JCR: Journal of Clinical Rheumatology最新文献

Objectives: This study aims to investigate the activation of the coagulation system of RA patients and assess changes during anti-inflammatory treatment with tumor necrosis factor blockers (anti-TNF) and Janus kinase inhibitors (JAKi).

Methods: Biomarkers for the coagulation system, including D-dimer, fibrinogen, prothrombin time, activated partial thrombin time, prothrombin fragment 1 + 2, thrombin-antithrombin complex (TAT), activated factor IX, antithrombin complex, and von Willebrand factor (vWF), were longitudinally measured in 83 RA patients treated with anti-TNF and 38 RA patients with JAKi. Data were collected at baseline, after 1, 3, and 6 months.

Results: The mean age was 57 (±14) years; 76% was female. The mean DAS28-CRP was 3.6 (±1.3) for anti-TNF users and 4.1 (±1.4) for JAKi users at baseline and declined in both groups. Baseline coagulation markers levels were comparable between groups. In anti-TNF users, D-dimer and fibrinogen levels significantly declined (-0.31 mg/L, p = 0.01 and -0.71 g/L, p < 0.001, respectively), whereas TAT significantly increased after 6 months follow-up (1.46 μg/L, p = 0.03) and no effect on vWF ( p = 0.98). In JAKi users, vWF declined significantly during the 6 months follow-up (-37.41%, p < 0.001); additionally, there were reductions of D-dimer, fibrinogen, and TAT that did not reach significance (-0.17 mg/L, p = 0.59; -0.49 g/L, p = 0.12; and 0.68 μg/L, p = 0.27, respectively).

Conclusions: The prothrombotic tendency in active RA declined during effective treatment with both anti-TNF and JAKi. Altogether, the biomarkers used in this study suggest that an increased VTE risk in the first 6 months due to either treatment with anti-TNF or JAKi is unlikely.

Objectives: Pulmonary arterial involvement (PAI) is one of the most common causes of mortality in Behçet's disease (BD). In this study, we aimed to evaluate the clinical features, course, and recurrence risk factors of BD-associated PAI.

Methods: BD patients who were followed up in Marmara University BD outpatient clinic between 1990 and 2023 were included. All data were acquired from the patients' medical records. PAIs were classified according to the type of the vascular involvement as thrombosis or aneurysm. Factors affecting the risk of relapses were determined using multivariate Cox regression analysis.

Results: Among 1350 BD patients, 110 (8.1%) had PAI. The mean age (SD) of patients with PAI was 42.4 (11.6) years, and the male/female ratio was 2.2 (76/34). Thirty-two (29.1%) of 110 patients were asymptomatic. Asymptomatic patients with PAI were significantly younger ( p = 0.031) than the symptomatic group. Also, a greater prevalence of females ( p = 0.001) and higher recurrence rates ( p = 0.019) were observed in the symptomatic group. Pulmonary arterial thrombosis was seen in 104 (94.5%) and aneurysms in 9 patients (6.6%). At least one PAI relapse was observed in 31 patients (28.2%). In multivariate analysis, the Cox regression model was significant ( p = 0.013), and not starting anticoagulants independently increased the PAI relapse risk (hazards ratio, 4.36; 95% confidence interval, 1.14-24.1; p = 0.042).

Conclusions: Pulmonary arterial thrombosis is the main presentation type of PAI in BD, whereas aneurysmatic formation is rare. Despite immunosuppressive treatment, relapses occur during follow-up in one third of patients with PAI. When anticoagulant therapy is added to immunosuppressive therapy, the relapse rate in BD patients with PAI is significantly reduced.

Background: Shared decision-making (SDM) is a principle of humanistic, patient-centered health care within the field of rheumatology. However, clear communication between patients and their clinicians regarding the benefits and risks of immunomodulators may be challenging in a clinical setting. The design-thinking process is a human-centered approach to quality improvement that can help to identify insights to uphold high-quality communication.

