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Tofacitinib is an approved treatment for rheumatoid arthritis (RA), but data on its use in the "real-world" are limited. We sought to analyse tofacitinib drug survival in the Israeli registry and compare it to other biologic agents.We included RA patients treated with tofacitinib, etanercept, golimumab, tocilizumab, or abatacept between 2010-2019. The primary endpoint was event-free survival (EFS), defined as the time from treatment initiation to a treatment failure event from any cause (i.e., inefficacy or intolerability). EFS was compared between agents using Cox regression and Kaplan-Meier analysis, stratifying patients by treatment line.A total of 964 eligible treatment courses were included (tocilizumab [325], etanercept [284], abatacept [127], tofacitinib [139], and golimumab [109]). In a univariate analysis, EFS with tofacitinib in the complete cohort was similar to etanercept, golimumab, and abatacept but was lower than tocilizumab) 3-year EFS 43% vs. 53%, HR 0.65). In a multivariable analysis, tofacitinib was similar to all other drugs, except for etanercept, which was inferior (HR 1.70); advanced treatment line was also associated with greater risk for failure (HR 1.64). In a univariable analysis stratified by the treatment line, tofacitinib had similar or better drug survival than other agents in the first and second lines. In the third line and beyond, tocilizumab had a higher EFS compared to tofacitinib (HR 0.57).Drug survival with tofacitinib is related to treatment line. Early introduction is associated with similar or better survival than other agents, whereas tocilizumab was superior in the third line or later.

OBJECTIVE There is an unmet need for a reliable biomarker for the differentiation of axial spondyloarthritis (AxSpA) from its mimickers. Serum levels of interleukin-22 (IL-22) have previously been found to be significantly elevated in patients with AxSpA compared with healthy individuals or persons with osteoarthritis. METHODS Consecutive patients with established or suspected AxSpA were enrolled. The clinical data, as well as results of laboratory and imaging studies, were acquired from patients' charts. The final diagnosis of definite or probable SpA, or an alternative diagnosis, was determined, and the serum levels of IL-22 were examined by enzyme-linked immunosorbent immunoassay. RESULTS Interleukin-22 levels were significantly higher in patients with definite AxSpA (29 patients) compared with patients with alternative diagnoses (14 patients) and healthy volunteers (16 individuals; P

Nailfold video capillaroscopy (NVC) enables us a direct view of the microvasculature. Only several capillaroscopy studies in adult patients with vasculitis have been reported.To characterize NVC changes in vasculitis.Vasculitis patients and healthy controls were evaluated by NVC. NVC changes associated with vasculitis were assessed retrospectively in a cohort of 100 patients with Raynaud's phenomenon (RP).17 patients with active vasculitis and 8 patients with vasculitis in remission were compared to 25 age and sex-matched healthy controls. Active vasculitis patients demonstrated higher rates of neoangiogenesis and capillary loss in comparison to other groups. Two novel NVC abnormalities were observed in patients with vasculitis: "Rolling" (slow capillary flow) and "peri-capillary stippling" (PCS), small deposits that may represent capillary leak. PCS was observed exclusively in 5 of 17 patients with active vasculitis. Retrospectively, we were able to detect PCS also in 14 % of 100 patients that were evaluated for RP, of whom 64 % were diagnosed with scleroderma or a related disorder.Patients with active vasculitis demonstrate frequent capillary abnormalities. Although these abnormalities are non-specific, we suggest that their combination may aid the diagnosis of vasculitis. Future studies are needed to validate our findings.

