Frailty assessment in patients with Behçet's syndrome: A cross-sectional monocentric study.

AIMS: Evidence evaluating the association between pre-frailty and frailty, and risk of adverse health outcomes in patients with Behçet's syndrome (BS) is limited in the literature. The aim of this study was to characterize the prevalence of frailty and associated factors in a single-centre cohort of patients with BS. METHODS: Based on the International Study Group's criteria, this was a monocentric cross-sectional study of BS patients. The Fried frailty criteria were used to define frailty. The Turkish version of the Behçet's Disease Current Activity Form was used to measure the disease activity of BS. Damage index was assessed with the Behçet's Syndrome Overall Damage Index. RESULTS: Forty-four patients were enrolled. According to Fried frailty criteria, patients were classified as 13.6% frail, 59% pre-frail, and 27.2% robust, respectively. Compared to pre-frail and robust patients, frail patients had higher levels of inflammatory markers at the time of diagnosis. CRP levels at time of diagnosis and at the last visit were higher in the frail group than in the pre-frail and robust groups (p=0.039 and p=0.023, respectively). When active drugs for BS were evaluated, systemic glucocorticoid (50%, p=0.030) and cyclophosphamide (33.3%, p=0.006) treatments were higher in the frail group. CONCLUSIONS: Frailty and pre-frailty are commonly detected even in younger patients with BS. Inflammation can be described as potential determinants of frailty status.

Interstitial Lung Disease in Primary Sjögren's Syndrome: Risk factors for occurrence and radiographic progression.

OBJECTIVES: The aim of this study is to investigate the characteristics of Primary Sjögren's syndrome (pSS)- interstitial lung disease (ILD) patients and compare them to those of pSS patients without ILD in the tertiary pSS-ILD cohort to evaluate potential risk factors for ILD occurrence and disease progression. METHODS: Patients followed up who met the 2016 American College of Rheumatology-European League Against Rheumatism classification criteria for pSS were retrospectively analyzed. The patients were grouped as those with ILD and those without ILD according to medical records. High-resolution computed tomography (HRCT)/ thorax CT (TCT) results of all ILD patients were evaluated. Data on demographics, comorbidities, clinical characteristics and laboratory findings were collected. RESULTS: A total of 378 pSS patients, including 60 with ILD and 318 without ILD were detected to have at least one obtainable HRCT/TCT and were included in the study. In the cohort of pSS patients with at least one HRCT or TCT, the frequency of ILD was 15.8%. In the ILD group, the most common HRCT pattern was NSIP, and the most common findings were ground glass opacities, traction bronchiectasis, and honeycombing. Logistic regression analysis showed that male gender (OR:2.90), being diagnosed with pSS over the age of 50(OR:4,24), smoking history (OR:2.38), elevated LDH(OR:3.27), elevated ESR(OR:2.51) and lymphopenia (OR:5.12) were related with development of ILD while being diagnosed with ILD after the age of 60 (OR:8.5) was related with radiographic progression. CONCLUSION: The study results provided a large spectrum view for pSS-ILD and pointed out several risk factors for ILD occurrence and radiographic progression.

Impact of celiac disease in Behçet's syndrome patients: a study based on the database of Türkiye.

Background/aim: Our primary aim was to investigate the effects of concomitant celiac disease (CD) on the clinical characteristics of Behçet's syndrome (BS) patients. Materials and method: The study was a retrospective, nationwide, multicenter study. Turkish Ministry of Health National Electronic Database (e-Nabiz) is used under Health Ministry's supervision to extract the subject's data. Statistical analysis: Statistical analyses were made by the Statistical Package for Social Sciences (SPSS) software version 20 (IBM Corp., Armonk, New York). Continuous variables were presented by mean ± standard derivation (SD) or median (min-max) according to normality and compared by student-t test. A binary logistic regression analysis was performed to further investigating the relation between having a concomitant CD with each BD manifestation and comorbidity, frequencies of which were detected to be significantly different in the student-test. Results: A total of 84,241 patients diagnosed with BS were analyzed, and CD was identified in 175 (0.21 %) patients. The group with CD had a mean age of 41.30 ± 13.69 which was significantly younger. the prevalence of females was significantly higher (71.4%). The mean age of first admission for BS was also significantly younger in the group with CD (36.64 ± 13.28). BS patients with CD had a significantly higher prevalence of inflammatory bowel disease (27.2% vs. 7.3%, p < 0.001). When comorbid conditions were investigated depression (35.4% vs. 23.3%, p < 0.001), migraine (7.4 % vs. 2.6%, p < 0.001), fibromyalgia (10.9% vs. 4.5%, p < 0.001) and osteoporosis (12.6% vs. 6.6%, p = 0.001) were significantly more frequent in BS patients with CD. Conclusion: Our results suggest coexistence of CD in BS patients is related to female dominance and probably to an earlier disease onset. Several CD-related comorbidities as well as inflammatory bowel disease were more frequent in the CD group which implied an increased overall disease burden.

