{"title":"Musculoskeletal Manifestations of Light Chain Amyloidosis: A Systematic Review.","authors":"Maroua Slouma, Yesmine Khrifech, Emna Razgallah, Malek Dhifallah, Samar Zarati, Hela Ghdira, Mounir Lamloum","doi":"10.2174/0115733971405453260128101503","DOIUrl":"https://doi.org/10.2174/0115733971405453260128101503","url":null,"abstract":"<p><strong>Introduction: </strong>Diagnosing amyloid arthropathy remains challenging because its manifestations often mimic those of other inflammatory musculoskeletal diseases. This review aimed to describe the clinical, biological, and imaging characteristics of musculoskeletal involvement in light-chain (AL) Amyloidosis.</p><p><strong>Methods: </strong>We conducted a review in accordance with the PRISMA guidelines. We searched SCOPUS and MEDLINE for case reports and series published between January 2000 and December 2023 that detailed musculoskeletal manifestations of AL amyloidosis. To minimize the risk of omitting relevant articles, we also manually searched the reference lists of the articles identified in the initial search. Reports describing isolated cardiac amyloidosis or patients under active chemotherapy were excluded.</p><p><strong>Results: </strong>We identified 140 patients, all of whom presented with musculoskeletal manifestations as the initial manifestation of systemic AL amyloidosis. The mean age was 62.6 ± 11.6 years. Musculoskeletal findings included joint involvement in 33.6% of cases, muscular symptoms in 10.7%, carpal tunnel syndrome in 30%, and bone lesions in 12.1%. Among patients with joint disease, 42 (30%) had a rheumatoid arthritis-like presentation, and five (3.6%) exhibited features resembling giant cell arteritis. Diagnosis was confirmed by positive immunostaining for lambda light chains in all cases. Multiple myeloma was identified in 37.5% of patients. Of the 46 patients with available treatment data and a median follow-up of 11.5 months (range: 6-23 months), 25 (17.8%) achieved hematologic remission after chemotherapy, whereas 21 (15%) died during follow- up.</p><p><strong>Discussion: </strong>Our review offers a more comprehensive picture of the diverse musculoskeletal manifestations of AL amyloidosis. It showed that amyloid arthropathy can mimic common rheumatologic disorders, especially rheumatoid arthritis. Publication bias was identified as the main study limitation.</p><p><strong>Conclusions: </strong>Our findings suggest that the diagnosis of AL amyloidosis should be considered when joint involvement is associated with macroglossia, nephrotic syndrome, polyneuropathy, periorbital purpura, cutaneous lesions, or heart failure.</p>","PeriodicalId":11188,"journal":{"name":"Current rheumatology reviews","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149220035","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"First Genetics Association Study of the ACE I/D Polymorphism with Inflammatory Arthropathy Susceptibility in the Western Algerian Population: A Case-Control Study.","authors":"Asmaa Azzoune, Chahinez-Amira Dahmani, Leila Adda Neggaz, Amina Benouda, Abdallah Boudjema","doi":"10.2174/0115733971464383260928045651","DOIUrl":"https://doi.org/10.2174/0115733971464383260928045651","url":null,"abstract":"<p><strong>Introduction: </strong>Several genetic association studies have investigated Inflammatory Arthropathy (IA), a term used in this study to refer specifically to rheumatoid arthritis classified according to the 1987 American College of Rheumatology (ACR) criteria and the 2010 ACR/EULAR classification criteria. However, limited data are available regarding the role of the Angiotensin‑Converting Enzyme (ACE) Insertion/Deletion (I/D) polymorphism across different populations. The present study aimed to evaluate the association between the ACE I/D polymorphism and IA susceptibility in the Western Algerian population Materials and Methods: A total of 101 patients diagnosed with IA and 140 healthy unrelated controls were enrolled. Genotyping of the ACE I/D polymorphism (rs4340) was performed using multiplex Polymerase Chain Reaction (PCR). Genotype and allele frequencies were compared between groups, and Odds Ratios (ORs) with 95% Confidence Intervals (CIs) were calculated using SPSS software.