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.
INTRODUCTION/OBJECTIVES: Axial spondyloarthritis (axSpA) is a heterogeneous disease with variable clinical features changing over the disease course, making diagnosis and monitoring challenging. Large progress has been m...INTRODUCTION/OBJECTIVES: Axial spondyloarthritis (axSpA) is a heterogeneous disease with variable clinical features changing over the disease course, making diagnosis and monitoring challenging. Large progress has been made in classification development, imaging, disease-activity assessment. However, there is limited insight in how these developments are addressed in daily clinical practice. Therefore, our study aim was to gain better insight into diagnosis, monitoring and treatment and to explore if non-radiographic (nr-)axSpA is addressed differently from radiographic (r-)axSpA. METHODS: A multiple-choice survey on diagnosis, monitoring, and treatment of axSpA was conducted in semi-structured, face-to-face interviews with 51 Dutch rheumatologists, representing a range of geographical locations, hospital types, and axSpA expertise. RESULTS: Of all participating rheumatologists, 78% worked in secondary referral centers, reflecting Dutch rheumatology practice. 52% felt insufficiently skilled to independently assess MRI sacroiliitis. Diagnostic uncertainty was higher for nr-axSpA (23%) than r-axSpA (10%). ASAS classification criteria were used to diagnose nr- and r-axSpA by 54% and 36% of rheumatologists. For monitoring disease activity, ASDAS was always or never used by 28% and 26% of rheumatologists. In treatment decisions concerning biological DMARDs, the level of pain was considered equally important as disease activity scores (ASDAS, BASDAI). Most rheumatologists (60%) did not use ASDAS or BASDAI cut-off scores or change to evaluate biological DMARDs' effectiveness. CONCLUSIONS: This study reveals that Dutch rheumatologists experienced difficulties in diagnosing axSpA in one out of four patients. ASAS classification criteria were often used for support, particularly in diagnosing nr-axSpA. The use of disease-activity assessments during monitoring was limited. Key Points •Diagnostic uncertainty remains common in daily practice, particularly for non-radiographic axial spondyloarthritis. •Although ASAS classification criteria were developed for research purposes, they are also used to support diagnosis. •ASDAS is used inconsistently to monitor disease activity and many rheumatologists seem to base treatment decisions on reported pain level. •There seems to be a gap between axial spondyloarthritis international management recommendations and routine rheumatology care.
OBJECTIVES: This study investigated serum pentosidine levels as an advanced glycation end product (AGE)-related marker of bone matrix deterioration and examined the association between b/tsDMARD use and prevalent vertebr...OBJECTIVES: This study investigated serum pentosidine levels as an advanced glycation end product (AGE)-related marker of bone matrix deterioration and examined the association between b/tsDMARD use and prevalent vertebral fractures in patients with RA in clinical remission. METHODS: Seventy-six patients with RA in clinical remission (DAS28-CRP < 2.3) were included. Serum pentosidine, bone turnover markers, and bone mineral density (BMD) were assessed. Lateral thoracolumbar spine radiographs were available for 51 patients, and prevalent vertebral fractures were evaluated using artificial-intelligence-assisted morphometry, with final physician confirmation. Multivariable regression analyses evaluated factors associated with serum pentosidine levels and prevalent vertebral fractures. RESULTS: Patients receiving b/tsDMARDs had lower serum pentosidine levels (P = 0.004) despite comparable BMD and bone turnover marker profiles. In multivariable linear regression, DAS28-ESR was associated with serum pentosidine levels (β = 0.00581, P = 0.011), while b/tsDMARD use showed a non-significant trend toward lower serum pentosidine levels (β = - 0.00544, P = 0.091). Vertebral fractures were numerically less common in patients receiving b/tsDMARDs (2/19 [10.5%] vs 11/32 [34.4%]). In logistic regression, older age was associated with higher odds of prevalent vertebral fractures (odds ratio 1.211 per year, 95% CI 1.074-1.439; P < 0.001), while b/tsDMARD use was associated with lower odds (odds ratio 0.144, 95% CI 0.015-0.841; P = 0.030). CONCLUSIONS: In patients with RA in clinical remission, b/tsDMARD use was associated with lower odds of prevalent vertebral fractures. Residual inflammation, reflected by DAS28-ESR and serum pentosidine levels, may be relevant to skeletal fragility beyond BMD. Key Points • Lower serum pentosidine levels were observed in b/tsDMARD-treated patients, whereas DAS28-ESR was independently associated with serum pentosidine levels in multivariable analysis. • In patients with rheumatoid arthritis in clinical remission, b/tsDMARD use was associated with lower odds of prevalent vertebral fractures. • Residual inflammation and bone matrix deterioration may be related to skeletal fragility beyond bone mineral density.
