OBJECTIVES: Systemic glucocorticoids are commonly used as short-term bridging treatment to control disease activity in juvenile idiopathic arthritis (JIA). This study aimed to analyze the factors affecting the use of sys...OBJECTIVES: Systemic glucocorticoids are commonly used as short-term bridging treatment to control disease activity in juvenile idiopathic arthritis (JIA). This study aimed to analyze the factors affecting the use of systemic glucocorticoid treatment and cumulative dose during the first year after JIA diagnosis. METHODS: This retrospective study included children who were diagnosed with JIA according to the International League for Rheumatology (ILAR) classification criteria, excluding systemic JIA, and followed up for at least 1 year. All systemic glucocorticoid treatments administered during follow-up were converted to prednisolone-equivalent doses, and the total cumulative dose was calculated for each patient. RESULTS: The study included 164 patients (53% female), of whom 110 (67.1%) received systemic glucocorticoid treatment. Higher baseline Juvenile Arthritis Disease Activity Score-71 (JADAS-71) scores (OR: 1.149, 95% CI: 1.003-1.316, p=0.045) and ankle joint involvement (OR: 5.740, 95% CI: 1.134-29.050, p=0.035) were associated with an increased use of systemic glucocorticoid treatment, while enthesitis-related arthritis (ERA) was associated with a decreased use (OR: 0.132, 95% CI: 0.036-0.487, p=0.002). Ankle involvement (β: 1.434, 95% CI: 0.328-2.539, p=0.012), antinuclear antibody (ANA) positivity (β: 1.232, 95% CI: 0.181-2.283, p=0.022), and higher baseline JADAS-71 scores (β: 0.101, 95% CI: 0.001-0.202, p=0.046) were strongly associated with increased cumulative systemic glucocorticoid dose. CONCLUSIONS: To our knowledge, this is the first study to comprehensively evaluate the factors influencing the use of systemic glucocorticoid treatment and cumulative dose in patients with JIA. Our findings emphasize disease subtypes, specific joint involvements, ANA positivity, and disease activities in this regard.
OBJECTIVE: To compare the incidence of herpes zoster (HZ) between patients with rheumatoid arthritis (RA) and matched controls from a health plan in Buenos Aires, and to assess incidence under different immunosuppressive...OBJECTIVE: To compare the incidence of herpes zoster (HZ) between patients with rheumatoid arthritis (RA) and matched controls from a health plan in Buenos Aires, and to assess incidence under different immunosuppressive treatments. METHODS: Patients with newly diagnosed RA between 2000 and 2023 were matched 1:2 by age and sex with controls. Incidence densities per 100 patient-years (PY) were calculated. Cox regression and Kaplan-Meier analyses were performed to identify factors associated with HZ. RESULTS: A total of 460 RA patients and 920 controls (81.3% women, mean age: 62.2 y) were included, with a median follow-up of 9 years. The RA cohort contributed 4184 PY and controls 9783 PY. Forty-six HZ events occurred in RA and 29 in controls. HZ incidence was 1.08 (95% CI: 0.80-1.47) per 100 PY in RA versus 0.29 (95% CI: 0.19-0.44) in controls [incidence rate ratio (IRR) 3.63; 95% CI: 2.28-5.77; p<0.001]. Compared with conventional DMARDs, biologic therapy increased risk (IRR: 2.48; p=0.023), and JAK inhibitors further elevated it (IRR: 4.47; p=0.001). In multivariate analysis, older age (HR: 1.04; p=0.005) and biologic use (HR: 2.39; p=0.006) were independent predictors. Most HZ cases involved one dermatome; 9.3% were disseminated, and 6.8% required hospitalization. Postherpetic neuralgia was more frequent in RA (42.2% vs. 7.7%; p=0.002). CONCLUSION: HZ incidence was over 3-fold higher in RA patients, particularly among those treated with biologics and JAK inhibitors.
