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Glucocorticoids (GCs) are still the mainstay of treatment in systemic vasculitides. Immunosuppressive agents such as cyclophosphamide, rituximab, azathiopurine, and mycophenolate mofetil are chosen as steroid sparing agents according to the type of vasculitis. Biologic treatments such as tumor necrosis factor inhibitors and tocilizumab are used in particularly large vessel vasculitis (LVVs) for refractory patients. Janus kinase (JAK)-signal transduction activator of transcription (STAT) pathway activation is involved in the pathogenesis of several inflammatory diseases. JAK inhibitors were also approved for the treatment of rheumatoid arthritis (RA), psoriatic arthritis, and ulcerative colitis. However, there are very limited data including mostly case series and open studies with JAK inhibitor usage in systemic vasculitides. Current data mostly come from LVVs and some from Behçet's disease. In the light of current data, we are quite far from suggesting the common usage of JAK inhibitors in systemic vasculitides. JAK/STAT pathway inhibition also may cause severe complications in these group of patients treated with higher dose GC and more potent immunosuppressives compared to RA and ankylosing spondylitis. Although we have limited data showing the efficacy of the JAK inhibitors for systemic vasculitis treatment, they may be used in patients refractory to standard immunosuppresives. JAK inhibitors seem to be promising therapeutic agents, especially for treating LWs. There are ongoing controlled studies with tofacitinib and upadacitinib in TAK; upadacitinib and baricitinib in giant cell arteritis. Larger and controlled studies will clarify the efficacy and safety of JAK inhibitors in the treatment of systemic vasculitides. [ABSTRACT FROM AUTHOR]

Objective: To assess the frequency and related factors of hypogammaglobulinemia (HGG) and severe infections in patients who received rituximab (RTX) for rheumatic diseases during routine followup. Methods: Patients who were followed in Marmara University Rheumatology Clinic and received RTX are evaluated retrospectively. The immunoglobulin (Ig) G, IgM, IgA levels and clinical manifestations were obtained from patient files. The HGG frequency and related factors were assessed. Severe infections were also analysed. Results: A total of 144 patients were included (F/M: 105/39, mean age 52.8±13.8). In the majority of the patients (67%) the diagnosis was rheumatoid arthritis (RA). At least one subgroup of HGG was observed in 30% (43/144) of the patients. During follow-up 17 (11.8%) patients had low IgG, 37 (26%) low IgM, and 7 (4%) had low IgA levels. HGG rate was similar between RA, connective tissue diseases and anti-neutrophil cytoplasmic antibody associated vasculitis patients (25%, 30%, 47%, respectively, p=0.13). HGG was more frequent in men (p=0.028), in patients with higher accumulated RTX dose (p=0.006) and with hypertension (p=0.033). Concomitant use of disease-modifying anti-rheumatic drugs, glucocorticoid use and prior cyclophosphamide was not associated with higher HGG. Methotrexate use with RTX was a protective factor for HGG (HGG rate methotrexate + vs -: 12% vs 33%, p=0.032). Two patients with HGG (5%) received intravenous immunoglobulin replacement. Twenty-seven patients (18%) had severe infections. Lower IgG levels [IgG levels odds rato (OR) (95% confidence interval [CI]) 0.82 (0.70-0.96), p=0.018] and chronic lung disease (CLD) [CLD present OR (95% CI) 3.7 (1.2-10.8), p=0.017] were associated with severe infections in multivariate analysis. A total of 38 patients died during follow-up. Mortality was more frequent in patients with HGG [mortality rate HGG+ vs HGG -: 40% (17/43) vs 21% (20/101), p=0.02]. Conclusion: While male gender, increased number of RTX courses and hypertension were found to be risk factors for HGG, CLD and lower IgG levels were associated with severe infections. Therefore, measuring Ig levels and assessing risk factors for HGG and severe infections during RTX treatment may provide information to prevent both conditions. [ABSTRACT FROM AUTHOR]