INTRODUCTION: To investigate the association between retinal microcirculation and renal function in childhood-onset systemic lupus erythematosus (cSLE) patients with lupus nephritis (LN). METHODS: This cross-sectional st...INTRODUCTION: To investigate the association between retinal microcirculation and renal function in childhood-onset systemic lupus erythematosus (cSLE) patients with lupus nephritis (LN). METHODS: This cross-sectional study involved 22 cSLE patients with LN (group 1), 24 cSLE patients without LN (group 2), and 23 normal controls (group 3). All participants underwent slit-lamp examination, best-corrected visual acuity testing, intraocular pressure measurement, fundus photography, and optical coherence tomography. Vessel density (VD) of the superficial capillary plexus (SCP), intermediate capillary plexus (ICP), deep capillary plexus (DCP), and choriocapillaris (CC), as well as the foveal avascular zone (FAZ) area, were measured from 4.5 × 4.5 mm optical coherence tomography angiography images. Laboratory parameters of renal function were collected from the patients. RESULTS: There were no statistical differences in the thickness of the whole, inner, and outer layers of the fovea among the three groups. The VD of the SCP decreased progressively from group 3 to group 2 to group 1. The VD of the ICP differed significantly only between group 1 and group 3 (P = 0.004). Compared with group 3, the VD of the CC in groups 1 and 2 decreased (P = 0.030 and P = 0.005, respectively), while the FAZ area increased (P = 0.013 and P = 0.005, respectively). The VD of the DCP did not differ significantly among the three groups. A positive correlation was found between creatinine and the VD of the SCP (r = 0.338, P = 0.021). The VD of the SCP showed diagnostic accuracy in discriminating between cSLE patients with and without LN (AUC = 0.706, P = 0.017). CONCLUSIONS: In cSLE patients with nephritis, the VD of the SCP decreases further. Although this decline does not parallel the severity of nephritis, it may help identify cSLE patients with nephritis. Key Points • Retinal blood flow of cSLE patients is affected even before visual acuity is impaired or lupus retinopathy develops. • When cSLE patients are complicated with LN, the VD of the SCP is further reduced.
INTRODUCTION: Familial Mediterranean fever (FMF) is an autoinflammatory condition caused by a single-gene mutation, inherited in an autosomal recessive pattern, and characterized by recurrent episodes of self-limited inf...INTRODUCTION: Familial Mediterranean fever (FMF) is an autoinflammatory condition caused by a single-gene mutation, inherited in an autosomal recessive pattern, and characterized by recurrent episodes of self-limited inflammation. Studies suggest that vitamin D levels may be lower in FMF patients, but evidence remains unclear. This study aims to assess the relationship between FMF and serum vitamin D levels. METHOD: We followed PRISMA guidelines and registered the protocol in PROSPERO (CRD420261299635). We searched PubMed, Scopus, and Web of Science up to December 2025 for observational studies comparing FMF patients with healthy controls. Our main outcomes were serum 25-hydroxyvitamin D, parathyroid hormone (PTH), and C-reactive protein (CRP). We combined the data as mean differences using a random-effects model. RESULT: Ten studies (563 FMF patients) were included. The pooled analysis showed a significant drop in vitamin D levels in FMF patients compared to healthy controls. However, there was high heterogeneity between studies (I = 89%), with a wide 95% prediction interval (- 2.7 to 0.46). During the summer, we found no difference in vitamin D levels, though Egyptian patients had lower levels than Turkish patients. There were no significant differences in PTH or CRP. CONCLUSION: Seasonal and regional factors play a significant role in determining vitamin D levels among FMF patients, with lower levels observed in winter and in Egyptian patients. Because of the high heterogeneity and low certainty, larger studies are needed to confirm the findings. Key Points • The first meta-analysis to compare vitamin D level between FMF patients and healthy individuals, findings show severe deficit in vitamin D level. • Many factors contribute to this decrease in vitamin D, starting from seasonal and geographical distribution and autoinflammatory state of FMF, making the fact of low vitamin D among FMF patients is highly individualized according to these prescribed factors. • Despite vitamin D deficit, there is no significant difference in PTH or CRP level.