Coronavirus Disease 2019 Outcomes in Amyloid A Protein Amyloidosis Secondary to Rheumatic Conditions and Signs of Post- Coronavirus Disease 2019 Proteinuria Progression.

BACKGROUND: We aimed to investigate coronavirus diease 2019 (COVID-19) outcomes in patients with amyloid A protein (AA) amyloidosis secondary to rheumatic diseases and discuss factors associated with disease course. METHODS: A retrospective cohort was formed from adult patients with a diagnosis of AA amyloidosis. In patients with a positive severe acute respiratory syndrome coronavirus 2 polymerase chain reaction (PCR) test, rates of hospitalization, intensive care unit admission and mortality due to COVID-19 were collected from medical records. Data regarding to demographics, comorbidities, laboratory tests, medical treatments, adherence to previous treatments during COVID-19 and treatment administered for COVID-19 were collected from hospital databases and patient reviews. RESULTS: In 96 patients with AA amyloidosis, 16 had COVID-19 with a positive PCR. Ten (62.5%) patients were hospitalized, 2 (12.5%) were admitted to ICU, 1 (6.25%) was died. Hospitalized patients tended to be older. Comorbidities seemed to be more frequent in hospitalized patients. None of the patients had rapid progression to end-stage renal disease post-COVID-19. Seven patients had pre-COVID-19 and post-COVID-19 proteinuria levels. Three had notable increase in proteinuria after COVID-19 in 2 of which amyloidosis treatment was revised accordingly. CONCLUSION: Despite high rates of hospitalization in AA amyloidosis patients, mortality was observed only in 1 patient. Progression of proteinuria requiring treatment adjustment may be an issue in these patients. Cite this article as: Güven SC, Erden A, Küçük H, et al. Coronavirus disease 2019 outcomes in amyloid A protein amyloidosis secondary to rheumatic conditions and signs of post-coronavirus disease 2019 proteinuria progression. Eur J Rheumatol. Published online April 4, 2024. DOI:10.5152/eurjrheum.2024.23050.

Serum netrin-1 levels are high in Rheumatoid arthritis associated interstitial lung disease.

BACKGROUND: Recent data show that netrin-1 has a role in development of pulmonary fibrosis. This study was aimed to investigate serum netrin-1 level and its relation to interstitial lung disease(ILD) in patients with rheumatoid arthritis (RA). METHOD: 42 RA patients with RA-ILD, 58 RA patients without RA-ILD (RA non-ILD group), and 61 healthy volunteers were included in this study. The modified DAS28-ESR score was used to calculate disease activity in RA patients. Using the quantitative immunoassay method, Serum netrin-1 levels were measured with an ELISA kit (Catalog number: E-EL-H2328; lab science, lot number: GZWTKZ5SWK, Texas, USA). RESULTS: The median value of netrin-1 was found to be significantly higher in the RA-ILD group (82.9 [59.9-124]) compared to both the RA non-ILD group(52.9 [49.5-73.1])(B = -0.006, OR = 0.994, CI 95 %=0.989-0.999, P = 0.018) and the control group(53.5 [49.5-87.5]) (B: -0.005, OR: 0.994, CI 95 %: 0.990-0.999, p: 0.022). A cut-off value of 61.78 for netrin-1 was found to have a sensitivity of 73.8 % and a specificity of 69 % for the diagnosis of RA-ILD (AUC [95 %Cl] = 0.771 [0.679-0.862], p < 0.0001).It was found that high serum netrin-1 level was strongly associated with the RA-usual interstitial pneumonia(UIP) pattern and poorly related to the RA-nonspecific interstitial pneumonia(NSIP) pattern compared to the RA non-ILD group. CONCLUSIONS: Netrin-1 is elevated in the serum of patients with RA-ILD, especially in the UIP pattern. Netrin-1 may be a potential candidate for predicting the development of RA-ILD that should be investigated in the pathophysiological and therapeutic fields..

Effects of intrauterine device use on attack frequency and severity in woman of reproductive age with familial Mediterranean fever.

