Rheumatology International最新文献

This study aims to investigate the clinical and pathological characteristics of patients who underwent minor salivary gland biopsy (mSGB) for suspected Sjögren disease (SjD). A total of one hundred five patients who underwent mSGB at a tertiary rheumatology center over a two-year period were included in the study. Demographic and disease characteristics of patients were recorded from electronic health records. The histopathological evaluation of the mSG was performed according to focus score (FS). The ESSDAI and ESSPRI scores were used for disease activity. The study data were analyzed by dividing patients into groups according to whether they met the SjD Classification Criteria and had focal lymphocytic sialadenitis (FLS). The median symptom duration of patients was 28.0 (8-102) months, and 68.5% (72; 105) of them met the SjD Classification Criteria before biopsy. 11.1% of SjD patients (8; 72) had also overlap syndrome. The number of patients with FS ≥ 1 (42, 58.33% vs. 11, 33.33%; p < 0.001), ESSDAI (4.21 ± 2.23 vs. 3.64 ± 2.12; p = 0.023), and ESSPRI-dryness scores (5.12 ± 1.51 vs. 4.72 ± 1.54; p = 0.018) were significantly higher in patients who met the SjD classification criteria than in patients who did not meet the criteria. According to histopathology findings, xerostomia (86.7% vs. 75.0%; p = 0.012), xerophthalmia (75.4% vs. 63.5%; p = 0.024), fatigue (54.7% vs. 42.3%; p = 0.035), ANA (81.1% vs. 69.2%; p < 0.001), SSA (35.8% vs. 26.9%; p = 0.041), and SSB (18.8% vs. 13.4%; p = 0.047) positivity rates were significantly higher in FLS positive (FS ≥ 1) group than the negative group (FS < 1). The unstimulated whole saliva flow [OR (95% CI) = 2.58 (0.94-7.02)] and Schirmer's test [OR (95% CI) = 2.89 (1.04-7.97)] were identified as predictors of FS. This study demonstrated that, whereas diagnostic biopsy findings are predominantly observed in seropositive patients and/or meeting the criteria, FLS positivity is relatively frequent in seronegative individuals who do not fulfill SjD classification criteria. The present study also represented that objective measurements of the sicca symptoms predict FLS.

This single-center study aimed to evaluate relationship between the platelets count (PLT) and the degree of synovial perfusion in patients with high disease activity of arthritis. Conducted at the Department of Early Arthritis Clinic in National Institute of Geriatrics, Rheumatology, and Rehabilitation in Warsaw, Poland. The study enrolled 60 patients diagnosed with rheumatoid arthritis (RA) and group of 20 patients with psoriasis arthritis (PsA). Different laboratory variables were assessed and particularly PLT, disease activity was assessed by DAS 28(ESR) and simplified disease activity index (SDAI). The degree of the synovial membrane vascularization was assessed using power doppler ultrasound (PDUS) and additionally vascularity index was measured by PDUS index (INDEX%) in the region of interest (ROI). Statistical analysis was performed using Prism 9.0 software package (GraphPad Software, Inc., USA). No significant correlation was observed between PLT and INDEX% synovial vascularity, which may indicate that PLT has no influence on microvascular changes in the synovium and its PDUS assessment in RA and PsA patients.