</p><p><strong>Results: </strong>Our findings indicated a strong risk of IA associated with genotypic distributions of ACE I/D, DD ((OR) = 4(1,02-15.72), p-value = 0.04, as well as the dominant model (ID+DD) (OR) =16,01(1-29), p-value = 0.01, in the development of IA. However, no significant association was observed between ACE I/D genotypes and age in our study.</p><p><strong>Discussion: </strong>These findings suggest that the ACE I/D polymorphism, particularly the D allele, may contribute to IA susceptibility, possibly through inflammatory pathways involving the renin-angiotensin system.</p><p><strong>Conclusion: </strong>This is the first study in the Algerian population to demonstrate a potential association between the ACE I/D polymorphism and rheumatoid arthritis.</p>","PeriodicalId":11188,"journal":{"name":"Current rheumatology reviews","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149220102","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Pulmonary Embolism Revealing an Eosinophilic Granulomatosis with Polyangiitis: A Case Report.","authors":"Marwa Ben Brahim, Ichrak Bannour, Sondess Arfa, Imen Touil, Olfa Jomaa, Amel Barhoumi, Olfa Berriche","doi":"10.2174/0115733971524209260929114608","DOIUrl":"https://doi.org/10.2174/0115733971524209260929114608","url":null,"abstract":"<p><strong>Introduction: </strong>Venous thromboembolism presents a rare clinical manifestation of Eosinophilic Granulomatosis with polyangiitis.</p><p><strong>Case presentation: </strong>We present a case of pulmonary embolism associated with Eosinophilic Granulomatosis with Polyangiitis (EGPA) systemic manifestations in a 26-year-old Tunisian female patient. She presented with dyspnea and blood hypereosinophilia. Blood tests revealed elevated D-dimer levels. Lung scintigraphy (V/Q-scan) revealed pulmonary artery embolism in the right lung. The Computed Tomography (CT) image showed circumferential wall thickness of the lower mediastinal oesophagus and gastroesophageal junction. The chest scan also showed multiple pulmonary infiltrates with a ground-glass appearance localized in the apex of the posterior segment of the right upper lobe. The CT scan of the sinuses showed circumferential thickening of the maxillary sinus. Based on the 2022 ACR/EULAR diagnosis criteria, the patient was diagnosed with Eosinophilic Granulomatosis with Polyangiitis. After one month of anti-vitamin K treatment, a spirometry showed an obstructive pattern. The patient underwent methylprednisolone pulses, followed by oral corticosteroids, which were slowly tapered concomitantly with a cyclophosphamide regimen. A marked improvement of the spirometry parameters was achieved.</p><p><strong>Conclusions: </strong>Clinicians should be vigilant in the diagnostic and therapeutic management of Pulmonary Embolism (PE) during the active phase of EGPA to establish adequate follow-up procedures.</p>","PeriodicalId":11188,"journal":{"name":"Current rheumatology reviews","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149164178","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A3 Adenosine Receptor in Rheumatoid Arthritis: Friend or Foe? A Key Mediator of Different Signaling Pathways.","authors":"Yousef Vojgani, Amirhosein Ghafouri-Asbagh, Elham Farhadi, Ahmadreza Jamshidi, Masoomeh Akhlaghi, Mahdi Mahmoudi","doi":"10.2174/0115733971457285260930222236","DOIUrl":"https://doi.org/10.2174/0115733971457285260930222236","url":null,"abstract":"<p><strong>Introduction: </strong>Rheumatoid Arthritis (RA) is an autoimmune disease that has heterogeneous clinical manifestations. The A3 Adenosine Receptor (A3AR), a protein implicated in numerous inflammatory pathways, is overexpressed in patients with RA.</p><p><strong>Methods: </strong>The available preclinical and clinical literature about A3AR signaling in RA was reviewed, and published data on its molecular pathways, immunomodulatory functions, and role in synovial fibroblasts were searched. Studies exploring A3AR agonists and their mechanistic interactions with NF-κB and p53 pathways were also reviewed.