AIM: Familial Mediterranean fever (FMF) is the most common inherited autoinflammatory disease in the world. There are known triggers to initiate an FMF attack, yet potential effects of intrauterine devices (IUD) in women of reproductive age have not been evaluated before. METHOD: Consecutive female patients with FMF who ever used IUD over the age of 18 were enrolled. Female patients with FMF were sub grouped according to the type of IUD they use. FMF attack frequency, severity, duration, presence of dysmenorrhea, severity of dysmenorrhea, having attacks during menstruation before and after IUD use were questioned. Demographic and clinical data were collected from hospital database. RESULTS: When all patients with IUD use were evaluated, it was found that the frequency of attacks increased after IUD insertion at 3rd and 12th months (median [min-max] attack frequency at 3rd month, 1 (0-3) vs 1 (0-6), p = 0.002, median [min-max] attack frequency at 12th month, 2 (0-12) vs 3.5 (0-18), p = 0.028). Attack severity measured by VAS pain was also significantly increased. Attack duration and menstrual pain was similar before and after IUD use. Attack frequency at 3rd and 12th months, attack severity and menstrual pain was all increased significantly in Cu-IUD users, whereas none of these parameters deteriorated in LNG-IUD group. CONCLUSION: IUD use, especially Cu-IUD, may increase the frequency and severity of attacks in female patients with FMF. Clinicians may benefit from considering LGN-IUD if IUDs are preferred as contraception in women of childbearing age with FMF.

Neurological Involvement in Patients with Primary Sjögren's Syndrome: A Retrospective Cross-Sectional Study.

Background: To determine the rate and types of neurological involvement in patients with primary Sjögren's syndrome (pSS) and to evaluate predictive clinical and immunologic features of neurological involvement. Methods: We retrospectively assessed 2127 patients with an ICD-10 code for Sjögren recorded in the hospital database. Among these patients, those meeting the pSS classification criteria and having neurological symptoms and an objective evaluation accordingly were enrolled. After comparing the patients with and without neurological involvement, peripheral and central involvement subtypes were also compared within themselves. Results: A total of 199 pSS patients were enrolled and neurological involvement was found in 31.6%. Peripheral nervous system (PNS) involvement was found in 23.5% of the patients, and central nervous system (CNS) involvement was found in 34.3%. Patients with neurological involvement had a higher frequency of Schirmer's test, anti-Ro/SS-A and anti-La/SS-B positivity and the presence of interstitial lung disease, articular involvement, lymphadenopathy, anemia and hypocomplementemia than patients without those. In multivariate regression analysis, only articular involvement had a higher risk for the development of neurologic involvement [OR 10.01 (4.18-23.97), P 0.0001]. Among the patients with PNS, the frequency of anti-Ro/SS-A positivity, low C3 and Schirmer's test positivity were statistically increased compared to those who were not in PNS (P = 0.032, P = 0.044, and P = 0.029, respectively). When compared in terms of CNS involvement, patients with CNS involvement were younger, had a shorter disease duration, and had a higher frequency of anti-Ro/SS-A positivity than patients without those (P = 0.041, P = 0.027, and P = 0.046, respectively). Conclusions: In our study, it was shown that one third of the symptomatic pSS patients had objective neurological involvement. The presence of neurological symptoms should be considered, especially in patients with articular involvement in pSS.

A single-center rheumatology experience of sarcoidosis: observations from 70 patients.

BACKGROUND: The aim of this study is to determine the demographic, clinical and laboratory characteristics of the patients who followed up with the diagnosis of sarcoidosis, to investigate the distribution frequency of rheumatological findings and to examine the disease management from the perspective of rheumatology. METHODS: Patients who were followed up with the diagnosis of sarcoidosis in the rheumatology clinic of Ankara City Hospital between November 2019 and November 2022 were evaluated. Demographic, clinical, radiological, serological, laboratory, and histopathological findings, and rheumatological, systemic, and locomotor system examination findings of the patients were obtained from the medical data registered in the hospital. RESULTS: A total of seventy sarcoidosis patients (48.98 ± 11.78 years, %75 female) were included in the study. Joint involvement was observed in 64.3% of cases, skin involvement in 48.6% of cases, and ocular involvement in 25.7% of cases. The ankle was the most frequently involved joint, followed by the knee and small joints in the foot. Corticosteroids were the most used therapeutic agent, and pulmonary and joint findings were the most common reasons for starting treatment. CONCLUSIONS: Sarcoidosis is a disease that mimics many diseases, misdiagnosis and treatment should be avoided with a good and fast differential diagnosis. Clinicians, especially rheumatologists, should remember sarcoidosis more frequently and keep it in mind in the differential diagnosis.