Systemic sclerosis (SSc) is a connective tissue disease characterized by immune dysregulation, fibrosis, and vasculopathy, frequently resulting in substantial impairment in health-related quality of life (HRQoL). Total serum immunoglobulin G (IgG), a central component of adaptive humoral immunity, may reflect cumulative disease burden, yet has not been previously studied in relation to patient-reported outcomes (PROs) in SSc. In this prospective cross-sectional study, 64 patients fulfilling classification criteria for SSc were evaluated. HRQoL was assessed using the Systemic Sclerosis Quality of Life Questionnaire (SScQoL), Health Assessment Questionnaire Disability Index (HAQ-DI), and visual analog scales (VAS) for pain and global patient - reported disease activity (PGA). Lower IgG levels were significantly associated with worse HRQoL across all instruments: SScQoL (Spearman's ρ = - 0.437, p = 0.0009), HAQ-DI (ρ = - 0.446, p = 0.0007), VAS pain (ρ = - 0.486, p = 0.0002), and PGA (ρ = - 0.584, p < 0.0001). These associations were consistent across major clinical subgroups, including those stratified by antibody status, interstitial lung disease, and skin involvement severity. In multivariable models adjusting for inflammation, fibrosis, and gastrointestinal symptoms, IgG remained an independent predictor of worse SScQoL (β = - 0.012, p = 0.003). Patients with gastrointestinal involvement had significantly lower IgG levels (p = 0.025). No differences in IgG or HRQoL were observed by immunosuppressive treatment status. These findings suggest that total serum IgG is inversely associated with patient-reported HRQoL in systemic sclerosis and may serve as a clinically relevant, treatment-independent biomarker of perceived disease burden.

Idiopathic inflammatory myopathies (IIM) are rare autoimmune disorders primarily affecting skeletal muscles, with occasional involvement of other organs and an increased risk of malignancy. Dermatomyositis (DM), a major IIM subtype, is characterized by proximal muscle weakness, distinctive skin manifestations, and characteristic biopsy findings. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV), including microscopic polyangiitis (MPA), involve small- to medium-sized vessels and can affect the kidneys and lungs. Although uncommon, overlap syndromes between IIM and AAV have been described. We report two cases of DM-MPA overlap. The first involved a 67-year-old male with interstitial lung disease (ILD), muscle weakness, and proteinuria. The second concerned a 53-year-old female presenting with characteristic skin rash, arthralgias, proteinuria, and mild pulmonary involvement. Both patients received high-dose corticosteroids and cyclophosphamide (CYC), resulting in clinical and laboratory improvement, including resolution of proteinuria and improved pulmonary function, maintained over a 6-month follow-up. The literature review identified five relevant case reports and one case series. Fifteen patients from these six studies were included, predominantly female (13/15), with ages ranging from 15 to 84 years. DM was the most common IIM subtype (7/15), followed by polymyositis (PM) (6/15), inclusion body myositis (IBM) (1/15), and clinically amyopathic dermatomyositis (CADM) (1/15). MPA accounted for 87% (13/15) of AAV cases, while GPA was reported in 2/15. Renal involvement was frequent, often presented as pauci-immune crescentic glomerulonephritis, whereas pulmonary involvement was less common. MPO-ANCA was positive in 12/15 patients. Initial therapy typically involved corticosteroids, with or without immunosuppressants, tailored according to the dominant organ involvement. Outcomes were variable; most patients achieved remission, although some experienced persistent organ dysfunction. IIM-AAV overlap is rare but potentially severe, frequently involving renal, pulmonary, muscular, and occasionally cardiac systems. Early recognition and individualized immunosuppressive therapy can yield favorable outcomes. Multicenter studies are required to clarify the epidemiology, clinical spectrum, and optimal management of this complex syndrome, and further research is needed to elucidate underlying pathogenic mechanisms.