</p><p><strong>Results: </strong>The mechanisms in which the A3AR is involved in alleviating inflammatory status resulting from alterations in transcriptional activity of the nuclear factor kappa B-light- chain-enhancer of activated inflammatory cells (NF-κB) in RA and the clinical and preclinical studies working on A3AR agonists alongside the apoptosis process by interacting with the p53 expression level. In contrast, we explained that the canonical and non-canonical pathways for A3AR, which may lead to worsening inflammation, were detailed.</p><p><strong>Discussion: </strong>The implication of A3AR in the worsening of destructive effects on RA synovium in cases of hypoxia, as well as the consequences of low oxygen levels on FLSs, was detailed.</p><p><strong>Conclusion: </strong>In conclusion, we hypothesized that the overexpressed A3AR could provide the activation of Ras, leading to the inflammatory and senescent phenotype of FLSs during the pathological processes in RA.</p>","PeriodicalId":11188,"journal":{"name":"Current rheumatology reviews","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149170664","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ghizlane Ghoullam, Imane El Binoune, Bouchra Amine, Ikram El Moubarik, Samira Rostom, Salma Zemrani, Rachid Bahiri
{"title":"Multimorbidity and Hospital-related Outcomes in Patients with Rheumatoid Arthritis: A Cross-sectional Study.","authors":"Ghizlane Ghoullam, Imane El Binoune, Bouchra Amine, Ikram El Moubarik, Samira Rostom, Salma Zemrani, Rachid Bahiri","doi":"10.2174/0115733971492149260914041246","DOIUrl":"https://doi.org/10.2174/0115733971492149260914041246","url":null,"abstract":"<p><strong>Introduction: </strong>Multimorbidity is frequent in rheumatoid arthritis (RA) and is associated with increased mortality, impaired quality of life, and complex clinical management. However, its association with hospitalization characteristics, particularly hospital length of stay and reasons for admission, remains insufficiently characterized.</p><p><strong>Methods: </strong>We conducted a five-month cross-sectional study (February to July 2024) in a tertiary university hospital, including adult patients diagnosed with rheumatoid arthritis according to the 2010 ACR/EULAR classification criteria. Multimorbidity was defined as the presence of at least two chronic comorbid conditions (CHCs), and patients were stratified into two categories: multimorbid (MMG) and non-multimorbid (N-MMG) groups. Data collected included demographic characteristics, clinical features, laboratory parameters, and imaging findings. Functional impairment was assessed using the Health Assessment Questionnaire (HAQ).</p><p><strong>Results: </strong>The study included 126 individuals with a mean age of 56.6 ± 13.4 years, of whom 84.9% were female. Multimorbidity was present in 52.4% of cases. Patients in the MMG were significantly older than those in the N-MMG (62.1 ± 9.9 vs 50.5 ± 14.2 years, p < 0.001). They also had significantly higher disease activity (DAS28, p < 0.001) and more impaired functional status as measured by HAQ (p < 0.001). Length of hospital stay was significantly longer in the multimorbid group. A hospital stay of 14 days or more was observed in 31% of MMG patients compared with 10.3% in the N-MMG cohort. Multivariate logistic regression analysis revealed that increasing age (OR = 1.11, 95% CI: 1.05-1.17, p < 0.001) and current corticosteroid use (OR = 4.73, 95% CI: 1.73-12.95, p = 0.002) were independently associated with multimorbidity. In addition, multimorbidity remained independently associated with prolonged hospitalization after adjustment for age, disease duration, disease activity, and corticosteroid use (adjusted OR = 3.42, 95% CI: 1.36-8.60; p = 0.009).</p><p><strong>Discussion: </strong>In this study, multimorbidity was associated with greater clinical complexity among hospitalized patients with RA and with less favorable hospital-related outcomes, particularly longer hospitalization duration. The higher disease activity and poorer functional status observed among multimorbid patients may partly explain the longer hospital stays observed.