OBJECTIVES: This study aimed to systematically summarize available clinical data on the use of chimeric antigen receptor (CAR) T-cell therapy in autoimmune diseases (ADs). METHODS: A systematic review was conducted using...OBJECTIVES: This study aimed to systematically summarize available clinical data on the use of chimeric antigen receptor (CAR) T-cell therapy in autoimmune diseases (ADs). METHODS: A systematic review was conducted using PubMed and other literature databases to identify publications on CAR-T therapy in ADs published since 2020. Eligible studies included single-case reports and case series from clinical trials, while studies involving duplicated patient populations were excluded in accordance with PRISMA guidelines. Extractable patient-level data were analyzed for key clinical outcomes. RESULTS: A total of 32 studies encompassing 124 patients with ADs who received CAR-T therapy were included. The primary diseases treated were systemic lupus erythematosus (SLE) and myasthenia gravis (MG), with smaller numbers of patients reported in other ADs. In SLE trials, 83% achieved clinical remission or low disease activity following CAR-T therapy, within the first few months following infusion. In MG trials, minimal symptom expression was achieved in 73%. CAR-T disappearance and B-cell reconstitution generally occurred within the first few months following infusion and were predominantly of a naïve phenotype. Mild cytokine release syndrome was the most common adverse event; serious adverse events were reported in 4.8% of patients without treatment-related deaths. CONCLUSION: Preliminary clinical evidence suggests that CAR-T therapy is associated with meaningful clinical response in selected ADs. Larger studies with standardized outcome measures and longer follow-up are needed to better define its safety and efficacy.
Immunoglobulin A vasculitis with renal involvement (IgAVN) is an immune complex-mediated glomerulonephritis characterized by mesangial deposition of IgA1-containing immune complexes. Although malignancy-associated vascul...Immunoglobulin A vasculitis with renal involvement (IgAVN) is an immune complex-mediated glomerulonephritis characterized by mesangial deposition of IgA1-containing immune complexes. Although malignancy-associated vasculitis is recognized, paraneoplastic IgAVN remains rare, and its clinical patterns and optimal management strategies are not well defined. We present an illustrative case of relapsing crescentic IgAVN temporally associated with metastatic meningioma and contextualize this presentation through a focused review of published cases from 2000 to 2025. A total of 21 adult cases of IgA vasculitis with renal involvement have been reported, predominantly affecting men. The reported malignancies included hematologic, gastrointestinal, pulmonary, renal, and genitourinary cancers. In most cases, vasculitis and malignancy were diagnosed concurrently or within a short temporal window, supporting a potential paraneoplastic relationship. Renal manifestations ranged from hematuria and proteinuria to rapidly progressive glomerulonephritis. The outcomes and prognosis varied for these patients: complete remission occurred in 43% of cases, partial remission in 24%, and progression in 24%. Our review suggests that combined malignancy-directed therapy and corticosteroids may be associated with more favorable renal outcomes compared with immunosuppression alone. Proposed mechanisms include tumor antigen-driven immune complex formation, dysregulated glycosylation of IgA1, and complement activation. However, mechanistic data remain limited. Recognition of this association is clinically important, as adult-onset, relapsing, or unusually aggressive IgAVN should prompt evaluation for underlying malignancy or cancer progression. Further study is needed to clarify pathophysiologic pathways and to guide evidence-based management in this rare but clinically significant entity.
J Clin Rheumatol
· 2026 Jun · PMID 42141848
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OBJECTIVE: Pegloticase, an infused pegylated uricase enzyme, rapidly lowers serum urate (SU) in patients with uncontrolled gout. Preinfusion intravenous glucocorticoids (GCs) are typically administered before pegloticase...OBJECTIVE: Pegloticase, an infused pegylated uricase enzyme, rapidly lowers serum urate (SU) in patients with uncontrolled gout. Preinfusion intravenous glucocorticoids (GCs) are typically administered before pegloticase to reduce risks of infusion reactions (IRs); however, they may exacerbate comorbidities in this patient population, and thus minimizing GC exposure is desirable. This study examined preinfusion GC dose reduction and elimination in patients with uncontrolled gout receiving pegloticase and methotrexate (MTX) co-therapy. METHODS: This retrospective case series of medical data (January 1, 2020, to April 1, 2023) from 1 rheumatology practice included patients receiving pegloticase (8 mg every 2 wk) with MTX co-therapy and undergoing preinfusion methylprednisolone dose reduction and elimination. The percentage of patients with SU <6 mg/dL (pegloticase responders) was assessed at 6 months and study completion; safety was assessed by frequency and severity of adverse events. RESULTS: Twelve patients with multiple comorbidities were evaluated (male, 92%; White, 58%; mean [SD] age, 60.3 [11.0] years; mean [SD] baseline SU, 8.8 [1.9] mg/dL). Patients received a mean (SD) of 14.3 (4.2) pegloticase infusions and discontinued GCs after a mean (SD) of 7.6 (1.3) infusions. At 24 weeks, 10/12 (83%) patients were responders and 9/12 (75%) continued to respond following GC reduction and elimination; 3/12 (25%) had an SU rise and discontinued pegloticase. The most common adverse event was gout flare (10/12 [83%] patients); no patients experienced IRs. CONCLUSIONS: This study demonstrated that preinfusion GC reduction and elimination are possible in patients with uncontrolled gout receiving pegloticase and MTX co-therapy.
