Systemic sclerosis (SSc) is a heterogeneous and debilitating multi-system autoimmune disease characterized by widespread microvascular vasculopathy and progressive multi-organ fibrosis. While localized cohorts have been described, there is a critical need for a standardized, national-scale multicenter validation to fully capture the epidemiological and phenotypic characteristics of SSc in Egypt. This study aimed to delineate the nationwide clinical, serological, and functional landscape of systemic sclerosis across Egypt utilizing the standardized EUSTAR registry framework across major geographic referral centers. A multi-center, retrospective registry-based review was conducted on 354 Egyptian SSc patients enrolled within the international EUSTAR database. Longitudinal and cross-sectional data were extracted across standardized registry parameters, including detailed demographics, clinical subsets (LeRoy criteria), comprehensive organ system involvement (evaluated via high-resolution computed tomography [HRCT], echocardiography, and nailfold capillaroscopy [NFC]), autoantibody profiles, and patient-reported quality-of-life indices (HAQ-DI and L-PF questionnaires). The cohort demonstrated a prominent female predominance (89.3
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease affecting young women. Budd–Chiari syndrome (BCS), caused by hepatic venous outflow obstruction, is a rare manifestation often associated with secondary antiphospholipid syndrome (APS). A 17-year-old girl presented with progressive abdominal distension, pedal edema, fever, weight loss, arthralgia, oral ulcers, alopecia, and amenorrhea. Initially managed as chronic liver disease after recurrent presumed spontaneous bacterial peritonitis, imaging revealed chronic BCS. Further evaluation demonstrated positive antinuclear and anti-double-stranded DNA antibodies, hypocomplementemia, proteinuria, and Class IV lupus nephritis. Persistent antiphospholipid antibody positivity confirmed secondary APS. Treatment with corticosteroids, mycophenolate mofetil, hydroxychloroquine, and planned indefinite anticoagulation resulted in marked clinical improvement. SLE with secondary APS should be considered in young women presenting with unexplained BCS to enable early diagnosis, prompt immunosuppressive therapy, and prevention of irreversible organ damage.
Abstract Background Psoriatic arthritis (PsA) is a chronic immune mediated inflammatory disease that may extend beyond the musculoskeletal and dermatological systems. Although cochlear and vestibular involvement has been reported in autoimmune rheumatic diseases, the frequency, pattern, and clinical correlates of audiovestibular dysfunction in PsA remain insufficiently characterized. Previous studies have generally focused on limited audiological or vestibular parameters. This study aimed to comprehensively evaluate cochlear and vestibular function in PsA patients using a multimodal audiovestibular test battery and to investigate the relationship between these findings and disease activity indices. Methods A total of 35 PsA patients and 35 healthy controls underwent pure tone audiometry, word recognition score (WRS), speech reception threshold (SRT), tympanometry, acoustic reflexes, video head impulse test (vHIT), cervical vestibular evoked myogenic potentials (cVEMP), videonystagmography (VNG), and bithermal caloric testing. Disease activity in PsA patients was quantified using the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Psoriasis Area and Severity Index (PASI), 28 - joint Disease Activity Score (DAS28), and Disease Activity Index for Psoriatic Arthritis (DAPSA28). Results PsA patients had higher hearing thresholds ( p ≤ 0.001), lower WRS ( p ≤ 0.001), and higher (poorer) SRT values ( p = 0.011) than controls. cVEMP P1–N1 peak-to-peak amplitudes were lower bilaterally (right: p = 0.002; left: p = 0.034). P1 latencies and right N1 latency were shorter in the PsA group, whereas left N1 latency was retained as a descriptive variable because of its markedly skewed distribution. vHIT showed lower VOR gains in the right lateral, bilateral anterior, and right posterior canals; left lateral vHIT gain was retained descriptively because of marked skewness. Abnormal caloric unilateral weakness was present in 34.3% of PsA patients and in no controls ( p ≤ 0.001). DAS28 was negatively correlated with right lateral vHIT gain (Spearman’s ρ = −0.36, p = 0.027), and DAPSA28 was negatively correlated with the participant-level bilateral mean cVEMP P1–N1 amplitude (Spearman’s ρ = −0.42, p = 0.018). Disease duration was positively correlated with right and left caloric SPV responses (Spearman’s ρ = 0.41 and ρ = 0.47, respectively; both p ≤ 0.01). PASI was negatively correlated with caloric canal paresis (Spearman’s ρ = −0.41, p = 0.014). Conclusion PsA was associated with subclinical auditory and vestibular abnormalities across complementary tests. Relationships with disease activity were exploratory and require confirmation in longitudinal studies.