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
OBJECTIVES: To evaluate the efficacy of rituximab therapy in patients with refractory neuropsychiatric systemic lupus erythematosus (NPSLE). METHODS: Seventeen patients with refractory NPSLE who received rituximab therap...OBJECTIVES: To evaluate the efficacy of rituximab therapy in patients with refractory neuropsychiatric systemic lupus erythematosus (NPSLE). METHODS: Seventeen patients with refractory NPSLE who received rituximab therapy at Ruijin Hospital between July 2016 and August 2025 were enrolled. Clinical manifestations and laboratory parameters were collected before and after treatment. Treatment response was evaluated using predefined manifestation-based clinical criteria, and paired longitudinal changes in laboratory parameters were analyzed using the Wilcoxon matched-pairs signed-rank test where data were available. RESULTS: Sixteen of 17 patients (94.1%) achieved significant clinical improvement, while one patient showed partial improvement with reduced seizure frequency. During a follow-up period of 3 to 102 months (median 45 months), none of the surviving patients experienced relapse of neuropsychiatric manifestations. Daily prednisone dose was reduced to a median of 5 mg/day at last follow-up. Rituximab-based treatment was associated with marked depletion of circulating CD19⁺ B cells, reductions in anti-dsDNA antibody titers and SLEDAI-2000 scores, and improvement in complement levels. Two patients died from COVID-19 pneumonia during follow-up, and one patient developed severe Pneumocystis jirovecii and cytomegalovirus pneumonia. CONCLUSION: Rituximab was associated with favorable outcomes in refractory NPSLE and may represent an alternative therapeutic option, particularly for patients who are refractory to conventional treatment with glucocorticoids and/or immunosuppressive agents. Key Points • Rituximab-based treatment was associated with clinical improvement in refractory NPSLE. • Sustained relapse-free remission was observed during long-term follow-up after rituximab therapy in patients with refractory NPSLE. • Rituximab was generally well tolerated, though infection risk requires careful monitoring.
OBJECTIVES: Rheumatoid arthritis (RA) imposes a substantial global health burden, contributing significantly to chronic disability and escalating healthcare costs. Accurate, long-term prevalence forecasts are essential t...OBJECTIVES: Rheumatoid arthritis (RA) imposes a substantial global health burden, contributing significantly to chronic disability and escalating healthcare costs. Accurate, long-term prevalence forecasts are essential to inform strategic planning for rheumatology services and health resource allocation. METHODS: Hence, we developed and calibrated a sex-specific illness-death model (IDM) at global, regional, and national scales using GBD data (1990-2021). To uncover underlying epidemiological patterns, we augmented our projections with a frontier analysis that evaluates the efficiency of RA burden relative to sociodemographic index (SDI). RESULTS: We project the global age-standardized prevalence rate (ASPR) will rise by 6.3% to 222.06 per 100,000 (95% CI, 220.49-223.6) by 2040. While females bear a higher absolute prevalence, the growth rate is projected to be faster in males (8.75% vs. 5.67%). Significant disparities exist at regional and national levels: Andean Latin America and Peru are anticipated to have the highest ASPRs, while Oceania and Indonesia are projected to have the lowest. An increasing trend is projected globally, across 18 GBD regions, and in 167 nations. Critically, frontier analysis reveals that higher sociodemographic development frequently coincides with a larger "RA efficiency gap," indicating economic progress alone is insufficient for disease control. CONCLUSION: Therefore, mitigating the projected rise in RA requires strategies that move beyond relying on general economic advancement. Future health policies must actively integrate targeted prevention of modifiable lifestyle risks and bridge healthcare access gaps to effectively curb the coming global burden. Key Points • Projects a 6.3% global increase in RA prevalence by 2040, with faster growth in males. • Identifies an "RA efficiency gap" where higher development correlates with higher burden. • Forecasts severe geographic disparities, with Andean Latin America having the highest burden. • Concludes that targeted prevention, not just economic progress, is needed to curb future burden.