OBJECTIVE: Area-level factors such as neighborhood poverty affect the health of individuals with rheumatic conditions. The connection of area-level data with health data via electronic health records (EHR) often relies u...OBJECTIVE: Area-level factors such as neighborhood poverty affect the health of individuals with rheumatic conditions. The connection of area-level data with health data via electronic health records (EHR) often relies upon the most recent patient address. We investigated longitudinal address history to understand mobility to areas with differing degrees of social vulnerability. METHODS: We identified individuals with rheumatic or musculoskeletal conditions receiving rheumatology care in a multihospital health care organization (2019-2024) in this cohort study. We geocoded available addresses for everyone over 5 years and merged them with the census tract-level Social Vulnerability Index. We used multinomial regression to investigate associations with patient characteristics and movement to areas with different SVIs. RESULTS: Among 10,376 individuals, 2563 (25%) moved ≥1 time. 504 (20%) moved to a census tract with higher vulnerability, and 420 (16%) moved to a lower one. Increased likelihood of moving to an area with higher vulnerability was associated with being Black (OR 3.43, 95% CI: 2.13-5.54) or a Medicaid beneficiary (OR 1.87, 95% CI: 1.34-2.61). Decreased likelihood of moving to a lower vulnerability area was associated with being Black (OR: 0.51, 95% CI: 0.31-0.83) or a Medicaid beneficiary (0.66, 95% CI: 0.45-0.98). CONCLUSIONS: Address histories from the EHR can provide a more nuanced understanding of area-level factors, especially for Black and lower-income individuals with rheumatic conditions. This information may help inform hospital-wide initiatives to identify patients who may be at higher risk for negative health outcomes based on their residential mobility.
Özçelik E, Uğur Es Y, Erdem Torun Ş
… +9 more, Öztürk D, Yoğun SN, Polat MC, Işiklar Ekici M, Çelikel E, Ekici Tekin Z, Ertem Ş, Karagöl C, Çelikel Acar B
OBJECTIVE: The objective of this study was to investigate differences between familial Mediterranean fever (FMF) patients with symptom onset at aged 2 years or younger and those with later onset, with a focus on clinical...OBJECTIVE: The objective of this study was to investigate differences between familial Mediterranean fever (FMF) patients with symptom onset at aged 2 years or younger and those with later onset, with a focus on clinical presentation, disease course, and treatment outcomes. MATERIALS AND METHODS: The medical records of FMF patients aged 0 to 18 years who were followed in the Pediatric Rheumatology Department between 2013 and 2024 were reviewed. Patients with symptom onset at aged 2 years or younger were classified as having early-onset, and patients with missing data or <6 months of follow-up were excluded. RESULTS: A total of 1255 patients with FMF were divided into 2 groups according to the age at symptom onset: ≤2 years (early-onset FMF, n = 346) and >2 years (later-onset FMF, n = 909). Early-onset FMF patients exhibited longer diagnostic delays, higher attack frequency, and more frequent fever attacks compared with later-onset patients (P < 0.05). Disease severity and the prevalence of colchicine-resistant FMF were higher in the early-onset group (P = 0.021 and P = 0.002). M694V homozygosity was more common among early-onset FMF patients (29.9% vs. 22.1%, P = 0.005). In multivariate analysis, a family history of colchicine-resistant FMF (OR = 2.64, 95% CI: 1.48-4.71) and fever (OR = 3.05, 95% CI: 2.12-4.40) were identified as independent predictors of early disease onset FMF. CONCLUSION: Early-onset FMF is associated with a more severe clinical presentation, including higher disease severity, increased colchicine resistance, and a greater frequency of M694V homozygosity. A family history of colchicine-resistant FMF is an independent predictor of early onset, highlighting the importance of detailed family history assessment in clinical practice.