Antineutrophil cytoplasmic autoantibody-associated vasculitis (AAV) is the most commonly encountered primary small-vessel vasculitis, with an annual incidence of 20 per million. The data on AAV from the Southeast Asian ethnicity is scarce. The present longitudinal observational study was undertaken to determine the clinical profile and outcomes in fifty-eight consecutive patients of AAV attending the Department of Rheumatology in a tertiary care centre in India. The majority (39, 67.2%) were females with a mean (± SD) age at baseline of 39.2 (± 15.2) years. Granulomatosis with polyangiitis (GPA) was the most common phenotype, accounting for 40 cases (69.0%). The patients were followed up for a mean (± SD) duration of 35.4 (± 31.4) months. The most commonly affected organ domains were the respiratory (46, 79.3%), musculoskeletal (41, 70.7%), and the renal domains (38, 65.5%). In our cohort, a significantly higher number of patients exhibited ophthalmological involvement (31, 53.4%). There were a total of 22 relapse events among 11 patients, representing a relapse rate of 19%. The case-fatality rate was 10.6%. The majority received treatment with intravenous methylprednisolone (46; 79.3%) and intravenous cyclophosphamide (42; 72.4%) during the induction phase, while rituximab (28; 48.3%) was the preferred first-line maintenance agent. BVAS of 22.5 was related to VDI ≥ 5 (p = 0.000). The mean (± SD) Vasculitis Damage Index (VDI) accrued was 2.41 (± 1.97). The most common treatment-related damage included osteoporosis (40%), diabetes (40%), and cataract (13%), whereas the common disease-related damage included diastolic hypertension (58%) and impaired lung function (27%). Higher Birmingham Vasculitis Activity Score (BVAS) at presentation (OR: 1.25, 95% CI 1.031-1.522) and relapse of scleritis (OR: 35.27, 95% CI 1.152 -1000.000) were found to be independent predictors of high VDI ≥ 5. This is the first study from Eastern India that has looked into the clinical profile of AAV and its damage index.

Management of ANCA-associated vasculitis (AAV) has significantly improved, yet up to 40% of patients experience relapses, leading to worse long-term outcomes and organ damage. This multicenter cohort study investigated the prognostic value of histopathological patterns in renal AAV for predicting relapse risk and treatment response. We retrospectively analyzed 264 patients with newly diagnosed granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) with renal involvement recruited at four tertiary rheumatology and/or nephrology centers. Baseline clinical data, disease activity scores, and kidney biopsy findings were assessed. Patients were followed for at least twelve months. An overall relapse was defined as renewed disease activity requiring changes in maintenance therapy, initiation of a new induction regimen, or an increase in prednisone dosage > 10 mg/day. A severe relapse required new immunosuppressive induction therapy. After a median follow-up of twelve months the renal domain of the Birmingham Vasculitis Activity Score (BVAS) and its subcategories were evaluated. AAV patients with moderate-to-high (> 25%) interstitial fibrosis and tubular atrophy (IFTA) had a lower risk for both, overall and severe relapses. Severe relapses occurred more frequently in older and male patients. Furthermore, we identified glomerular sclerosis (≥ 50%) to be the strongest predictor for ongoing activity in the renal BVAS domain. Histopathological features do not only help to predict renal recovery and need for dialysis but also to forecast relapse risk and renal BVAS activity. Incorporating these patterns into clinical decision-making could enable more personalized therapy approaches, highlighting the need for prospective validation.

Growing evidence indicates that antiphospholipid antibodies (aPL) and antiphospholipid syndrome (APS) are not only associated with arterial thrombosis, but also enhanced premature atherosclerosis and stenotic lesions in various vascular beds. Atherosclerotic vascular disease involving coronary, carotid, and peripheral arteries is accelerated in APS to a larger extent when systemic lupus erythematosus (SLE) or other autoimmune disorders co-exist. However, the presence of aPL by itself may enhance atherosclerosis and increase the risk of arterial thromboembolic events also in older patients who did not meet the APS classification criteria. Traditional cardiovascular risk factors in particular hypertension and hypercholesterolemia largely contribute to the development and progression of cardiovascular disease and the occurrence of its thrombotic manifestations also in patients with aPL, therefore they should be vigorously treated like in patients free of autoimmune disorders. Nevertheless, antiplatelet agents alone and in combination with vitamin K antagonists (VKAs) remain a mainstay in prevention of arterial thrombosis in APS, despite controversy around the impact of typical atherosclerotic vascular disease and its risk factors on therapeutic strategies in the presence of IgG and/or IgM aPL at significant titers. The present overview summarizes clinical evidence for the role of aPL in subclinical and clinically overt atherosclerotic vascular disease and its management.