Best Pract Res Clin Rheumatol
· 2026 Mar · PMID 42115015
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Immune checkpoint inhibitors (ICI) have transformed the field of oncology and can induce a durable cancer treatment response in select cancer patients. ICI binding to these checkpoints allows the immune system to be acti...Immune checkpoint inhibitors (ICI) have transformed the field of oncology and can induce a durable cancer treatment response in select cancer patients. ICI binding to these checkpoints allows the immune system to be activated in order to target tumor death, but this activation also brings multiple off-target side effects called immune related adverse events (irAE's) including rheumatic irAE's. Review aims will explore novel insights into rheumatic irAE's etiology and etiopathology including ICI-inflammatory arthritis, ICI-Polymyalgia Rheumatica, ICI-activated osteoarthritis, sicca-like syndrome, and ICI induced myositis. This chapter will review rheumatic irAE's and define their proposed mechanisms, which include generalized immune activation owing to checkpoint neutralization, direct off-target effects of checkpoint inhibitors and epitope spreading. Research using cellular profiling methods such as single cell transcriptomics and T cell profiling have helped explore the novel mechanism of these heterogenous rheumatic irAE's. Future goals of research include gaining a better understanding of pathogenesis of rheumatic irAE's to help target precision rheumatic irAE treatment.
Best Pract Res Clin Rheumatol
· 2026 Mar · PMID 42115014
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Rheumatology as a specialty is moving past cytokine inhibitors which have dominated the therapeutic landscape since the turn of the century, with novel cell-targeting therapies emerging to treat immune-mediated multisyst...Rheumatology as a specialty is moving past cytokine inhibitors which have dominated the therapeutic landscape since the turn of the century, with novel cell-targeting therapies emerging to treat immune-mediated multisystem inflammatory diseases such as systemic lupus, systemic sclerosis, small vessel vasculitides, and inflammatory myositis. Like many inflammatory arthritides, axial spondyloarthritis (axSpA) therapeutics has been limited to either inhibitors of TNF and IL-17, or blocking intracellular signaling of cytokines through Janus Kinase inhibitors. This systematic review chronicles the clinical progress made in the field of selective T cell depleting agents, the potential for novel cellular targeting therapies using Chimeric Antigen Receptor - T (CAR-T) therapy, as well the challenges posed by this approach for treatment of axSpA. A review of the MEDLINE, OVID and clinicaltrials.gov databases was performed which revealed no ongoing or completed open label or completed registered clinical trials. However recent data have highlighted potential for selective CD8 T cell targets with proof of concept using a monoclonal antibody against TRBV9+ CD8 T cells from a published case study and one randomized placebo-controlled trial, as well as other emerging therapeutics that are shifting focus from cytokine-based therapeutics to T cell targeting agents. In this review, we identified emerging therapeutic platforms of cellular immunotherapy, potential therapeutic targets, and discuss potential adverse effects related to cell-targeting therapies. While at present there is a dearth of clinical data on cellular targeting treatment options in axSpA, ongoing investigations into the pathogenesis of axSpA and identification of new molecular targets may help facilitate opportunities to develop CAR-T and other cell-based therapies for treatment of axSpA.