Background: Antiphospholipid syndrome is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis. Chronic thromboembolism is one of the known established pathogenesis of pulmonary hypertension, known as chronic thromboembolic pulmonary hypertension. Pulmonary endarterectomy is the treatment of choice for chronic thromboembolic pulmonary hypertension. The aim of this study is to evaluate the efficacy and risk of pulmonary endarterectomy in patients with antiphospholipid syndrome-associated chronic thromboembolic pulmonary hypertension. Methods: Data were prospectively collected and retrospectively analyzed, for patients who underwent pulmonary endarterectomy between March 2011 and March 2020. Results: Seventeen patients (4 male and 13 female) were identified. Thirteen patients had primary antiphospholipid syndrome and 4 had secondary antiphospholipid syndrome. The mean age was 34.82 ± 10.07 years and the mean time interval between the diagnosis and surgery was 26.94 ± 17.35 months. Dyspnea on exertion was the main symptom in all patients. Seven patients had previous deep vein thrombosis, 5 patients had a history of recurrent abortions, and 2 patients had hemoptysis. Following surgery, mean pulmonary artery pressure decreased from 47.82 ± 13.11 mm Hg to 22.24 ± 4.56 mm Hg (P < .001), and pulmonary vascular resistance improved from 756.50 ± 393.91 dyn/s/cm-5 to 298.31 ± 132.84 dyn/s/cm-5 (P < .001). There was no in-hospital mortality with a mean follow-up of 75.29 ± 40.21 months. The functional capacity of all patients improved from 269.46 ± 111.7 m to 490 ± 105.34 m on a 6-minute walking test. Conclusions: Pulmonary endarterectomy is a safe and curative treatment in patients with antiphospholipid syndrome-associated chronic thromboembolic pulmonary hypertension. It has a favorable outcome by increasing the quality of life. A multidisciplinary experienced chronic thromboembolic pulmonary hypertension team is critical in the management of these unique patients. [ABSTRACT FROM AUTHOR]

Objective To evaluate key factors for presenteeism and activity impairment in multinational patients with Behçet's syndrome (BS) and recurrent aphthous stomatitis (RAS). Methods In this cross-sectional study, 364 BS patients from Jordan, Brazil, the United Kingdom and Turkey and 143 RAS patients from the United Kingdom and Turkey were included. The Work Productivity Activity Impairment (WPAI) scale was used for presenteeism and activity impairment. Mediation analyses were performed to evaluate both direct and indirect causal effects. Results Presenteeism score was higher in active patients with genital ulcers and eye involvement as well as patients with comorbidities and current smokers than the others in BS (P  < 0.05). In RAS, presenteeism score was elevated by oral ulcer activity in the direct path (P  = 0.0073) and long disease duration as a mediator in the indirect path (P  = 0.0191). Patients with active joint involvement had poor scores in absenteeism, presenteeism, overall impairment and activity impairment compared with those of inactive patients (P  < 0.05). Using mediation analysis, the activity impairment score was directly mediated by joint activity (P  = 0.0001) and indirectly mediated through oral ulcer-related pain in BS (P  = 0.0309). Conclusion In BS, presenteeism was associated with disease activity, presence of comorbidities and being a current smoker, whereas in RAS, presenteeism was associated with oral ulcer activity and increased length of the disease. Moreover, activity impairment was adversely affected by joint activity and oral ulcer related pain in BS. Patients need to be empowered by using appropriate treatment strategies in their working environment and daily life. [ABSTRACT FROM AUTHOR]

Background/aim: The aim of this study was to evaluate the value of the PET vascular activity score (PETVAS) during the follow-up of patients with Takayasu arteritis. Materials and methods: Takayasu arteritis patients who underwent 18F-Fluorodeoxyglucose (FDG) PET imaging were evaluated retrospectively. In 8 patients both 1 and 2-h imagings were also performed prospectively. For PETVAS, 9 arterial areas were scored between 0-3 according to the FDG uptake. Results: Forty-six images of 34 patients were evaluated. PETVAS was higher in patients with clinically active disease (p = 0.03) and in the C-reactive protein (CRP) elevated group among clinically inactive patients (p = 0.0015). PETVAS correlated with CRP (p = 0.003, r = 0.53) and erythrocyte sedimentation rate (p = 0.005, r = 0.41), whereas age, disease duration, immunosuppressive, and glucocorticoid (GC) treatments were not associated with PETVAS. First vs. 2nd-h PETVAS was similar in patients who had both 1st and 2nd h PET scans (p = 0.67). Conclusion: We observed higher PETVAS in patients with active disease and elevated acute phase reactants. Although scores in our study (performed at one-h) were lower compared to the original PETVAS study performed at two h, PETVAS seems to be a reliable tool to quantify FDG PET scores in routine practice. [ABSTRACT FROM AUTHOR]