OBJECTIVE: Inflammatory arthritis, especially rheumatoid arthritis (RA), imposes substantial morbidity that diminishes quality of life and escalates health care costs when left untreated. Timely diagnosis and treatment a...OBJECTIVE: Inflammatory arthritis, especially rheumatoid arthritis (RA), imposes substantial morbidity that diminishes quality of life and escalates health care costs when left untreated. Timely diagnosis and treatment are pivotal. However, limited access to rheumatologists underscores the importance of triaging referrals based on symptom severity. We developed a protocol for using infrared thermography (IRT) to detect joint inflammation and implemented it in an academic primary care clinic as a screening tool for rheumatology referrals. MATERIALS AND METHODS: We enrolled people with RA, osteoarthritis (OA), and controls to undergo joint power doppler ultrasound (PDUS) and IRT. IRT image analysis employed manual segmentation with specialized software to determine surface joint temperatures. IRT temperature cutoff points using PDUS as the gold standard for joint inflammation were established. Subsequently, we recruited people with hand or foot joint pain and fast-tracked to rheumatology those surpassing IRT cutoff points for joint inflammation. RESULTS: Thirty-two people with RA, 10 with OA, and 9 controls were enrolled. Most participants (86.3%) were females, and 37.3% were black. Temperature measurements showed robust interrater reliability. Cutoff points at metacarpophalangeal (MCP) (T center ≥32.93 °C, T mean ≥32.67 °C) and wrist (T center ≥33.76 °C, T mean ≥33.79 °C) joints discriminated between inflamed and noninflamed joints. Of the 20 pilot participants, N = 10 (50%) were referred to rheumatology using IRT findings, with N = 8 (80%) seen within 4 weeks. Diagnoses included osteoarthritis, gout, and calcium pyrophosphate deposition disease. CONCLUSION: IRT demonstrates potential as a reliable tool for identifying inflamed joints and offers a feasible pathway for identifying patients with joint-level thermal abnormalities who may benefit from expedited rheumatologic evaluation. Further research is needed to refine IRT-based rheumatology referral protocols and optimize their utilization.
BACKGROUND: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children. Although primarily affecting the joints, the prolonged inflammatory process characteristic of this chronic conditi...BACKGROUND: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children. Although primarily affecting the joints, the prolonged inflammatory process characteristic of this chronic condition may also contribute to early-onset atherosclerosis. This study aimed to evaluate early markers of atherosclerosis in patients with JIA and to investigate their association with disease activity. METHODS: This cross-sectional study was conducted between March and September 2025 and included patients under 16 years of age diagnosed with JIA. The healthy control group consisted of age-matched and sex-matched children. Disease activity was assessed using the Juvenile Arthritis Disease Activity Score (JADAS-27). Epicardial adipose tissue thickness (EATT) and carotid intima-media thickness (CIMT) were measured. RESULTS: A total of 48 children diagnosed with JIA (43.8% male, mean age 12.6±4.1 y) and 25 age-matched, BMI-matched, and sex-matched healthy controls (44.0% male, mean age 12.7±3.8 y) were included. Twenty-three (48%) of the patients had oligo-JIA, 11 (23%) had RF (-) poly-JIA, and 9 (19%) had enthesitis-related JIA. Patients with JIA showed significantly lower mitral E waves and higher right and left CIMT and eEATT than healthy controls ( p <0.01). EATT positively correlated with age, JADAS-27 score, and CIMT ( p <0.001). An EATT cutoff >3.15 mm predicted high disease activity (JADAS-27 >25) with 71.4% sensitivity and 60% specificity (AUC=0.77, p <0.001). Multiple regression analysis identified EATT >3.15 mm as an independent predictor of high disease activity (β=2.37, 95% CI: 0.52-4.22, p =0.01). CONCLUSIONS: This study demonstrated that both EATT and CIMT were elevated in JIA patients compared with healthy controls, and that increased EATT independently predicted higher disease activity. Clinicians managing JIA patients with high disease activity should be aware of the potential presence of atherosclerosis.
