Advances in Rheumatology最新文献

{"title":"Rituximab combined with intravenous immunoglobulin in autoimmune diseases: a systematic review.","authors":"Jozélio Freire de Carvalho, Thelma Laroca Skare","doi":"10.1186/s42358-025-00450-x","DOIUrl":"https://doi.org/10.1186/s42358-025-00450-x","url":null,"abstract":"<p><strong>Background: </strong>Although using Rituximab (RTX) and intravenous immunoglobulin (IVIg) alone or sequentially is a well-established treatment for several autoimmune diseases, the combination of these two forms of therapy is still rare, and its use is poorly studied.</p><p><strong>Aim: </strong>To perform a systematic review on the use of RTX associated with IVIG in autoimmune conditions.</p><p><strong>Methods: </strong>PubMed/MEDLINE, EMBASE, and Scielo databases were screened for articles on RTX plus IVIg in autoimmune diseases until May 2024.</p><p><strong>Results: </strong>The review encompassed 21 studies evaluating RTX and IVIg for autoimmune diseases. Ten studies focused on pemphigus, involving 85 patients with diverse subtypes (47 pemphigus vulgaris, 27 pemphigoids, and 11 other variants). Most were case reports or series, with one retrospective study including controls. Positive outcomes were reported across all but one case of paraneoplastic pemphigus. Infections, such as P. jirovecii pneumonia, were noted in three studies, highlighting a potential risk. The other 11 studies involved 24 patients with conditions like polyneuropathies, lupus with CNS involvement, and neuromyelitis optica. While most reported favorable outcomes, one trial on IVIg-dependent polyneuropathies found RTX ineffective in reducing IVIg needs. Adverse events included pneumonia, venous thrombosis with pulmonary embolism, and infusion reactions, demonstrating the need for careful monitoring.</p><p><strong>Conclusion: </strong>RTX plus IVIg seems to be an alternative option for the treatment of refractory autoimmune diseases. However, more studies with a larger number of participants and in different autoimmune diseases are desired.</p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"65 1","pages":"19"},"PeriodicalIF":2.0,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143711875","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Brazilian recommendations for the management of tuberculosis infection in immune-mediated inflammatory diseases.","authors":"Viviane Angelina de Souza, Ana Luiza Mendes Amorim Caparroz, Virginia Fernandes Moça Trevisani, Anna Carolina Faria Moreira Gomes Tavares, Ana Karla Guedes de Melo, Anete Trajman, Ana Cristina de Medeiros-Ribeiro, Marcelo de Medeiros Pinheiro, Ricardo Machado Xavier, Odirlei Andre Monticielo, Maria Fernanda Brandão de Resende Guimarães, Flavio Sztajnbok, Sidney Bombarda, Liliana Andrade Chebli, Adriana Maria Kakehasi, Ana Luiza Bierrenbach, Ana Paula Monteiro Gomides Reis, Blanca Elena Rios Gomes Bica, Claudia Diniz Lopes Marques, Cristina Flores, Denise Silva Rodrigues, Eduardo Dos Santos Paiva, Eliana Dias Matos, Fernanda Dockhorn Costa Johansen, Helio Arthur Bacha, Joana Starling de Carvalho, José Roberto Provenza, Ketty Lysie Libardi Lira Machado, Licia Maria Henrique da Mota, Lilian David de Azevedo Valadares, Marco Antônio Araújo da Rocha Loures, Margareth Maria Pretti Dalcolmo, Maria Cecilia de Carvalho Bortoletto, Max Igor Banks Ferreira Lopes, Rejane Maria Rodrigues de Abreu Vieira, Ricardo Romiti, Rogerio Saad-Hossne, Rozana Mesquita Ciconelli, Valderilio Feijó Azevedo, Valéria Maria Augusto, Vitor Alves Cruz, Gecilmara Cristina Salviato Pileggi","doi":"10.1186/s42358-025-00449-4","DOIUrl":"https://doi.org/10.1186/s42358-025-00449-4","url":null,"abstract":"<p><strong>Background: </strong>The risk of tuberculosis infection (TBI) and its progression to tuberculosis disease (TBD) among persons with immune-mediated inflammatory diseases (IMID) results from a complex interplay of patient and disease characteristics, immunosuppression level, and the epidemiological context. Brazilian recommendations are unclear about TBI screening and its preventive treatment (TPT) in persons with IMID.</p><p><strong>Objective: </strong>To provide a comprehensive and evidence-based guideline for managing TBI in persons with IMID in Brazil.</p><p><strong>Methods: </strong>This task force was constituded by 42 specialists with interest in IMID and TBD. A core leadership team (CLT) drafted fourteen clinical questions on the risk of tuberculosis and indications of TPT among persons with IMID who started, or are about to start immunosuppressive drugs. The CLT supervised the systematic reviews and formulated the recommendations. The experts voted using the Delphi Method.