Kuzma I, Mathias KR, Lovell J
… +1 more, Gilotra NA
Best Pract Res Clin Rheumatol
· 2026 May · PMID 42115013
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Cardiac sarcoidosis (CS) results from the formation of non-necrotizing granulomas infiltrating the myocardium, resulting in a wide variety of clinical presentations including fatal arrhythmias and cardiomyopathy. Correct...Cardiac sarcoidosis (CS) results from the formation of non-necrotizing granulomas infiltrating the myocardium, resulting in a wide variety of clinical presentations including fatal arrhythmias and cardiomyopathy. Correctly identifying CS from its many mimics, including several rheumatologic conditions, is crucial for treating patients and preventing significant morbidity and mortality. In this review, we discuss diagnostic strategies for CS, methods to differentiate it from clinically similar cardiac pathologies with a focus on other rheumatologic conditions involving the heart, and approaches to CS management and treatment.
OBJECTIVE: Although pulmonary hypertension (PH) and interstitial lung disease (ILD) are major contributors to mortality in patients with connective tissue disease (CTD), data on concomitant PH specifically in those with...OBJECTIVE: Although pulmonary hypertension (PH) and interstitial lung disease (ILD) are major contributors to mortality in patients with connective tissue disease (CTD), data on concomitant PH specifically in those with CTD-associated ILD (CTD-ILD) remain limited. We aimed to identify predictors for PH development in CTD-ILD patients, and assess their impact on survival in this population. METHOD: This retrospective observational study included 224 patients with CTD-ILD, confirmed through multidisciplinary discussion, including patients with inflammatory myopathy-associated ILD (IIM-ILD, n = 88), systemic sclerosis-associated ILD (SSc-ILD, n = 57), rheumatoid arthritis-associated ILD (RA-ILD, n = 55), and primary Sjögren's syndrome-associated ILD (pSS-ILD, n = 24). PH was diagnosed using right heart catheterization (RHC) or, in patients unable to undergo RHC, by transthoracic echocardiography. Autoantibodies related to IIM and SSc were assessed using Euroimmun immunoblot assays. RESULTS: Among CTD-ILD patients, 28 (12.5%) had concomitant PH: SSc-ILD (21.1%), IIM-ILD (13.6%), RA-ILD (5.5%), and pSS-ILD (4.2%). Multivariate regression analysis revealed CTD duration, exertional dyspnea, nonspecific interstitial pneumonitis in HRCT, ANA-speckled pattern, anti-Ro52/SSA positivity, and anti-fibrotic agents were significant predictors of concomitant PH in overall CTD-ILD patients (all p < 0.05), and only anti-Ro52/SSA positivity is still a significant predictor in SSc-ILD/IIM-ILD subtypes. Anti-Ro52/SSA antibody positivity could predict PH emergence with AUC 0.64, specificity 75.4%, accuracy 72.3%, and negative predictive value 91.3% in CTD-ILD patients. Furthermore, CTD-ILD patients with PH and anti-Ro52/SSA positivity had higher mortality rates and shorter survival than those without PH or these autoantibodies. CONCLUSION: Anti-Ro52/SSA antibody positivity may serve as a promising predictor of PH development and poorer survival in patients with CTD-ILD. Key Points • Pulmonary hypertension (PH) was identified in 12.5% of patients with CTD-ILD, with the highest prevalence in systemic sclerosis-ILD and inflammatory myopathy-ILD. • Anti-Ro52/SSA antibody positivity was independently associated with concomitant PH in CTD-ILD and remained significant in the SSc-ILD/IIM-ILD subgroup. • PH and anti-Ro52/SSA positivity were each associated with worse survival in CTD-ILD, supporting a potential role for anti-Ro52/SSA in risk stratification.