Facet joints' (FJ) ankylosis was reported in patients with radiographic axial spondyloarthritis (r-AxSpA). However, a detailed FJ evaluation over the whole spectrum of AxSpA was not performed. We aimed to analyze structural lesions in the FJ of patients with different forms of AxSpA, using computed tomography (CT).CT studies of the cervical/thoracic/lumbar spine, or of the chest/abdomen of patients with r-AxSpA or non-radiographic AxSpA (nr-AxSpA) (age ≤ 50 years) were analyzed for the presence of erosions, ankylosis, joint-space narrowing, osteophytes, subchondral sclerosis, subchondral cysts and vacuum phenomenon. Age- and gender-matched subjects without known rheumatic disease who performed spinal CT, formed the control group. Findings were compared between groups, separately for each spinal segment. Further, FJ findings between three subgroups of the axSpA subjects, including r-AxSpA with or without syndesmophytes, and nr-AxSpA, were compared.959/666 FJs (49/44 patients) were assessed in the AxSpA/control group patients, respectively. The study group consisted of 16 r-AxSpA patients with syndesmophytes and 22 r-AxSpA patients without syndesmophytes, and 11 nr-AxSpA patients. FJ ankylosis was significantly more prevalent in all spinal segments of the r-AxSpA patients with syndesmophytes. Erosions were seen almost exclusively in patients with r-AxSpA. Joint-space narrowing and osteophytes were noted in all segments and all subgroups of AxSpA patients, including those with nrAxSpA.Disease-specific FJ changes present almost exclusively in patients with r-AxSpA, while degenerative FJ changes are prevalent in all spinal segments and all AxSpA subgroups, suggesting that FJs can be affected early in the disease course.

Purpose To reassess the diagnostic approach to a patient with a monoarticular disease in light of the up-to-date medical literature and to examine the practical utility of traditional and newer imaging tools in the setting of monoarthritis. Results The monoarticular disease can represent a medical emergency on the one hand and be a diagnostic conundrum on the other. The management rules of patients with monoarthritis have been established long ago, but various pitfalls still lead physicians off the right diagnosis at times. Septic, pseudoseptic arthritis and hemarthrosis are the most common diagnoses made in patients with an acute presentation, and a decision not to perform a diagnostic arthrocentesis is the most prevalent cause of misdiagnosis in this setting. Many rheumatic and infectious diseases can present with more indolent monoarthritis; careful history and physical examination frequently provide clues to the straightforward diagnosis in some cases, but the extensive investigation is needed in others. Imaging methods become indispensable in individuals with the non-inflammatory monoarticular disease, with magnetic resonance imaging being the gold standard for diagnosing pigmented villonodular synovitis, lipoma arborescence, avascular necrosis, or neuropathic arthropathy. Conclusions A great variety of medical disorders can present as a monoarticular disease. The disease presentation dictates different diagnostic behavior, while knowing the available imaging methods' diagnostic potential should further shorten the diagnostic process.

Clinical decisions in allogeneic hematopoietic stem cell transplantation (allo-HSCT) are supported by the use of prognostic scores for outcome prediction. Scores vary in their features and in the composition of development cohorts. We sought to externally validate and compare the performance of 8 commonly applied scoring systems on a cohort of allo-HSCT recipients. Among 528 patients studied, acute myeloid leukemia was the leading transplant indication (44%) and 46% of patients had a matched sibling donor. Most models successfully grouped patients into higher and lower risk strata, supporting their use for risk classification. However, discrimination varied (2-year overall survival area under the receiver operating characteristic curve [AUC]: revised Pretransplantation Assessment of Mortality [rPAM], 0.64; PAM, 0.63; revised Disease Risk Index [rDRI], 0.62; Endothelial Activation and Stress Index [EASIx], 0.60; combined European Society for Blood and Marrow Transplantation [EBMT]/Hematopoietic Cell Transplantation-specific Comorbidity Index [HCT-CI], 0.58; EBMT, 0.58; Comorbidity-Age, 0.58; HCT-CI, 0.55); AUC ranges from 0.5 (random) to 1.0 (perfect prediction). rPAM and PAM, which had the greatest predictive capacity across all outcomes, are comprehensive models including patient, disease, and transplantation information. Interestingly, EASIx, a biomarker-driven model, had comparable performance for nonrelapse mortality (NRM; 2-year AUC, 0.65) but no predictive value for relapse (2-year AUC, 0.53). Overall, allo-HSCT prognostic systems may be useful for risk stratification, but individual prediction remains a challenge, as reflected by the scores’ limited discriminative capacity.