</p><p><strong>Results: </strong>Nine recommendations were established. More than 80% of panelists voted \"agree\" and \"strongly agree\" with all statements. In brief, all persons with IMID starting or about to start immunosuppressive treatment should undergo tuberculin skin testing (TST) or interferon-gamma release assays (IGRAs), a chest imaging test and investigation of contact with active pulmonary or laryngeal TBD. TPT is mandatory for those with any positive result after excluding TBD. Exceptions include individuals with a history of TBD or a past positive TBI infection test. IGRA is preferred only in persons BCG-vaccinated in the past 2 years. Those with inconclusive IGRA results can have the test repeated once, and TPT should be offered if it remains indeterminate. TST or IGRA should be repeated yearly, for three years, when the previous test was negative, when starting or changing to a different class of immunosuppressive drug. Overall, the included studies had a low quality of evidence and high risk of bias.</p><p><strong>Conclusions: </strong>These guidelines are meant to improve the management of TBI in IMID. Health professionals must consider the epidemiological risk, host features, the social scenario, the characteristics of the disease, the access to health resources, and the development of an individualized plan for every patient.</p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"65 1","pages":"18"},"PeriodicalIF":2.0,"publicationDate":"2025-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143671542","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Suppression of skin lesions and SLE nephritis by increasing Treg in MRL/FAS^lpr mice by administration of bee venom Apitoxin^®.","authors":"Duk-Yeon Cho, Young-Mo Kang, SangHo Seol","doi":"10.1186/s42358-025-00448-5","DOIUrl":"https://doi.org/10.1186/s42358-025-00448-5","url":null,"abstract":"<p><strong>Background: </strong>Apitoxin^®, a drug based on bee venom was approved and released in Korea in 2003 as the ethical drug (ETC). It is well-known for its pain-relieving properties due to its potent anti-inflammatory effects. This raises the question of whether bee venom has benefits for other inflammatory disorders. Since its effectiveness in treating inflammation and pain associated with autoimmune diseases has been observed in several clinical cases in Korea, we conducted an efficacy study using an animal model of the systemic lupus erythematosus (SLE), an autoimmune disease with high medical unmet needs. In this research, we aim to confirm the potential therapeutic efficacy for SLE through the immunomodulation induced by bee venom.</p><p><strong>Methods: </strong>MRL/FAS^lpr mice were injected subcutaneously with Apitoxin^® and evaluated for clinical parameters including proteinuria, skin lesions, and lymphadenopathy, flow cytometric evaluation of regulatory T cells (Treg), quantitative evaluation of anti-dsDNA antibody in serum by ELISA, and histomorphometric analysis of kidney tissues.</p><p><strong>Results: </strong>Treatment with Apitoxin^® revealed a reduction in proteinuria, skin lesions, and lymphadenopathy in MRL/FAS^lpr mice. The percentage of CD3⁺CD4⁺CD25⁺FoxP3 (Treg) cells, which are associated with autoimmune diseases, was increased compared to the negative control (vehicle). Quantitative analysis of autoantibodies in the blood of MRL/FAS^lpr mice showed a decreasing tendency in the treatment groups with Apitoxin^®. Moreover, mesangial proliferation and inflammatory cell infiltration in glomeruli were significantly reduced in the treatment group with Apitoxin^®, which was associated with a statistically significant decrease in the amount of IgG infiltrated into the glomeruli.</p><p><strong>Conclusion: </strong>Overall, the results confirmed that Apitoxin^® induced clinical improvement in SLE by increasing the proportion of Treg cells and decreasing anti-dsDNA antibodies in the blood, which resulted in therapeutic effects on glomerulonephritis associated with decreased renal infiltration of immune complexes.</p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"65 1","pages":"17"},"PeriodicalIF":2.0,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143665189","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical and laboratory markers defining MIS-C and hyperinflammation in COVID-19: a cross-sectional study in a tertiary hospital.","authors":"Ana Paula Radünz Vieira, Paulo Roberto Antonaccio Carvalho, Sandra Helena Machado, Taís Sica da Rocha","doi":"10.1186/s42358-025-00447-6","DOIUrl":"https://doi.org/10.1186/s42358-025-00447-6","url":null,"abstract":"<p><strong>Background: </strong>Numerous inflammatory complications related to COVID are described, including the Multisystem inflammatory Syndrome in Children (MIS-C) and Hyperinflammation. There is a scarcity of studies comparing these two groups.