</p><p><strong>Conclusion: </strong>Our findings indicate that multimorbidity is associated with a more complex clinical profile in hospitalized patients with rheumatoid arthritis and with poorer hospital-related outcomes, particularly prolonged length of stay. These associations may partly reflect the higher disease activity and greater functional impairment observed among multimorbid patients.</p>","PeriodicalId":11188,"journal":{"name":"Current rheumatology reviews","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149105442","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Improving Vaccination Coverage among Rheumatic Disease Patients by Overcoming Existing Barriers.","authors":"Saurabh RamBihariLal Shrivastava, Prateek Sudhakar Bobhate","doi":"10.2174/0115733971486719260915120020","DOIUrl":"https://doi.org/10.2174/0115733971486719260915120020","url":null,"abstract":"<p><p>Rheumatic disease patients tend to have a high burden of infectious diseases, including opportunistic infections, compared with the general population. The extent of vaccination coverage, namely influenza and pneumococcal vaccination, among rheumatic disease patients varies across different populations and regions based on multiple factors. We have failed to capitalize on key opportunities, namely during diagnosis, before initiation of immunosuppressive therapy, during routine follow-up visits, and upon hospitalization, to improve vaccination coverage among rheumatic disease patients. Given the rising burden of rheumatic diseases, suboptimal vaccination rates, and existing barriers that influence vaccine uptake, there is an urgent need to improve vaccine coverage and minimize the resulting consequences. To conclude, vaccines play a crucial role in reducing the risk of infections among rheumatic disease patients, especially on immunosuppressive therapy. It is essential to identify the drivers of vaccine hesitancy among patients and then address them with the help of a multipronged and concerted approach to augment vaccine coverage.</p>","PeriodicalId":11188,"journal":{"name":"Current rheumatology reviews","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149037058","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A Randomized Controlled Trial: Efficacy of Wangbi Capsule Plus Conventional Therapy for Knee Osteoarthritis and Its Modulation of the Ferroptosis Pathway.","authors":"Xiaoqing Wang, Mei Wang, Zhenyi Zhao, Zhong Wen, Yanhui Li, Xiu Li, Quan-Bo Zhang, Yu-Feng Qing","doi":"10.2174/0115733971512249260902052806","DOIUrl":"https://doi.org/10.2174/0115733971512249260902052806","url":null,"abstract":"<p><strong>Introduction: </strong>Knee osteoarthritis (KOA) is a prevalent degenerative disease lacking structural disease-modifying agents. According to TCM theory, its progression involves internal liver-kidney deficiency and external qi/blood stasis with meridian obstruction. We evaluated Wangbi Capsule (WBC) plus diacerein/glucosamine vs. controls (n=180) over 12 weeks. Primary: WOMAC change; secondary: VAS, Lequesne, PhGA, HAQ. In an exploratory subgroup (n=75), PBMC mRNA for GPX4, SLC7A11, ACSL4, plus ESR, hs-CRP, and β-CTX were measured.</p><p><strong>Results: </strong>WBC yielded superior WOMAC reduction (17.77 vs. 22.81, P=0.025), higher excellent response rates, and better all secondary outcomes (P<0.05). hs-CRP fell more with WBC. KOA patients had lower GPX4 and higher ACSL4 vs. healthy controls. Post-treatment, WBC restored both GPX4 and ACSL4 to healthy levels, while controls only reduced ACSL4. In the WBC group, ΔGPX4 and ΔSLC7A11 inversely correlated with ΔESR, Δhs-CRP, and Δβ-CTX (all P<0.01). Safety was comparable.</p><p><strong>Discussion: </strong>These exploratory PBMC mRNA findings suggest WBC may restore systemic redox homeostasis and lipid metabolism via the GPX4/ACSL4 axis, potentially improving the joint's \"metabolic-inflammatory\" microenvironment. However, these are correlational, not causal, and are limited to mRNA data without protein or functional ferroptosis markers (e.g., MDA, glutathione), absence of joint tissue validation, an open-label design, and a small subgroup size. Furthermore, the lack of P53 changes hints at possible pathways independent of classical P53-mediated ferroptosis, but this remains speculative. Thus, all mechanistic inferences are hypothesis-generating.</p><p><strong>Conclusion: </strong>WBC plus conventional therapy improved symptoms safely over 12 weeks; its ferroptosis-related effects await verification in dedicated mechanistic studies.</p>","PeriodicalId":11188,"journal":{"name":"Current rheumatology reviews","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148967002","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Luis Javier Cajas Santana, José S Cortés, Julia Recalde, Sebastian Molina, Yamit Niquefa V, Maria Carolina Torres V