The Possible Relation of Demodex and Facial Erythema in Connective Tissue Diseases.

Background: We aimed to investigate the frequency of Demodex infestation and clinical implications in connective tissue disease patients with facial erythema. Methods: Patients diagnosed with a connective tissue disease and had facial erythema were consecutively enrolled in the study from 2019-2020. An age and gender matched control group was formed from healthy volunteers. Presence of Demodex was investigated by standardized skin surface biopsy. Number of Demodex mites over 5 per centimeter square was considered meaningful for infestation. Topical or systemic metronidazole treatment was given to the connective tissue disease patients with Demodex infestation. Facial erythema visual analog scale was questioned in patients at treatment onset and one month after. Results: A total of 31 connective tissue disease patients with facial erythema were enrolled. Control group included 31 healthy volunteers. Demographics and comorbidities were similar between groups. Demodex infestation was present in 58.1% of the disease group and in 25.8% of the control group (P=0.01). Pruritus was the most common symptom in patients with infestation. Median (IQR) facial erythema visual analog scale score was 6 (3) at treatment onset and was 2 (2.5) one month later (P<0.001). Conclusion: When evaluating facial cutaneous lesions, Demodex infestation should not be overlooked in a patient group like connective tissue diseases with dysfunctional immune system.

14-3-3 η ETA protein as a potential marker of joint damage in gout.

INTRODUCTION: The aim of the study is to investigate serum levels of 14-3-3 η (ETA) protein in patients with gout and possible relations with joint damage. METHOD: This cross-sectional study included 43 gout patients and 30 control patients. RESULTS: Serum 14-3-3 η protein levels were significantly higher in gout patients (median [IQR], 3.1 [2.0] vs 2.2 [1.0], p = 0.007). In subgroup analyses of gout patients, serum 14-3-3 η protein levels did not differ between patients with and without a flare, tophaceous disease, elevated CRP and serum uric acid levels and a history of chronic kidney disease; however, were significantly higher in the patients with erosions (Median [IQR], 4.1 [2.7] vs 2.7 [1.5], p = 0.002). According to ROC curve, serum 14-3-3 η protein had 86.0% sensitivity and 30% specifity at a cut-off point of 1.7 ng/mL and had 74.7% sensitivity and 43.3% specifity at a cut-off point of 2.0 ng/mL. CONCLUSION: Our results demonstrated elevated levels of 14-3-3 η protein in gout patients which is more prominent in patients with erosive changes, implying role of 14-3-3 η protein in inflammatory and structural damage related pathways and suggesting a potential as a marker for disease severity.

Long-Term Results of Immunogenicity of Booster Vaccination against SARS-CoV-2 (Hybrid COV-RAPEL TR Study) in Turkiye: A Double-Blind, Randomized, Controlled, Multicenter Phase 2 Clinical Study.

The immunogenicity of vaccines decreases over time, causing a need for booster doses. This study aimed to present the long-term (Day 84) immunogenicity results of the double-blind, randomized, controlled, phase II Hybrid COV-RAPEL TR Study (NCT04979949), in which the TURKOVAC or CoronaVac vaccines were used as a booster after the second dose of primary vaccination with CoronaVac. A total of 190 participants from the Hybrid COV-RAPEL TR Study, who had both Day 28 and Day 84 immunogenicity results, were included. The immunogenicity on Day 84, regarding the neutralizing antibody positivity (Wuhan and Delta variants) and anti-spike immunoglobulin (Ig) G (IgG) antibody positivity, was compared between TURKOVAC and CoronaVac vaccine arms according to sex and age groups. Overall, antibody positivity showed a slight decrease on Day 84 vs. Day 28, but was not different between TURKOVAC and CoronaVac arms either for sexes or for age groups. However, TURKOVAC produced better antibody response against the Delta variant than CoronaVac, while CoronaVac was superior over TURKOVAC regarding neutralizing antibody positivity in the 50-60 years age group, regardless of the variant. A single booster dose, after the completion of the primary vaccination, increases antibody positivity on Day 28 which persists until Day 84 with a slight decrease. However, an additional booster dose may be required thereafter, since the decrease in antibody titer may be faster over time.

Rheumatology experience with FDG PET / CT in inflammation of unknown origin: a single - centre report for determining factors associated with diagnostic precision.

