lower Urinary Tract Symptoms in Women.

OBJECTIVES: In the literature, the effects of vitamin D on lower urinary tract symptoms (LUTS) have been investigated. Conflicting results have been reported in these studies conducted. LUTS is more common in women. In this study, we aimed to evaluate the relationship between vitamin D and LUTS in female patients using the uroflowmetric method. METHODS: This retrospective cohort study included 186 female patients who were admitted with LUTS. Demographic characteristics, medical history, calcium (Ca) and vitamin D, including laboratory studies and uroflowmetry results, as maximum urine flow rate (Qmax), average urine flow rate (Qav) and voided volume (V) were recorded. Patients were divided into two groups according to age (18-50 and ≥51) and vitamin D levels (<20 and ≥20). Laboratory parameters and uroflowmetry results were compared between groups. RESULTS: Mean age was 56.85±12.95 years. Mean vitamin D level was 21.19±13.93 ng/mL (2.5-83.5). Mean Qmax value was 35.41±12.63, whereas the mean Qav was 19.13±9.89, and the mean V was 446.60±165.08 mL. Vitamin D levels differed according to age groups (p=0.044). No significant difference was observed between groups according to Qmax, Qav and V values (p>0.05). No significant correlation was detected between vitamin D level and Qmax, Qav and V values. However, a negative correlation was detected between serum Ca level and V values (p=0.042) in the low vitamin D group. CONCLUSION: There was no direct relationship between vitamin D levels and LUTS in respect to uroflowmetry. However, we determined that Ca levels affect the uroflowmetry parameter in patients with low vitamin D levels. There is a need for further studies emphasizing serum Ca levels in addition to vitamin D levels in patients with LUTS.

Favorable retention rates and safety of conventional anti-rheumatic drugs in older patients with rheumatoid arthritis.

Physicians are challenged by the recognition and treatment of older patients with rheumatoid arthritis (RA). The aim of this case-control study was to evaluate the retention and safety of conventional disease-modifying anti-rheumatic drugs in older patients with RA.In this observational case-control study, we assessed older patients with RA (≥65 years) who were diagnosed in 3 different rheumatology centers from Turkey. Patients were divided as to those aged ≥65 years (elderly rheumatoid arthritis [ERA]) and those aged <65 years (young rheumatoid arthritis [YRA]) at the time of conventional DMARD treatment initiation. The Mann-Whitney U test was used for the comparison of 2 non-normally distributed groups. The Chi-square (χ) test was used for categorical variables. Survival analysis were performed using the Kaplan-Meier method.Four hundred eighteen patients with RA (296 females [71%]) were included from January 2010 to January 2018. The age of treatment onset of 190 (47%) patients was in the elderly period and they were included in the ERA group. In the analysis of drug retention rates, there was no significant difference between the ERA and YRA groups for each conventional DMARD (methotrexate 71.2% in ERA, 62.7% in YRA, P = .817; hydroxychloroquine 82.9% in ERA, 78.8% in YRA, P = .899; leflunomide 81.4% in ERA, 84.4% in YRA, P = .205; sulfasalazine 37.5% in ERA, 40.9% in YRA, P = .380). The adverse event data were also similar in both groups.The drug retention and adverse effect rates in older patients with RA using conventional DMARDS are similar to the rates in young patients with RA.

The assessment of malnutrition and severity of gastrointestinal disease by using symptom-based questionnaires in systemic sclerosis: is it related to severe organ involvement or capillary rarefaction at microcirculation?

OBJECTIVES: Gastrointestinal (GI) system is commonly affected in sytemic sclerosis (SSc) patients who are also known to be at risk for malnutrition. We aimed to investigate the relationship between severity of GI disease, malnutrition and severity of organ involvement including microvasculopathy. METHODS: A hundred and thirty-four SSc patients were included into the study; disease activity and severity, the University of California, Los Angeles, Scleroderma Clinical Trials Consortium Scleroderma Gastrointestinal scale 2.0 (UCLA SCTC GIT 2.0) and malnutrition universal screening tool (MUST) were cross-sectionally assessed. Nailfold video-capillaroscopy(NVC) was performed to evaluate microvasculopathy. RESULTS: SSc patients who are at medium to high risk for malnutrition (n=20); had more frequently limited pulmonary function, lung involvement, pulmonary hypertension, capillary rarefaction and NVC late pattern than those at low risk for malnutrition. Capillary rarefaction (≤6/mm) was shown to be independently associated with medium to high risk for malnutrition defined by using MUST. Capillary rarefaction and severe skin involvement were found to be independently related to 'severe' or 'very severe' GI disease defined by using UCLA SCTC GIT 2.0. UCLA SCTC GIT 2.0 scores were not found to be good discriminative in patients at risk for malnutrition allowing for a ROC curve of area under the curve (AUC)<0.8). CONCLUSIONS: Assessment of gastrointestinal complaints and nutritional status by using symptom based questionnaires reflected the severity of GI disease and malnutrition including some limitations. Capillary rarefaction and severe skin involvement might be determining factors for malnutrition risk and severe GI disease.