This study aimed to investigate the effects of neuropathic pain and fibromyalgia syndrome (FMS) on the quality of life of patients with multiple sclerosis (MS), as assessed by functional status, disability, pain intensity, mood, and sleep quality. A total of 190 MS patients (137 females, 53 males; mean age: 45.2 ± 11.5 years; range: 20-72 years) were included in this cross-sectional study. Pain, functional status and disability of the patients were evaluated using the following tools: visual analog scale (VAS), Fibromyalgia Impact Questionnaire (FIQ), Douleur Neuropathique 4 Questions (DN4), Kurtzke Expanded Disability Status Scale (EDSS), Beck Depression Inventory (BDI), Pittsburgh Sleep Quality Index (PSQI) and Health Assessment Quality (HAQ) questionnaires. Neuropathic pain and FMS were present in 34.7% and 32.6% of the patients, respectively. The presence of both conditions was strongly associated with significantly worse scores across all tested parameters (VAS, BDI, FIQ, PSQI, EDSS, and HAQ) (p < 0.05). On most of the tools tested, male patients scored better than female patients (p < 0.05). Neuropathic pain and FMS are very common conditions in MS patients, and they appear to be associated with impaired quality of life, impaired functional status, and disability.

Early diagnosis of Systemic Sclerosis (SSc) is challenging due to nonspecific symptoms and clinical overlap with other autoimmune diseases. To investigate factors associated with diagnostic delays in SSc, focusing on the number and specialties of physicians consulted before diagnosis. This cross-sectional study included SSc patients registered at a university rheumatology clinic. Demographics, clinical features, and physical examination findings were recorded. The number and specialties of physicians consulted before diagnosis were obtained through structured face-to-face interviews. Of 240 screened patients, 135 were included (120 female, 88.8%; 55 with diffuse SSc, 40.7%; mean age 52.1 ± 11.5 years). The median time to diagnosis was 36 months (IQR 12-96) from Raynaud's phenomenon (RP) onset and 11(IQR 0-35) months from the first non-RP symptom. Patients with limited SSc had a significantly longer RP-to-diagnosis interval than those with diffuse SSc (44 vs. 20 months, p = 0.024). The median number of physicians consulted before diagnosis was 3 (range 1-9). Higher educational level (IRR = 0.97, p = 0.036) and referral by a familiar healthcare professional (IRR = 0.78, p = 0.035) were independently associated with fewer pre-diagnostic consultations. Among 119 patients who recalled their first physician, 42 (35.3%) initially consulted an internist, 28 (23.5%) a family physician, 11 (9.2%) a dermatologist, and 10 (8.4%) a cardiovascular surgeon; the remainder visited other specialists. Only 8 patients (6.7%) received an SSc diagnosis at their first consultation. SSc diagnosis is often delayed, requiring multiple consultations. Greater physician awareness and timely rheumatology referrals are essential.

Inflammatory rheumatic diseases (IRDs) are chronic autoimmune conditions that affect a variety of organs and systems. These disorders have a major impact on hand function, restricting independence in daily activities and lowering quality of life. They are characterized by significant reductions in the hand's fine motor skills, grip strength, and coordination capacities. These impairments in hand function have a detrimental influence on physical capability, psychological adjustment, and occupational productivity. Since IRDs are chronic and progressive, early detection, continuous follow-up, and multifaceted rehabilitation strategies are essential. Self-report measures and performance-based assessments are utilized to evaluate hand function. Furthermore, imaging techniques, including ultrasonography, magnetic resonance imaging, and dual-energy computed tomography, provide an objective evaluation of subclinical inflammation and structural damage. Rehabilitation approaches, as a complement to medical care, play an important role in maintaining and enhancing hand function. Multicomponent therapies, including exercise, splinting, occupational therapy, manual therapy, massage, and patient education, are beneficial for preserving functional gains. Future research is anticipated to concentrate on discovering biomarkers for early diagnosis, creating sensor-based digital evaluation tools and telerehabilitation programs, and developing individualized, AI-powered rehabilitation procedures. These developments will make a substantial contribution to preserving hand function and enhancing the quality of life for IRD patients.