BACKGROUND: Dermatomyositis (DM) is an inflammatory myopathy frequently accompanied by pruritus, which can be severe, treatment-refractory, and associated with significant impairment in quality of life. Interleukin-31 (I...BACKGROUND: Dermatomyositis (DM) is an inflammatory myopathy frequently accompanied by pruritus, which can be severe, treatment-refractory, and associated with significant impairment in quality of life. Interleukin-31 (IL-31) has emerged as a key mediator of itch and has been implicated in the inflammatory pathways of dermatomyositis, suggesting a potential therapeutic role for IL-31 receptor blockade. METHODS: We conducted a retrospective case series of 5 patients with dermatomyositis who were treated with nemolizumab for moderate-to-severe pruritus at Mount Sinai Dermatology between June 2025 and November 2025. Clinical response was assessed using patient-reported itch severity measured by numeric rating scale (NRS) scores and the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI). Adverse events and short-term outcomes were recorded. Nemolizumab dosed according to approved atopic dermatitis/prurigo nodularis regimens (60 mg SC loading dose followed by 30 mg every 4 wk). RESULTS: All patients experienced improvement in pruritus following nemolizumab initiation, with a reduction in itch observed in all cases and anecdotal improvement reported as early as 2 days in select patients. Improvements in cutaneous disease activity, as measured by CDASI, were also observed. Nemolizumab was well tolerated, with no serious adverse events. CONCLUSIONS: In this case series, nemolizumab was associated with rapid improvement in pruritus and cutaneous disease activity in patients with dermatomyositis, including those with longstanding or treatment-refractory disease. These findings support further investigation of IL-31 blockade as a targeted antipruritic strategy and cutaneous disease improvement in dermatomyositis through larger, prospective studies.
do Rosário DC, Parente Costa Seguro L, Formiga FFC
… +5 more, Bertoglio IM, De Lucena Valim JM, Marreiros Nunes Filho D, Remião Ugolini Lopes M, Bonfa E
OBJECTIVE: To characterize adult hospitalizations for eosinophilic granulomatosis with polyangiitis (EGPA) in the United States and identify clinical and sociodemographic factors independently associated with in‑hospital...OBJECTIVE: To characterize adult hospitalizations for eosinophilic granulomatosis with polyangiitis (EGPA) in the United States and identify clinical and sociodemographic factors independently associated with in‑hospital mortality. METHODS: We conducted a retrospective analysis of the National Inpatient Sample Database (2016-2022) to identify adult EGPA hospitalizations. Demographics, comorbidities, complications, insurance type, income quartile, length of stay, and total charges were compared between survivors and nonsurvivors. Variables with p ≤ 0.2 in univariable analysis were entered into multivariable logistic regression to determine independent predictors of in‑hospital death. RESULTS: Among 12,900 EGPA hospitalizations, 355 patients died (2.75%). Nonsurvivors were older, had longer hospitalizations, and incurred higher total hospital charges. In multivariable analysis, cardiac disease (OR: 1.94; 95% CI: 1.157-3.237), central nervous system (CNS) involvement (OR: 2.91; 95% CI: 1.004-8.453), gastrointestinal (GI) disease (OR: 3.05; 95% CI: 1.159-8.042), infection (OR: 3.87; 95% CI: 2.123-7.046), interstitial lung disease (OR: 2.62; 95% CI: 1.227-5.598), and renal disease (OR: 5.20; 95% CI: 2.784-9.708) were independently associated with in‑hospital mortality. Demographic and socioeconomic variables, including sex, race/ethnicity, insurance type, and income quartile, were not independent predictors of in-hospital death. CONCLUSIONS: In this nationally representative cohort, in‑hospital mortality for EGPA was 2.75%. Renal, infectious, cardiac, CNS, GI, and pulmonary complications were strongly associated with death. These findings underscore the need for early recognition and aggressive management of organ‑threatening disease and infection in hospitalized EGPA patients.