Short report: correlates of functional disability with disease activity in elderly patients with rheumatoid arthritis.

Rheumatoid arthritis (RA) prevalence increases with age and old people are special patient population. The recognition of functional disability related to RA could be challenging in elderly patients because aging itself and potential co-morbid disease may also cause functional disability. In this study, we aimed to look at the correlation between disease activity and functional disability in elderly RA patients. Elderly RA patients, ≥65 years old at their routine visits were included in the study. The composite 'disease activity score' in 28 joints (DAS-28) was used to determine disease activity groups. Health assessment questionnaire (HAQ) scores were calculated to describe the functional disability and compared across the disease activity groups. Two hundred and fifty-eight RA patients with the mean age of 71 ± 5 (65-90) and a total disease duration of 8.4 ± 8.5 (.5-50) years were recruited. The proportion of patients with high and moderate disease activity was 70%. HAQ scores were significantly correlated with disease activity (p < .05). Functional disability estimated by HAQ was correlated with disease activity in elderly patients with RA.

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 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.

Cluster analysis of autoantibodies in 852 patients with systemic lupus erythematosus from a single center.

OBJECTIVE: Associations between autoantibodies and clinical features have been described in systemic lupus erythematosus (SLE). Herein, we aimed to define autoantibody clusters and their clinical correlations in a large cohort of patients with SLE. METHODS: We analyzed 852 patients with SLE who attended our clinic. Seven autoantibodies were selected for cluster analysis: anti-DNA, anti-Sm, anti-RNP, anticardiolipin (aCL) immunoglobulin (Ig)G or IgM, lupus anticoagulant (LAC), anti-Ro, and anti-La. Two-step clustering and Kaplan-Meier survival analyses were used. RESULTS: Five clusters were identified. A cluster consisted of patients with only anti-dsDNA antibodies, a cluster of anti-Sm and anti-RNP, a cluster of aCL IgG/M and LAC, and a cluster of anti-Ro and anti-La antibodies. Analysis revealed 1 more cluster that consisted of patients who did not belong to any of the clusters formed by antibodies chosen for cluster analysis. Sm/RNP cluster had significantly higher incidence of pulmonary hypertension and Raynaud phenomenon. DsDNA cluster had the highest incidence of renal involvement. In the aCL/LAC cluster, there were significantly more patients with neuropsychiatric involvement, antiphospholipid syndrome, autoimmune hemolytic anemia, and thrombocytopenia. According to the Systemic Lupus International Collaborating Clinics damage index, the highest frequency of damage was in the aCL/LAC cluster. Comparison of 10 and 20 years survival showed reduced survival in the aCL/LAC cluster. CONCLUSION: This study supports the existence of autoantibody clusters with distinct clinical features in SLE and shows that forming clinical subsets according to autoantibody clusters may be useful in predicting the outcome of the disease. Autoantibody clusters in SLE may exhibit differences according to the clinical setting or population.

[Pulmonary arterial hypertension related to connective tissue diseases]. / Bag dokusu hastaliklarina bagli pulmoner arteriyel hipertansiyon.

Pulmonary arterial hypertension (PAH) is an important complication of connective tissue diseases (CTD) and especially seen in systemic sclerosis, systemic lupus erythematosis (SLE), and mixed connective tissue disease (MCTD). In systemic sclerosis, PAH is isolated or accompanied by interstitial lung disease and currently, a major cause of mortality. It has been shown to be developed in approximately 10% of cases and annual screening with echocardiography has been recommended. Right heart catheterization is required for definite diagnosis. Limited skin involvement, late onset, Raynaud's phenomenon, digital ulcers, telangiectasias, diminished nail fold capillaries, anti-U3RNP and anticentromere antibodies are known as risk factors for PAH development in systemic sclerosis. Following diffusion lung capacity for carbon monoxide (DLCO) and pro-brain natriuretic peptide (pro-BNP) levels can be helpful for evaluating PAH development. PAH in SLE linked to antiphospholipid antibodies and Raynaud's phenomenon in some studies. MCTD is an overlap syndrome with features of systemic sclerosis, SLE, polymyositis and positive anti-U1RNP antibodies. PAH develops in 9-27% of the patients and the leading cause of mortality in patients with MCTD. Endothelin receptor antagonists, prostacyclin analogs and phosphodiesterase 5 inhibitors are being used in patients with systemic sclerosis. In SLE/MCTD patients with early diagnosis immunosuppressive treatments may be effective.