{"title":"Exploring the Autoimmune Spectrum of Combined Pulmonary Fibrosis and Emphysema: Inflammatory Myopathy Cases and a Systematic Review.","authors":"Luis Javier Cajas Santana, José S Cortés, Julia Recalde, Sebastian Molina, Yamit Niquefa V, Maria Carolina Torres V","doi":"10.2174/0115733971482602260903045754","DOIUrl":"https://doi.org/10.2174/0115733971482602260903045754","url":null,"abstract":"<p><strong>Introduction: </strong>Combined Pulmonary Fibrosis and Emphysema (CPFE) is a syndrome characterized by the coexistence of emphysema and pulmonary fibrosis on High-Resolution Computed Tomography (HRCT), typically presenting with preserved lung volumes and markedly reduced diffusing capacity, and may occur in association with connective tissue diseases. A systematic review of the literature was conducted to improve understanding of its clinical profile and present two unusual cases linked to idiopathic inflammatory myopathies.</p><p><strong>Methods: </strong>A systematic search of Medline, Embase, and Scopus was performed following PRISMA guidelines. Sixty articles were included, addressing diagnosis, pathophysiology, risk factors, prognosis, and treatment. A summary of reported cases was also compiled.</p><p><strong>Results: </strong>An international consensus statement established a definition for clinical and research purposes, requiring emphysema to involve≥15% of the lung parenchyma. Reported risk factors include older age, male sex, smoking, mediastinal lymphadenopathy, fibrosis extent, high-titer rheumatoid factor, or anti-Ro/SSA antibodies. Prognostic determinants of mortality or progression include the extent of fibrosis, decline in DLCO, and pulmonary hypertension. Paraseptal emphysema and usual interstitial pneumonia are the most common radiologic findings. Twenty-seven studies reported patients, mainly associated with systemic sclerosis (19.7% in smokers, 7.5% in never-smokers) and rheumatoid arthritis, while only two cases were linked to myopathies. Management relies on immunosuppressive therapy directed at the underlying disease, with early initiation potentially modifying disease course. Additional measures include inhalation therapy, antifibrotic agents, pulmonary hypertension treatment, and rehabilitation.</p><p><strong>Discussion: </strong>CPFE is an uncommon but clinically relevant complication with distinct risk and prognostic factors. Pulmonary hypertension is frequent. Cases associated with myopathies are rare. Immunosuppressive therapy remains central and may slow disease progression.</p><p><strong>Conclusion: </strong>CPFE is a distinct and clinically significant condition associated with increased morbidity and mortality, requiring accurate recognition and multidisciplinary management.</p>","PeriodicalId":11188,"journal":{"name":"Current rheumatology reviews","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148967000","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Association of ERAP1 and IL23R Gene Polymorphisms with Susceptibility and Clinical Manifestations of Spondyloarthritis in Tunisian Patients.","authors":"Kaouther Maatallah, Maissa Abbes, Imen Ayedi, Dorra Ben Nessib, Maryam Kallel-Sellami, Lobna Kharrat, Dorra ElHaj Mahmoud, Fatma Majdoub, Wajih Kaabachi, Dhia Kaffel, Ferjani Hanene, Lilia Laadhar, Wafa Hamdi","doi":"10.2174/0115733971395851251127070902","DOIUrl":"https://doi.org/10.2174/0115733971395851251127070902","url":null,"abstract":"<p><strong>Introduction: </strong>Among the non-HLA loci, ERAP1 and IL-23R gene polymorphisms have been implicated in spondyloarthritis (SpA). This study aimed to determine their association in a Tunisian cohort and to assess their influence on disease characteristics.