BACKGROUND / AIM: The use of PET / CT is becoming more common in the elucidation of inflammatory processes in which the underlying cause cannot be determined by conventional examinations. Although PET / CT is an effective method for detecting inflammatory foci, the precise diagnosis may not be obtained in all cases. In addition, considering factors such as radiation exposure and cost, it becomes important to identify patients who can get results with PET / CT. In this study, it was aimed to examine the factors that can predict the differential diagnostic value of PET / CT by retrospectively scanning patients who underwent PET / CT for inflammation of unknown origin (IUO) in rheumatology practice. METHODS: Demographic, clinical and laboratory information of the patients followed up in our clinic and who underwent PET / CT for differential diagnosis were enrolled. Whether they were diagnosed after PET / CT and during the follow - up period, and their diagnoses were examined. RESULTS: A total of 132 patients were included in the study. A previous diagnosis of rheumatic disease was present in 28.8 % of the patients, and a history of malignancy was present in 2.3 % . The patients were divided into three groups: group 1 patients with increased FDG uptake in PET / CT and diagnosis confirmed by PET / CT, group 2 patients with increased FDG uptake in PET / CT but diagnosis was not confirmed, and group 3 patients without increased FDG uptake in PET / CT. Increased FDG uptake in PET / CT was detected in 73 % of the patients. While PET / CT helped the diagnosis in 47 (35.6 %) patients (group 1), it did not help the diagnosis in 85 (64.4 %) (groups 2 and 3). Thirty - one (65.9 %) of the diagnosed patients were diagnosed with a rheumatologic disease. When the 3 groups were compared, male gender, advanced age, CRP levels, presence of constitutional symptoms, SUVmax values and number of different organs with increased FDG uptake were higher in Group 1. Sixty - six percent and 74 % of the patients in groups 2 and 3 were not diagnosed during the follow - up period. No patient in group 3 was diagnosed with malignancy during follow - up. CONCLUSION: PET / CT has high diagnostic value when combined with clinical and laboratory data in the diagnosis of IUO. Our study revealed that various factors can affect the diagnostic value of PET / CT. Similar to the literature, the statistically significant difference in CRP levels shows that patients with high CRP levels are more likely to be diagnosed with an aetiology in PET / CT. Although detection of involvement in PET / CT is not always diagnostic, there was an important finding that no malignancy was detected in the follow - up in any patient without PET / CT involvement. Key points • PET / CT is an effective method for detecting inflammatory foci. • PET / CT has proven to be effective in the diagnosis of rheumatological diseases, the extent of disease and the evaluation of response to treatment. • Indications for the use of PET / CT in the field of rheumatology and the associated factors and clinical features supporting the diagnosis with PET / CT are still to be fully clarified. • In routine practice, with PET / CT, both delays in diagnosis and examinations performed during diagnosis and the cost can be reduced.

Anti-tumor necrosis factor alpha treatment does not influence serum levels of the markers associated with radiographic progression in ankylosing spondylitis.

Objectives: The study aimed to determine the levels of change of the markers related to radiographic progression, such as Dickkopf-1 (DKK-1), sclerostin (SOST), bone morphogenetic protein (BMP)-2 and -4, and interleukin (IL)-17 and -23, in ankylosing spondyloarthritis (AS) during anti-tumor necrosis factor alpha (TNF-α) treatment. Patients and methods: Fifty-three anti-TNF-α naïve AS patients (34 males, 19 females; median: 38 years; range, 20 to 52 years) refractory to conventional treatments meeting the modified New York criteria or Assessment of SpondyloArthritis International Society classification criteria were enrolled to this cross-sectional, controlled study between October 2015 and January 2017. Fifty healthy volunteers (35 males, 15 females; median: 36 years; range, 18 to 55 years) with similar age and sex characteristics were recruited. Serum DKK-1, BMP-2, BMP-4, SOST, IL-17, and IL-23 levels were measured in both groups. The serum levels of the markers were measured again after about two years (mean follow-up duration of 21.7±6.4 months) in AS patients who started anti-TNF-α treatment. Demographic, clinical characteristics, and laboratory parameters were recorded. The disease activity at the time of inclusion was assessed through the Bath Ankylosing Spondylitis Disease Activity Index. Results: Serum DKK-1, SOST, IL-17, and IL-23 levels in the AS group before anti-TNF-a treatment were significantly higher compared to the control group (p<0.01 for DKK-1, p<0.001 for others). There was no difference regarding serum BMP-4 levels, whereas BMP-2 levels were significantly higher in the control group (p<0.01). Forty (75.47%) AS patients had serum marker levels measured after anti-TNF-α treatment. No significant change was observed in the serum levels of these 40 patients measured 21.7±6.4 months after the initiation of anti-TNF-α treatment (p>0.05 for all). Conclusion: In AS patients, there was no change in DKK-1/SOST, BMP, and IL-17/23 cascade with anti-TNF-α treatment. This finding may suggest that these pathways act independently of each other, and their local effects are not influenced by systemic inflammation.