Methods: The development process adhered to the Stanford design thinking process framework, encompassing 5 stages: (1) empathize, (2) define, (3) ideate, (4) prototype, and (5) test. During the empathy stage, quality improvement members spent 4 hours immersed in the clinical setting observing how patients and clinicians engage in SDM conversations. These observations were augmented by unstructured debriefing sessions to better understand the needs and drivers of high-quality SDM. Following this, a rapid ideation workshop was convened to generate creative solutions. These led to rapid prototyping and testing, yielding a final product.

Results: The iterative design process identified 4 critical needs: (1) ensuring comprehensibility of materials, (2) upholding accuracy of information, (3) balancing standardization with individualization, and (4) promoting retention of knowledge. During the rapid ideation workshop, the concept of a Worksheet for Immunomodulator Shared Decision-Making (WISDM) was introduced and selected for further elaboration. This led to the creation of 5 prototypes for methotrexate, which were subsequently tested. These were reconciled and modified to make a final product.

Conclusion: The WISDM template contains 7 elements that support SDM. Forty-five WISDMs were created for 23 immunomodulators. Further investigation will focus on how WISDMs exactly impact SDM.

Abstract: Wong-type dermatomyositis (WTDM) was first formally discussed in the literature in 1969 by Dr. K.O. Wong. This rare variant of dermatomyositis (DM) is characterized by overlapping features of both classic DM and the cutaneous features of pityriasis rubra pilaris. Since 1969, few cases of WTDM have been published in the literature likely due to the rarity of this condition or lack of recognition by clinicians. This narrative review presents the current published English literature on WTDM, analyzing its clinical presentation, diagnostic testing, and treatments along with a comparison to classic DM. Given the overlap of features of both diseases and patients experiencing a better response to classic DM treatments, our results suggest that WTDM is a rare subtype of DM rather than simply an overlap of pityriasis rubra pilaris and DM presenting in 1 patient. We suggest that clinicians evaluate WTDM patients with very thorough histories, physical examinations, histopathology, and appropriate serological studies and monitor closely for systemic symptoms and development of malignancy. WTDM should be treated using conventional treatments for classical DM. Further studies are needed to understand the pathogenesis of WTDM including more specific and distinguishing autoantibody profiles from classical DM, as well as long-term clinical course of WTDM for best management, including recently available biological treatments.

Objective: Diagnosis of periprosthetic joint infection (PJI) in patients with inflammatory arthritis (IA) is challenging, as features of IA flares can mimic infection. We aimed to cross-sectionally determine if the optimal tests to diagnose PJI in osteoarthritis were present in patients with IA flares.

Methods: We enrolled patients from October 2020 to July 2022 in 3 groups: ( a ) PJI-total joint arthroplasty patients undergoing revision for infection, ( b ) IA Flare-IA patients with a flaring native joint, and ( c ) IA Aseptic-total joint arthroplasty patients with IA undergoing aseptic arthroplasty revision. We compared blood and synovial fluid markers between the cohorts using Kruskal-Wallis and Fisher exact tests to assess marker sensitivity and specificity.

Results: Of 52 cases overall, 40% had rheumatoid arthritis, 20% psoriatic arthritis, and 11% osteoarthritis (in PJI group). PJI cases had higher C-reactive protein (CRP) and synovial fluid polymorphonuclear neutrophil percentage (%PMN). Alpha-defensin tested positive in 93% of PJI cases, 20% of IA Flares, and 6% of IA Aseptic ( p < 0.01). Synovial white blood cell count >3000/μL and positive alpha-defensin were highly sensitive (100%) in diagnosing infection; however, specificity was 50% for white blood cell counts and 79% for alpha-defensin. PJI diagnosis was nearly 5 times more likely with positive alpha-defensin and almost 6 times more likely with %PMNs >80. Blood markers interleukin-6, procalcitonin, and d -dimer were neither sensitive nor specific, whereas erythrocyte sedimentation rate and CRP showed 80% sensitivity, but 47% and 58% respective specificities.

Conclusions: Although synovial %PMNs, CRP, and alpha-defensin are sensitive tests for diagnosing PJI, they are less specific and may be positive in IA flares.