Background Prediction of clinical outcomes following allogeneic hematopoietic stem cell transplantation (Allo-HSCT) may promote informed decisions and treatment personalization. We sought to evaluate the predictive performance of six commonly used models in the prediction of transplantation outcomes. Methods This was a single-center retrospective study including patients undergoing Allo-HSCT between 2011 and 2015. We calculated the following scores: Hematopoietic Cell Transplantation-Comorbidity Index (HCTCI), Comorbidity-Age index (AGE-HCTCI), Endothelial Activation and Stress Index (EASIx), Pretransplant Assessment of Mortality (PAM) and revised PAM (rPAM) scores, and European Group for Blood and Marrow Transplantation (EBMT) score. Predictive accuracy was measured by discrimination (AUC), a measure ranging from 0.5 to 1, corresponding with poor to excellent performance. Outcomes evaluation were overall survival (OS), non-relapse mortality (NRM), and relapse. Results Among 528 patients included, indications for transplantation varied, with acute myeloid leukemia (44%) being most common. Non-malignant causes accounted for 3% of cases. Most patients received myeloablative conditioning (74%, MAC) and grafts from HLA matched-sibling donors (46%). Median follow-up was 2.5 years (intra-quantile range 1.7-3.9). The rPAM, which includes patient, disease, donor, and transplantation characteristics, had the highest discrimination for OS, NRM, and relapse across all time-points (Figure 1). EASIx, a biomarker based-model integrating pre-transplantation creatinine, lactate dehydrogenase, and thrombocyte levels, had among the highest AUCs for day-100 OS. Across outcomes, AUCs were mostly 0.6 or below for the HCT-CI, AGE-HCTCI, and EBMT score. In a sub-analysis, the rPAM, EASIx, and AGE-HCTCI were more accurate in the prediction of 2-year OS in patients receiving MAC, while the EBMT score did better in patients receiving reduced-intensity conditioning (Figure 2). Conclusions Prognostic models in Allo-HSCT have limited predictive performance. Among models evaluated, the rPAM had the highest discrimination overall. Biomarker-based prediction with EASIx is promising for the prediction of short-term outcomes.

A 54-year old man, mechanically ventilated after cardiopulmonary resuscitation secondary to constrictive effusive metastatic pericarditis of primary lung cancer, developed acute kidney failure. Creatinine increased from 2.4 to 8.8 mg/dl in 10 days, with concomitant hyperkalemia (serum potassium1⁄4 6 mEq/l). Treatment of the hyperkalemia included continuous insulin infusion, beta agonists, and 30 g of sodium polystyrene sulfonate (SPS)—sorbitol free, three to four times a day, for a duration of 10 days, administered via a nasogastric tube. A trial of hemodialysis was abstained because of hemodynamic instability. After 12 days, the patient passed away. On post-mortem examination, a huge, non-obstructive bezoar (Figure 1) was incidentally found in a significantly dilated but undamaged stomach. Analysis of the bezoar revealed inorganic material diagnosed as SPS (Figure 2), which is a strongly acidic cation exchange resin, widely used orally or by enema for managing hyperkalemia via binding and removing of potassium in the bowel in return for sodium ions. Reports on SPS-associated gastrointestinal injury have mainly involved the lower tract,1 although one case series described five newborns with SPS gastric bezoars.2 In the presented patient, possible mechanisms precipitating impaction of SPS might include the following: (1) reduced gastric mobility secondary to uremia or mesenteric ischemia; (2) reduced gastric secretions leading to SPS crystallization; (3) high doses of SPS; and (4) redissolution of SPS in the presence of high concentrations of multivalent salts, which may cause condensation and precipitation of soluble polystyrene sulfonate in solution.3 Further research is warranted to clarify the exact mechanism underlying bezoar formation with SPS administration. Nevertheless, it appears that unstable critically ill patients, as the one described, are at risk for upper gastrointestinal injury––an increasingly recognized complication of SPS treatment.