Tofacitinib experience in patients with enteropathic arthritis.

Background/aim: The JAK-STAT pathway is involved in the pathogenesis of both the inflammatory bowel disease (IBD) and spondyloarthropathy group of diseases. The purpose of this study was to evaluate the effectiveness of tofacitinib, a Janus kinase inhibitor, in the treatment of enteropathic arthritis (EA). Materials & methods: The study included seven patients, four from the authors' follow-up and three from the literature. All cases were recorded for demographic characteristics, comorbidities, IBD and EA symptoms, medical treatments and changes in clinical and laboratory results with treatment. Results & conclusion: Clinical and laboratory remission in terms of IBD and EA was achieved in three patients after tofacitinib treatment. For both spondyloarthritis spectrum diseases and IBD, tofacitinib may be an appropriate choice, as its efficacy has been shown in both conditions.

Tofacitinib, which inhibits the JAK enzyme, is an oral, nonbiologic, disease-modifying drug used in the treatment of rheumatologic diseases. Tofacitinib modulates the immune response and reduces or prevents inflammation. There are limited data on the efficacy of tofacitinib for the treatment of enteropathic arthritis. This study aimed to evaluate the efficacy of tofacitinib in the treatment of enteropathic arthritis. Seven patients, four from the authors' follow-up and three from the literature, were included in the study. All cases were recorded in terms of demographic characteristics, comorbidities, symptoms of inflammatory bowel disease and enteropathic arthritis, medical treatments and changes in treatment and clinical and laboratory results. After tofacitinib treatment, clinical and laboratory improvement was achieved in three patients. As a result, tofacitinib may be a suitable choice for enteropathic arthritis.

Semaphorin 3A levels in vascular and nonvascular phenotypes in systemic sclerosis.

OBJECTIVE: Semaphorin 3A (Sema3A) plays a regulatory role in immune responses. The aim of this study was to evaluate Sema3A levels in patients with systemic sclerosis (SSc), especially in major vascular involvements such as digital ulcer (DU), scleroderma renal crisis (SRC), pulmonary arterial hypertension (PAH), and to compare Sema3A level with SSc disease activity. METHODS: In SSc patients, patients with DU, SRC, or PAH were grouped as major vascular involvements and those without as nonvascular, and Sema3A levels were compared between the groups and with a healthy control group. The Sema3A levels and acute phase reactants in SSc patients, as well as their association with the Valentini disease activity index and modified Rodnan skin score, were evaluated. RESULTS: The Sema3A values (mean ±â€…SD) were 57.60 ±â€…19.81 ng/mL in the control group (n = 31), 44.32 ±â€…5.87 ng/mL in patients with major vascular involvement SSc (n = 21), and 49.96 ±â€…14.00 ng/mL in the nonvascular SSc group (n = 35). When all SSc patients were examined as a single group, the mean Sema3A value was significantly lower than controls (P = .016). The SSc with major vascular involvement group had significantly lower Sema3A levels than SSc with nonmajor vascular involvement group (P = .04). No correlation was found between Sema3A, acute phase reactants, and disease activity scores. Also, no relationship was observed between Sema3A levels and diffuse (48.36 ±â€…11.47 ng/mL) or limited (47.43 ±â€…12.38 ng/mL) SSc types (P = .775). CONCLUSION: Our study suggests that Sema3A may play a significant role in the pathogenesis of vasculopathy and can be used as a biomarker in SSc patients with vascular complications such as DU and PAH.

Ustekinumab in enteropathic arthritis.

Objective: The aim of this study was to evaluate the efficacy of ustekinumab in the treatment of enteropathic arthritis. Materials & methods: A systematic literature search was performed in the Pubmed database of publications between January 2010 and October 2021. Demographic characteristics, comorbidities, inflammatory bowel disease and enteropathic arthritis symptoms, other extraintestinal findings, medical treatments and clinical and laboratory findings for all cases were recorded. Results: A total of 11 patients were included. While clinical and laboratory remission was achieved in terms of inflammatory bowel disease in all patients and enteropathic arthritis in nine patients after ustekinumab treatment, other extraintestinal findings for all patients completely regressed after treatment. Conclusion: Ustekinumab may be an appropriate treatment option for this patient group, considering both pathogenesis and successful treatment responses.