OBJECTIVES: Connective tissue disease-associated interstitial lung disease (CTD-ILD) exhibits heterogeneous clinical outcomes, and traditional ILD-GAP score provides limited prognostic precision. We hypothesized that int...OBJECTIVES: Connective tissue disease-associated interstitial lung disease (CTD-ILD) exhibits heterogeneous clinical outcomes, and traditional ILD-GAP score provides limited prognostic precision. We hypothesized that integration of imaging-derived fibrosis measures would improve prognostic discrimination compared with physiology-based models. We aimed to develop and externally validate integrated prognostic systems for risk stratification in CTD-ILD. METHOD: In this multicenter retrospective study, patients with CTD-ILD confirmed by multidisciplinary evaluation were enrolled from two tertiary centers. Baseline demographic, functional, laboratory, and imaging data were collected at diagnosis. Three prognostic systems were evaluated: System A (ILD-GAP and fibrosis score independently), system B (a composite model integrating GAP and fibrosis scores), and system C (machine learning models using clinical and imaging variables). The primary endpoint was a composite adverse outcome during follow-up. Model discrimination was assessed using the area under the receiver operating characteristic curve (AUC) with internal and external validation. RESULTS: In external validation, the fibrosis score demonstrated stronger discrimination than the ILD-GAP index (AUC 0.798 vs. 0.768). The composite model showed modest improvement (AUC 0.821). Machine learning models achieved the highest discrimination, with random forest and support vector machine yielding AUCs of 0.833 (95% CI 0.741-0.927) and 0.826 (95% CI 0.735-0.910), respectively. SHAP analysis identified fibrosis extent, DLCO, and age as key contributors to prediction. CONCLUSIONS: In CTD-ILD, imaging-informed and data-driven models improve prognostic discrimination compared with traditional physiology-based indices. The fibrosis score plays a central role in outcome prediction, while composite and machine learning approaches provide incremental refinement for individualized risk stratification. Key Points • Imaging-derived fibrosis score demonstrated superior prognostic discrimination compared with the traditional ILD-GAP index in CTD-ILD, highlighting the central role of structural lung damage in outcome prediction. • Integration of clinical, functional, and HRCT-derived variables through optimized composite modeling significantly improved risk stratification, with machine learning approaches (random forest and support vector machine) achieving the highest predictive performance. • SHAP analysis identified fibrosis extent, DLCO, and age as the dominant contributors to adverse outcomes, underscoring the complementary value of imaging and physiology in individualized risk assessment.
Concurrent anti-neutrophil cytoplasmic antibody (ANCA)-associated nephritis and immunoglobulin G4 (IgG4)-related nephritis is a rare clinical entity with incompletely understood pathogenesis and progression. We describe...Concurrent anti-neutrophil cytoplasmic antibody (ANCA)-associated nephritis and immunoglobulin G4 (IgG4)-related nephritis is a rare clinical entity with incompletely understood pathogenesis and progression. We describe the case of a 56-year-old woman, without significant past medical history, who presented with a recurrent low-grade fever for over 3 months, occasional non-productive cough, and acute renal insufficiency, but without chills. Empirical anti-infective therapy was ineffective. Laboratory evaluation revealed moderate anemia and microscopic hematuria without proteinuria on urinalysis. Immunological testing was positive for anti-myeloperoxidase (MPO) antibodies, supporting a preliminary diagnosis of ANCA-associated vasculitis with renal involvement. Renal biopsy demonstrated crescentic glomerulonephritis coexisting with tubulointerstitial nephritis (TIN), with predominant infiltration by IgG4-producing plasma cells, despite a normal serum IgG4 concentration. Following confirmation of no therapeutic contraindications, the patient received corticosteroids in combination with rituximab. At a 2-week follow-up, renal function had improved, erythrocyte sedimentation rate and C-reactive protein were normalized, and urinalysis was negative for occult blood and protein, indicating effective disease control. Cases with concurrent ANCA-associated vasculitis and IgG4-related renal disease are exceedingly uncommon. Their clinical manifestations are often atypical, and routine laboratory assessments are insufficient for differentiation, typically requiring integrated histopathological analysis for definitive diagnosis. Further clinical observations and laboratory studies are needed to clarify the pathogenesis, improve diagnostic accuracy, and establish optimal treatment strategies for this complex condition.