Abstract Background Takayasu arteritis is a rare, chronic large-vessel vasculitis that primarily affects young women, particularly the aorta and its branches. It is traditionally regarded as an isolated vascular disease, and its coexistence with other systemic autoimmune diseases is uncommon, having been described mainly in case reports and small series. These reports, together with shared genetic and immunological features, have nevertheless prompted interest in whether TAK can be considered within the concept of polyautoimmunity. Objective This narrative review explores the relationship between Takayasu arteritis and polyautoimmunity, highlighting current evidence on shared immune mechanisms, disease clustering, and cardiovascular manifestations, and considers whether these findings support an autoimmune spectrum. Methods A comprehensive literature search was conducted in PubMed, Google Scholar, and the Cochrane Library for studies published since 2002. MeSH terms and keywords related to Takayasu arteritis (TAK), polyautoimmunity, and cardiovascular manifestations were used. Evidence from international registries, meta-analyses, and retrospective cohorts was reviewed. Studies were selected based on relevance to shared immunopathogenic mechanisms, the 2022 ACR/EULAR classification criteria, and Numano angiographic types. The literature was synthesized to evaluate the impact of coexisting autoimmune diseases on cardiovascular outcomes and treatment response in TAK. Results Takayasu arteritis (TAK) has been reported to coexist with other autoimmune diseases, particularly spondyloarthritis-spectrum disorders, and, less frequently and largely in case reports, systemic lupus erythematosus, rheumatoid arthritis, Behçet disease, and inflammatory bowel disease. These reported associations are supported by shared genetic susceptibility, especially HLA-B52, and common immune pathways involving the Th1/Th17 axis, IL-6, and TNF-α. Persistent activation of these pathways contributes to chronic systemic inflammation, which may worsen vascular damage and promote cardiovascular complications such as aortic regurgitation, renovascular hypertension, coronary ostial stenosis, and cerebrovascular ischemic events. Limited observational data suggest that coexisting autoimmune disease may be linked to a more treatment-resistant course, with a higher need for biologic agents such as tocilizumab or TNF inhibitors. Conclusion Although coexistence with other autoimmune diseases is uncommon and the supporting evidence is limited, considering Takayasu arteritis (TAK) within the spectrum of polyautoimmunity may help clinicians recognize shared immune mechanisms. Evaluation for coexisting autoimmune conditions at diagnosis and during follow-up, guided by clinical suspicion, may facilitate earlier recognition, guide individualized immunomodulatory treatment, and, where appropriate, enable a single therapeutic agent to address overlapping diseases. A multidisciplinary approach may further improve long-term outcomes by reducing the risk of severe cardiovascular and other end-organ complications.
Abstract Background Rheumatoid arthritis (RA) is a chronic autoimmune disease characterized by persistent joint inflammation, often resulting in significant functional impairment and reduced health-related quality of life (HRQoL). Biologic and targeted synthetic disease-modifying antirheumatic drugs (b/tsDMARDs) have substantially transformed RA management. Objectives To evaluate the impact of b/tsDMARD therapy on disease activity and HRQoL in a cohort of Egyptian RA patients unresponsive to conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) and to explore factors influencing baseline HRQoL. Methods This ambispective (prospective and retrospective) cohort study included 78 Egyptian RA patients. Patients met the 2010 ACR/EULAR RA classification criteria and received b/tsDMARDs after conventional treatment failure. Disease activity was assessed using the Clinical Disease Activity Index (CDAI), while HRQoL was evaluated using the Arabic version of the RAND short form (SF)-36 health survey at baseline and six months post-treatment. Clinical, laboratory, and sociodemographic data were also analyzed. Results CDAI scores significantly decreased from 27 ± 12 to 6 ± 3 ( p < 0.001), with marked reductions in erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels ( p < 0.001). Significant improvements were observed across all SF-36 domains, with physical and mental component scores increasing from 20 to 81 and 27 to 89, respectively ( p < 0.001). The median time from disease onset to b/tsDMARD initiation was 6.5 years. Longer treatment delays were associated with poorer HRQoL at six months, including physical functioning, vitality, body pain, general health, and physical and mental component scores. Baseline HRQoL was influenced by employment status and comorbidities, while older age was associated with poorer physical functioning. Higher baseline disease activity was associated with poorer HRQoL. Conclusion Treatment with b/ts DMARD therapy significantly improved disease activity and HRQoL in a cohort of Egyptian RA patients. However, longer treatment delay was associated with poorer outcomes.