Treatments targeting the IL-23 pathway are highly effective in psoriasis, psoriatic arthritis and inflammatory bowel diseases. However, their efficacy in patients with enteropathic arthritis with peripheral and/or axial joint involvement is unclear. Ustekinumab may be a valuable option for patients who cannot adhere to other treatment options due to side effects or ineffectiveness. Considering its positive effects on joint involvement in patients with psoriatic arthritis, the current work was designed to investigate the efficacy of ustekinumab in patients with enteropathic arthritis on both gut and extraintestinal involvement, particularly musculoskeletal symptoms.

Effects of breast milk on Behçet's disease clinical features.

BACKGROUND: The etiology of Behçet's disease (BD) is not clearly known, however, abnormal activity in T helper (Th) 1, Th 17, and regulatory T cells (Treg) has critical importance in pathogenesis. It has been shown that the intestinal microbiome can be effective in the modulation of these immune abnormalities in BD patients. Breastfeeding increases the maturation of the infant's intestinal permeability by affecting the newborn's immature intestinal microbiome and metagenome. We aimed to examine the effects of breastfeeding on disease related symptoms, organ involvements and course of the disease in BD patients. METHODS: This study was designed as a cross-sectional study in Ankara City Hospital rheumatology clinic between December 2021 and March 2022. Patients who were diagnosed with BD by meeting the criteria of the 'International Study Group' and whose information we could access by agreeing to participate in the study were enrolled. The mothers of the patients were also contacted and asked whether these patients were breastfed, the duration of breastfeeding, and the mode of birth. Demographic and clinical data of the patients, comorbid diseases, and drugs used for BD were collected from the records in the hospital database. The presence of sacroiliitis in patients was evaluated with sacroiliac X-ray and/or magnetic resonance imaging (MRI), which was requested because of low back pain symptoms and only patients with previous sacroiliac imaging for low back pain were included in the study. BD-related organ damage was measured by the Vasculitis Damage Index (VDI) and Behçet's syndrome Overall Damage Index (BODI) scores. RESULTS: : A total of 304 patients were included in the study. The percentage of patients who were reported to have ever breastfed (median duration (IQR): 12(12) months, 33.5% < 6 months, 66.4% ≥ 6 months, and 59.6% ≥ 12 months) is 92%. When the breastfed and nonbreastfed patients were compared, 6.8% of the breastfed patients needed TNF-i against 18.2% of the nonbreastfed patients (p = 0.052). While the rate of having at least one comorbidity was 26.4% for those who were breastfed, this rate was 50% for those who had never been breastfed. When the organ and system involvements of the patients were compared, the incidence of sacroiliitis was statistically significantly higher in the nonbreastfed group (p = 0.025). Patients who were breastfed for less than 6 months were diagnosed with BD at an earlier age than those who were breastfed for more than 6 months, and those who were breastfed for less than 12 months compared to those who were breastfed for more than 12 months (respectively, p = 0.039, p = 0.035). DISCUSSION: Our results imply that history of breastfeeding may have some positive effects on the course of the disease in BD patients.

COVID-19 disease frequency, risk factors, and re-infection rates in patients with autoimmune rheumatic disease receiving rituximab.

INTRODUCTION: Rituximab, which is used in autoimmune rheumatic diseases (ARD), can cause both an increased risk of development of COVID-19 disease and re-infection due to its potent and long-acting immunosuppression. So, we aimed to evaluate the frequency, risk factors and re-infection rates of COVID-19 in ARD patients receiving rituximab. METHODS: A single-center retrospective study was performed with patients receiving rituximab for ARD in 12 months before the onset of COVID-19 in Turkey. The data regarding severe acute respiratory syndrome-coronavirus 2 reverse transcription polymerized chain reaction (RT-PCR) test, clinical, laboratory, and mortality data of all patients were collected from medical records. Logistic regression analysis was used for predictors of COVID-19 disease. COVID-19 re-infection was defined as RT-PCR positivity and recurrence of acute COVID-19 symptoms after at least 1 negative RT-PCR in patients with clinical improvement. RESULTS: Ninety-eight ARD patients with rituximab were evaluated and 23 (23%) of them had COVID-19. The presence of hypogammaglobulinemia increased the risk of COVID-19 disease 8-fold. COVID-19 pneumonia occurred in 13 (57%) and these patients' age was higher than those without pneumonia (59.6 ± 11.8 vs 44.9 ± 14.2 years, P = 0.013). Mortality due to COVID-19 was 13% and COVID-19 re-infection was seen in 20% of survivors. CONCLUSION: Regardless of the underlying rheumatic disease and organ involvements, hypogammaglobulinemia in ARD could be a risk factor for COVID-19 development, and advanced age could be for COVID-19 severity. Moreover, COVID-19 re-infection rates are high.