</p><p><strong>Methods: </strong>Retrospective longitudinal outcome-conditioned study. Demographic, clinical, and laboratory variables are analyzed. Patients with history of COVID contact or infection with at least 24 h of fever, two or more systems involved and up to 21 years were included. Patients with no laboratory signal of inflammation or with other diagnoses for the condition were excluded. Demographic and laboratory data are presented as medians with interquartile ranges. Dichotomous variables and prevalences are reported as percentages. A ROC curve analysis was conducted to assess the discriminatory ability of these tests in relation to the MIS-C and hyperinflammation groups.</p><p><strong>Results: </strong>We present fifty-four patients, thirty-one with MIS-C and twenty-three with hyperinflammation. The most frequent symptom in the MIS-C group was altered mental status in 61% vs. 46% (p = 0.014) and conjunctival hyperemia in 29% vs. 4% (p = 0.032). The most frequent laboratory findings were hypoalbuminemia in 68% vs. 26% (p = 0.002), increased serum troponin in 42% vs. 26% (p = 0.034), increased d-dimers in 94% vs. 76% (p = 0.015), as well as increased BNP in 55% vs. 17% (p = 0.02). On the other hand, the hyperinflammation group more frequently presented respiratory dysfunction in 57% vs. 13% (p = < 0.001) and serum ferritin equal or greater than 500 ng/mL in 94% vs. 77% (p = 0.046).</p><p><strong>Conclusions: </strong>This is an original study comparing clinical and laboratory findings between MIS-C and hyperinflammation due to COVID. Altered mental status is more frequently associated with MIS-C while respiratory symptoms are associated with hyperinflammation. In addition, regarding laboratory tests, there is hypoalbuminemia, increase in serum troponin, BNP, and D-dimers specially in the MIS-C group and hyperferritinemia in the hyperinflammation group. Further studies are needed to assess the cutoff point of biological markers such as BNP, troponin, and d-dimers for diagnosis and/or prognosis in the pediatric population with MIS-C.</p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"65 1","pages":"16"},"PeriodicalIF":2.0,"publicationDate":"2025-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143651474","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Spirulina ingestion and autoimmune disease onset or flare.","authors":"Jozélio Freire de Carvalho, Ana Tereza Amoedo Martinez","doi":"10.1186/s42358-025-00446-7","DOIUrl":"https://doi.org/10.1186/s42358-025-00446-7","url":null,"abstract":"","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"65 1","pages":"15"},"PeriodicalIF":2.0,"publicationDate":"2025-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143634859","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Correlates of depression, anxiety, and stress among patients with ankylosing spondylitis.","authors":"Kun Yang, Yifan Gong, Zhaoyang Geng, Xan Xu, Shiyan Yan, Haoran Zhang, Quan Jiang, Hongxiao Liu","doi":"10.1186/s42358-025-00439-6","DOIUrl":"https://doi.org/10.1186/s42358-025-00439-6","url":null,"abstract":"<p><strong>Background: </strong>The recurrent nature and prolonged course of ankylosing spondylitis (AS) impose substantial psychological disorders on patients. The aim of this study was to assess psychological disorders and analyze the overall risk of psychological disorders as well as the factors associated with depression, anxiety, and stress in ankylosing spondylitis.</p><p><strong>Methods: </strong>Patients diagnosed with AS were selected from the China Rheumatoid Arthritis Registry of Patients with Chinese Medicine (CERTAIN) database for data analysis. General demographic characteristics and disease-related features of the patients were collected. The study analyzed clinical differences between patients with and without psychological disorders. Specific clinical characteristics of depression, anxiety, and stress were statistically analyzed. Clinical factors associated with overall psychological status and specific psychological disorders (depression, anxiety and stress) were analyzed by multivariate logistic regression.</p><p><strong>Results: </strong>In our study cohort, 26.72% of AS patients were identified with psychological disorders, with 17.5% experiencing depression, 21.1% suffering from anxiety, and 7.9% reporting stress. We also observed significant overlaps among depression, anxiety, and stress in AS patients, with 53.47% experiencing multiple psychological disorders. Disease activity, health index, fatigue levels, and PGA were identified as significant factors associated with psychological disorders. Age, health index, fatigue levels, and PGA were the main influencing factors for depression; disease activity and PGA for anxiety; and disease activity, ASAS-HI, and fatigue for stress.