BACKGROUND: This study aimed to evaluate physical activity levels of children with Familial Mediterranean fever (FMF), and to compare these levels with age- and sex-matched healthy peers. A secondary aim was to investiga...BACKGROUND: This study aimed to evaluate physical activity levels of children with Familial Mediterranean fever (FMF), and to compare these levels with age- and sex-matched healthy peers. A secondary aim was to investigate associations between physical activity, acute phase reactants (APRs), and disease activity scores. METHODS: This cross-sectional study enrolled FMF patients aged 9-14 years and matched healthy controls. Disease activity was assessed using Pras scores, Auto-Inflammatory Diseases Activity Index (AIDAI), and International Severity Scoring System for FMF (ISSF). Physical activity was evaluated weekly using the Physical Activity Questionnaire for Children (PAQ-C) over three months. Correlations of PAQ-C scores with APRs and disease activity parameters were analyzed at multiple time points, and repeated-measures analyses were performed. RESULTS: Overall, 140 participants were included (70 in control group and 70 in patient group). The study group had significantly lower PAQ-C scores than controls (2.75±0.71 vs. 3.37±0.54; p<0.001). Patients with exercise-related leg pain had significantly lower PAQ-C scores than those without pain (2.54±0.60 vs. 2.99±0.76; p=0.007). A significant negative correlation was found between age and PAQ-C scores in the study group (r=-0.377, p=0.001). PAQ-C scores were negatively correlated with ISSF scores (r=-0.278, p=0.020 and r=-0.288, p=0.016) and the AIDAI scores (r=-0.305, p=0.010 and r=-0.396, p<0.001) at first and the third months. CONCLUSION: This study demonstrates that children with FMF have reduced physical activity, particularly those with exercise-induced leg pain. The decline with age may reflect increased disease awareness, emphasizing the need for monitoring and promoting safe physical engagement in disease management.
OBJECTIVE: Biological treatments have transformed juvenile idiopathic arthritis (JIA) outcomes, yet their availability and cost remain challenging. We investigated the impact of 2 different health care systems on the JIA...OBJECTIVE: Biological treatments have transformed juvenile idiopathic arthritis (JIA) outcomes, yet their availability and cost remain challenging. We investigated the impact of 2 different health care systems on the JIA disease course and outcomes. MATERIALS AND METHODS: JIA patients presenting to 2 tertiary centers in Türkiye and India between January 2014 and January 2025, with ≥1 year of follow-up, were enrolled. Systemic JIA (sJIA) patients were analyzed separately due to differing management and prognosis. Data at 6 months, 1, 2, 3, and 5 years if follow-up occurred, were obtained retrospectively. RESULTS: A total of 207 patients were enrolled: 15 sJIA and 129 non-sJIA from Türkiye, and 12 sJIA and 51 non-sJIA from India. Biological disease-modifying antirheumatic drugs (bDMARDs) were prescribed significantly more often in the Turkish cohort (46% vs. 23%, p=0.001). In contrast, systemic corticosteroid use was significantly higher in the Indian cohort (26% vs. 69%, p=0.001). Initial remission was achieved more rapidly in the Turkish cohort (median month 2 vs. 4, p<0.001), and the number of patients with active joints and elevated acute-phase reactants was consistently higher in the Indian cohort throughout all follow-up periods. CONCLUSIONS: Universal health care coverage was associated with early initiation and sustained use of bDMARDs, leading to more rapid control of disease activity. In contrast, when bDMARDs were unavailable, physicians were forced to rely more heavily on systemic corticosteroids. Interestingly, JIA-related complications such as chronic joint involvement and uveitis did not significantly differ between the Turkish and Indian cohorts, possibly due to differences in ethnic and disease characteristics, survivor bias, and the limited number of patients with such complications.
A 36-year-old female patient, previously diagnosed with psoriatic arthritis and initially unresponsive to multiple treatments, experienced a subsequent asymptomatic period before developing new symptoms, including limite...A 36-year-old female patient, previously diagnosed with psoriatic arthritis and initially unresponsive to multiple treatments, experienced a subsequent asymptomatic period before developing new symptoms, including limited right-sided inflammatory lumbosacral pain and pustules on her palms and soles. Magnetic resonance imaging revealed changes consistent with osteitis. These findings prompted a reassessment of her condition, leading to a revised diagnosis of SAPHO syndrome.