Effects of sulfasalazine in axial spondyloarthritis on COVID-19 outcomes: real-life data from a single center.

INTRODUCTION: Compared to biological agents, little is known about the impact of sulfasalazine therapy on COVID-19 outcomes in patients with Axial Spondyloarthritis (AxSpA). Therefore, we aimed to evaluate the COVID-19 severity in AxSpAs receiving sulfasalazine and biologic-agent. MATERIALS AND METHODS: A total of 219 SARS-CoV-2 positive AxSpA patients were retrospectively analyzed. COVID-19 pneumonia, hospitalization rate, and length of stay were used to determine COVID-19 severity. AxSpA patients were mainly grouped and compared as sulfasalazine and non-sulfasalazine. Afterward, we excluded no-treatment patients to reveal the drug's effects more clearly and regrouped AxSpA patients as sulfasalazine-monotherapy (34.3%), biologic-monotherapy (33.7%), and sulfasalazine + biologic (7.3%). RESULTS: Fifty-nine percent of the patients were male and the mean age was 45.0 years. Peripheral arthritis was 35% and uveitis 15%. In total, 41.5% of them have received sulfasalazine and 41.0% biologic agents, and the remaining patients with no AxSpA-specific treatment. In the first comparison, the sulfasalazine group had a higher age, more frequent COVID-19 pneumonia, hospitalization, and longer hospitalization than a non-sulfasalazine group. In the pairwise comparison of 3 treatment groups, the demographic and clinical features, the hospitalization rate and the length of hospital stay were similar but the sulfasalazine-monotherapy group had a higher frequency of COVID-19 pneumonia than the biologic-monotherapy group (23% vs. 7%, p = 0.008). CONCLUSION: Our results imply sulfasalazine may be related to more severe COVID-19 in AxSpA patients. These patients should be followed more carefully in the presence of COVID-19, regardless of reasons such as age, comorbidity, and extra-axial disease, and consideration of discontinuing sulfasalazine maybe even thought.

Eosinophilic granulomatosis with polyangiitis as a rare cause of the syndrome of inappropriate antidiuretic hormone secretion.

Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) is a rare multisystem necrotizing vasculitis that involves small- to medium-sized blood vessels. We report a rare case of syndrome of the inappropriate antidiuretic hormone (ADH) secretion (SIADH) secondary to EGPA. A 53-year-old man applied with complaints of pain in the large joints and morning stiffness in knee for 2 months. The patient had the history of impaired fasting glucose, asthma, nasal polyps, and urticaria. Physical examination revealed intrinsic muscle atrophy and weakness in the right hand. Peripheral eosinophil count was 9.78 × 109/L (0.02-0.5), erythrocyte sedimentation rate 39 mm/h (0-20), and C-reactive protein 5.77 mg/dL (0-0.5). Migratory ground-glass pulmonary opacities had been reported in previous chest computed tomography scans. Echocardiography revealed findings compatible with eosinophilic involvement. Electroneuromyographic evaluation showed acute distal axonal neuropathy of right ulnar nerve. EGPA was considered. Oral methylprednisolone treatment was initiated. Intravenous immunoglobulin (IVIG) and cyclophosphamide treatment and gradual tapering of oral steroids were planned. In 24-h urine analysis, sodium was 387 mEq, creatinine was 1156 mg, and volume was 3000 mL. When his medical records were investigated, it was observed that hyponatremia was present for nearly 2 years. While serum osmolality was 270, urine osmolality was 604 mOsm/kg H2O. So, SIADH diagnosis was made. Fluid intake was restricted. Although the patient's sodium level did not return to normal, it rose up to 130 mEq/L. After second cycle of EGPA treatment (cyclophosphamide and IVIG), serum sodium was normal. There is only four other documented cases of SIADH associated with EGPA. We hypothesized that blood supply to the hypothalamus and/or posterior hypophysis might be affected from EGPA vasculitis. Here, in this case, with effective treatment of EGPA, SIADH was resolved which implies a causality between two conditions.