Introduction: There is scarce information on the prevalence of axial spondylarthritis (axSpA) using the Assessment of SpondyloArthritis International Society (ASAS) criteria and even less in Latin America. This study aimed to estimate the prevalence of axSpA by applying the ASAS 2009 criteria to a medical records review study of young people with chronic low back pain (LBP) at a university hospital-based health management organization.

Methods: Electronic medical records from the Hospital Italiano de Buenos Aires health management organization were reviewed to estimate the prevalence of axSpA (radiographic axSpA [r-axSpA] and nonradiographic axSpA [nr-axSpA]) using the ASAS 2009 axSpA criteria in all patients with chronic LBP (≥3 months) aged <45 years at the first LBP appointment, observed between 2009 and 2019.

Results: Among 795 young people with CLBP, the estimated prevalence of axSpA was 5.78% (r-axSpA, 2.76%; nr-axSpA, 3.02%). Ten of 46 patients (21.74%) with axSpA (all nr-axSpA) were undiagnosed, with an undiagnosed axSpA prevalence of 1.26%. The median interval between the first LBP appointment and diagnosis was 34.6 months for axSpA (58.7 vs. 23.1 months for r-axSpA vs. nr-axSpA). Previously diagnosed r-axSpA and nr-axSpA patients had comparable use of biological disease-modifying antirheumatic drugs (bDMARDs) (45% vs. 36%) and delays between nonsteroidal anti-inflammatory drug failure and bDMARD initiation (median, 2.76 vs. 2.66 months).

Conclusion: In our cohort of young persons with chronic LBP, the prevalence of axSpA was approximately 6%, with a high prevalence of undiagnosed axSpA, which could explain the low prevalence of axSpA reported in previous studies in Latin America.

Abstract: Lyme disease is commonly associated with musculoskeletal features, inflammatory and noninflammatory. The precise pathogenesis of the clinical features of this infection are complex and often multiple. A better understanding of how Borrelia burgdorferi causes these musculoskeletal manifestations is necessary in order to determine the proper treatment and eschew that which is unlikely to work, often associated with toxicities. The following review seeks to summarize the various immunopathogenic mechanisms that may cause these features of Lyme disease and suggests a series of approaches based on the most likely underlying mechanism(s).

Objectives: Behçet disease (BD) has heterogeneous presentations, mainly mucocutaneous, vascular, and ocular manifestations. The mechanisms associated with different phenotypes have not been clarified. We aimed to investigate the expression of innate and adaptive immunity-related cytokines in these 3 main BD phenotypes in active and untreated states and remission after treatment to be able to develop a cytokine-based treatment algorithm.

Methods: Serum samples were isolated from 41 patients with newly diagnosed active BD (aBD), which consisted of 19 mucocutaneous aBD, 11 ocular aBD (o-aBD), and 11 vascular aBD patients, 35 patients in remission (rBD), and 9 healthy controls (HC). Serum levels of each cytokine were measured with sandwich enzyme-linked immunosorbent assay and analyzed as both raw measurements and corrected levels for each 1 million white blood cells.

Results: The study included 41 aBD patients (female/male [F/M]: 9/32; median age, 29 years), 35 rBD patients (F/M: 9/26; median age, 29 years), and 9 HC (F/M: 3/6; median age, 28 years). The serum interferon γ level was significantly higher in the aBD group than in the rBD (116 vs. 92 pg/mL, p = 0.022). The serum interleukin 35 (IL-35) level was significantly higher in the HC group compared with aBD and rBD ( p = 0.05). IL-17-related cytokines were lower in o-aBD. With treatment, they increased in o-aBD but decreased in mucocutaneous aBD and vascular aBD patients.

Conclusion: This study supports the involvement of both innate and T H 1-predominated adaptive immune responses across all BD phenotypes. The IL-17 and T H 17-related immune responses appear less prominent in ocular BD, which may explain the ineffectiveness of IL-17 blockade in treating ocular BD. These findings support the need for further studies using comprehensive gene expression analyses to develop targeted treatment strategies for BD phenotypes.