Journal of Clinical Rheumatology : Practical Reports on Rheumatic & Musculoskeletal Diseases

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)...

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...

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BACKGROUND/OBJECTIVE: Despite advances in clinical care and treatment options, adolescents with lupus continue to experience adverse health outcomes. Poor adherence to medication regimens is a major contributor to these negative outcomes. The utility of short message service (SMS) in tracking barriers to adherence prospectively remains untested for adolescents with lupus. Our objectives were (1) feasibility of incorporating a Health Insurance Portability and Accountability Act (HIPAA)-compliant SMS text messaging application into REDCap and (2) acceptability of using SMS text messaging to track barriers to medication adherence in adolescents with lupus...

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...

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...

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OBJECTIVE: Protracted febrile myalgia syndrome (PFMS) is characterized by severe myalgia, fever, abdominal pain, and arthralgia/arthritis episodes lasting for several weeks in patients with familial Mediterranean fever. Treatment options include nonsteroidal anti-inflammatory drugs, corticosteroids, and anti-interleukin-1 therapy. This study aimed to share our experiences of PFMS so as to shed light on this rare and elusive condition. METHODS: This cross-sectional analysis included 17 patients diagnosed with PFMS at our pediatric rheumatology clinic between January 2018 and September 2023...

INTRODUCTION: Interferon regulatory factor 5 (IRF5) is one of the pivotal genes implicated in systemic lupus erythematosus (SLE) among diverse ethnic groups, including Europeans, Asians, Hispanics, and Africans. Notably, its significance appears particularly pronounced among Hispanic populations. Previous studies have identified several single-nucleotide variants within IRF5, such as rs2004640G/T, rs2070197T/C, and rs10954213G/A, as associated with susceptibility to SLE among patients from Mexico City...

BACKGROUND: Hospital-acquired bacterial infections are associated with high morbidity and mortality rates in patients with systemic lupus erythematosus (SLE). This study aimed to develop and validate predictive models for the risk of hospital-acquired bacterial infections in patients with SLE. METHODS: A historical cohort study was designed for development, and another bidirectional cohort study was used for external validation. The risk of bacterial infection was assessed upon admission and after 5 days of hospitalization...

INTRODUCTION: Audiovestibular dysfunction has been reported in many autoimmune connective tissue diseases, including systemic sclerosis (SSc). OBJECTIVE: To assess the prevalence and features of audiological and vestibular disturbances in SSc patients and evaluate their relationship with disease duration, clinical features, nailfold videocapillaroscopy pattern, and immunologic profiles. METHOD: A matched case-control study was conducted in a rheumatology clinic of a second-level hospital over 24 months...

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OBJECTIVE: ​​This study aimed to characterize the profile of myositis-specific and myositis-associated autoantibodies (MSAs/MAAs) in an Indian cohort of juvenile dermatomyositis (JDM) patients and correlate them with clinical features and outcomes. METHODS: Forty-three children diagnosed with JDM were enrolled for this observational study. Clinical details (presentation, course, and outcome) were noted in a predesigned proforma. Serum samples were tested for 16 MSAs/MAAs by line immunoassay...

OBJECTIVE: The exact effects of MEFV variants on inflammation are still under investigation, and reports on variants of unknown significance, particularly the E148Q variant, have been conflicting. Therefore, this study aims to investigate patients exhibiting E148Q heterozygosity, focusing on diagnoses and disease courses to assist physicians in interpreting the variant. METHODS: Data of pediatric patients presenting to the Pediatric Rheumatology clinic between November 2016 and September 2023, exhibiting only E148Q heterozygosity in MEFV gene analysis, were extracted...

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OBJECTIVES: To determine the association between radiologic joint damage (JD) and a lower lean body mass (LBM) in rheumatoid arthritis (RA) patients. METHODS: A cross-sectional study from a single center established RA cohort. JD and appendicular LBM (arms and legs) were measured with the Sharp/van der Heijde (SvdH) score and dual x-ray absorptiometry expressed as kg/m2, respectively. A univariable analysis was used to determine the association between JD an LBM; then, a multivariable regression model was performed to evaluate the persistence of this association, adjusted by age, gender, disease duration, socioeconomic status (by the Graffar method), tobacco use, anticitrullinated protein antibody levels, Disease Activity Score in 28 joints for RA with erythrocyte sedimentation rate, glucocorticoid use (as prednisone equivalent), disease-modifying antirheumatic drug use, body mass index, and disability (by the multidimensional Health Assessment Questionnaire)...

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Women with systemic chronic inflammatory disease, such as those with scleroderma, systemic vasculitis, and Sjögren syndrome, need preconception evaluation by a multidisciplinary team. Counseling and pregnancy management should be tailored to patients' needs, considering specific disease features, organ involvement, treatment options, and risk factors to minimize risks of maternal-fetal complications during pregnancy.Additionally, considerations regarding fertility, assisted reproductive techniques, and contraception also need to be addressed for these women...