BACKGROUND AND OBJECTIVE: Sinonasal symptoms occur in up to 80% of patients with granulomatosis with polyangiitis (GPA), affecting quality of life. This study aimed to evaluate the association between self-perceived sino...BACKGROUND AND OBJECTIVE: Sinonasal symptoms occur in up to 80% of patients with granulomatosis with polyangiitis (GPA), affecting quality of life. This study aimed to evaluate the association between self-perceived sinonasal symptoms (SNOT-22 questionnaire), and clinical variables, and to describe discrepancies between self-reported symptoms, ENT findings, and radiographic abnormalities. METHODS: A cross-sectional study was conducted at a tertiary center in Mexico City (November 2024-July 2025). Patients with GPA and a paranasal sinus CT scan within two years were included. Data collection comprised demographic, clinical, laboratory, and treatment variables. Assessments included disease activity (BVAS/GPA), cumulative damage (VDI), patient and physician global assessments (PhGA and PtGA), and PROMs (SNOT-22, AAV-PRO). All patients underwent ENT examination with nasal endoscopy. RESULTS: Fifty patients were included, 64% women, median age 49.5 years, and disease duration 88.5 months. Most were in remission (median BVAS/GPA 0). The median VDI was 3, PtGA 10.7mm, and PhGA 1.8mm. The median SNOT-22 score was 30.5. Common ENT findings were rhinorrhea (64%), hypertrophic turbinates or hyperemic mucosa (36%), nasal crusts (34%), and septal perforation (20%). CT abnormalities included chronic sinusitis (46%) and sinus destruction (32%). Self-reported symptoms showed moderate-to-strong correlations with AAV-PRO domains and global assessments, but no associations with disease activity, damage, or treatment. Discrepancies were observed between SNOT-22 items and ENT or CT findings. CONCLUSIONS: Self-reported sinonasal symptoms correlated with PROMs, whereas associations with disease activity or damage were not evident in this cohort. Discrepancies highlight the need for GPA-specific studies integrating PROMs, standardized ENT assessments, and imaging.
Izaguirre Germain MP, Villamizar Pérez S, Josianne Paris N
… +8 more, Serna Góngora MB, Brener A, Claros J, Gonzalez E, Pérez Cepas BC, Laura Micelli M, Sequeira G, Mario Kerzberg E
INTRODUCTION: Breastfeeding in systemic lupus erythematosus (SLE) is often influenced by concerns about medication safety, with limited data on patients' perceptions and decision-making. OBJECTIVES: To assess breastfeedi...INTRODUCTION: Breastfeeding in systemic lupus erythematosus (SLE) is often influenced by concerns about medication safety, with limited data on patients' perceptions and decision-making. OBJECTIVES: To assess breastfeeding knowledge, intentions, and practices among women with SLE, focusing on disease activity and medication-related perceptions, particularly regarding hydroxychloroquine and prednisone. METHODS: A cross-sectional survey was conducted with women diagnosed with SLE. Participants answered questions on demographics, previous pregnancy and breastfeeding experiences, knowledge of breastfeeding with SLE, and perceptions of breastfeeding safety with hydroxychloroquine and prednisone. The survey also assessed their intention to breastfeed in future pregnancies, especially during active disease. RESULTS: The survey was completed by 85 patients, with a mean age of 35 years (SD 9). Of these, 56% reported at least one pregnancy, 53% had breastfed, and 32.9% had a child after being diagnosed with SLE (SLE&Child group). When considering future pregnancies, 88% intended to breastfeed, but 62% prioritized treatment over breastfeeding if SLE was active (p<0.001). After reading patient information leaflets (PILs), 64% considered breastfeeding on prednisone, while only 26% thought it safe with hydroxychloroquine. Breastfeeding knowledge was higher in the SLE&Child group. Initiation rates were 79.3% in the SLE&Child group and 93.2% in controls (p=0.08). The average breastfeeding duration was 14 months in the SLE&Child group and 17 months in controls (log-rank test=0.5). CONCLUSIONS: Most patients with SLE intended to breastfeed, but this intention decreased during active disease. Hydroxychloroquine PILs appeared to discourage breastfeeding despite the drug's safety.