</p><p><strong>Methods: </strong>We genotyped 100 SpA patients and 100 sex- and region-matched healthy controls using real-time PCR for ERAP1 (rs27044, rs30187) and IL-23R (rs7530511) SNPs. Allelic, genotypic, and haplotypic frequencies were compared. HLA-B27 status and data on clinical and paraclinical characteristics of the disease were collected.</p><p><strong>Results: </strong>The mean age was 42.8 ± 12.2 years, with a sex ratio M/F = 1.7. HLA-B27 was found in 55.6% of patients (OR = 25, 95% CI [9.5-69.2]; p < 0.001). None of the SNPs of ERAP1 or IL- 23R was associated with SpA susceptibility, even after analysis according to HLA-B27 status. Clinically, the rs7530511 G variant was associated with disease activity. In HLA-B27-positive patients, rs27044 C and rs30187 T alleles were significantly associated with less active forms of SpA. In multivariate analysis, the rs30187 T allele was independently associated with the risk of psoriasis. The rs30187 C allele and the C-C haplotype of ERAP1 were independent factors for quality-of-life impairment.</p><p><strong>Discussion: </strong>Although these SNPs showed no relationship with disease susceptibility, some were associated with disease severity and comorbidities and are therefore likely to contribute to clinical expression rather than disease predisposition.</p><p><strong>Conclusion: </strong>The studied polymorphisms of ERAP1 and IL-23R were not associated with SpA susceptibility in Tunisian subjects. HLA-B27 status did not appear to influence these relationships, but some SNPs may influence clinical manifestations.</p>","PeriodicalId":11188,"journal":{"name":"Current rheumatology reviews","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149016066","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sandra S Rini, Pande Kurniari, Gede Kambayana, Ni Made Dwita Pratiwi
{"title":"A Rare Atypical Cardiac-Arrest Onset of Fulminant Systemic Lupus Erythematosus with Multiorgan Failure, Myocarditis, and AMAN- Variant Guillain-Barré Syndrome Successfully Treated with Multimodal Immunotherapy.","authors":"Sandra S Rini, Pande Kurniari, Gede Kambayana, Ni Made Dwita Pratiwi","doi":"10.2174/0115733971487668260818115544","DOIUrl":"https://doi.org/10.2174/0115733971487668260818115544","url":null,"abstract":"<p><strong>Background: </strong>This report highlights a rare, catastrophic presentation of Systemic Lupus Erythematosus (SLE) manifesting as sudden cardiovascular collapse in a patient with no prior autoimmune history. The case is particularly novel due to the concurrent development of the Acute Motor Axonal Neuropathy (AMAN) variant of Guillain-Barré Syndrome (GBS) alongside multiorgan failure, mimicking septic and cardiogenic shock.</p><p><strong>Case presentation: </strong>A 49-year-old woman suffered an out-of-hospital cardiac arrest following acute gastroenteritis, characterized by recurrent ventricular fibrillation and tachycardia. She rapidly developed multiorgan failure, including severe myocardial dysfunction (EF 29%), acute kidney injury, metabolic acidosis, and altered consciousness. While the initial presentation suggested sepsis, subsequent testing revealed positive antinuclear antibodies, lupus anticoagulant, and hypocomplementemia, meeting ACR/EULAR criteria for SLE. Electrophysiological studies for persistent weakness confirmed AMAN-type GBS. Following an aggressive multimodal immunotherapy regimen of corticosteroid pulses, Intravenous Immunoglobulin (IVIG), and rituximab, the patient achieved significant recovery of cardiac, renal, and neurological functions.</p><p><strong>Conclusions: </strong>This case demonstrates that SLE can present as a life-threatening \"autoimmune storm\" mimicking sepsis or primary heart failure. Early identification is vital in atypical critical illness, as aggressive immunotherapy can reverse even severe multiorgan dysfunction. Clinicians should consider autoimmune screening in cases of unexplained cardiac arrest and multiorgan failure to prevent diagnostic delays and improve outcomes.</p>","PeriodicalId":11188,"journal":{"name":"Current rheumatology reviews","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148905399","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}