</p><p><strong>Conclusions: </strong>The study reveals a significant prevalence of psychological disorders among individuals with AS, which correlates closely with disease activity, health index, fatigue levels, and PGA. These findings highlight the imperative for assessment of psychological conditions into the comprehensive management approach for AS patients.</p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"65 1","pages":"14"},"PeriodicalIF":2.0,"publicationDate":"2025-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143634912","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Health-related quality of life significantly improved in obese patients with psoriatic arthritis one year after a structured weight loss intervention.","authors":"Anton J Landgren, Annelie Bilberg, Björn Eliasson, Linda Torres, Mats Dehlin, Lennart T H Jacobsson, Ingrid Larsson, Eva Klingberg","doi":"10.1186/s42358-025-00444-9","DOIUrl":"https://doi.org/10.1186/s42358-025-00444-9","url":null,"abstract":"<p><strong>Objective: </strong>In this interventional weight loss study, health-related quality of life (HRQoL), anxiety, depression and fatigue were compared at baseline (BL) and at 12 months (M12) in patients with psoriatic arthritis (PsA) and controls.</p><p><strong>Methods: </strong>PsA patients (n = 39) between 25 and 75 years of age, with body mass index (BMI) ≥ 33 kg/m² were included in a weight loss intervention with very low energy diet (VLED) for 12 or 16 weeks depending on BL BMI < 40 or ≥ 40 kg/m². The 36-item short-form health survey (SF-36) was used to assess HRQoL. Anxiety and depression were assessed by the Hospital Anxiety and Depression Scale. Assessments were done at BL, M3, M6 and M12. As controls (n = 39), obese individuals, already planned for VLED treatment were recruited and matched for sex, age and weight to the PsA patients.</p><p><strong>Results: </strong>In PsA patients, physical HRQoL, as demonstrated by the physical component summary (PCS) of SF-36, improved from median (IQR) 34 (25-45) at BL to 43 (34-50) at M12, p = 0.009. No significant effect on mental HRQoL, demonstrated by the mental component summary (MCS) score, was seen. Similarly in controls, PCS significantly improved (median IQR, 44 (36-50) at BL to 52 (44-55) at M12, p < 0.001), whereas no significant improvement was seen in MCS. Anxiety and depression decreased significantly in both PsA patients and controls.</p><p><strong>Conclusions: </strong>The weight loss intervention was associated with significant improvements in physical HRQoL, as well as anxiety and depression, in PsA patients and controls.</p><p><strong>Trial registration: </strong>ClinicalTrials.gov identifier: NCT02917434, registered on September 21, 2016, retrospectively registered.</p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"65 1","pages":"13"},"PeriodicalIF":2.0,"publicationDate":"2025-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143634855","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Tripled and sustained change in pregnancy-related annual mortality rates with systemic lupus erythematosus involvement: a nationwide temporal trends study, Brazil, 2006-2022.","authors":"Rodrigo Poubel Vieira de Rezende, Bruno Santos Caxias, Anna Beatriz da Silva Rodrigues, Omar Hazem Ashmawi, Luiz Eduardo da Costa Oliveira","doi":"10.1186/s42358-025-00445-8","DOIUrl":"https://doi.org/10.1186/s42358-025-00445-8","url":null,"abstract":"","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"65 1","pages":"12"},"PeriodicalIF":2.0,"publicationDate":"2025-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143634860","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Efficacy of two doses of intra-articular ozone therapy for pain and functional mobility in knee osteoarthritis: a double-blind randomized trial.","authors":"Zahra Arjmanddoust, Ahmad Nazari, Azar Moezy","doi":"10.1186/s42358-025-00443-w","DOIUrl":"https://doi.org/10.1186/s42358-025-00443-w","url":null,"abstract":"<p><strong>Aim: </strong>This double-blind, trial sought to assess the effectiveness of intra-articular ozone therapy at concentrations of 20 µg/mL and 40 µg/mL in managing pain and enhancing functional mobility in patients with knee osteoarthritis (KOA).</p><p><strong>Method: </strong>This parallel, three-arm randomized controlled trial, conducted between 2022 and 2023, included 59 knee osteoarthritis (KOA) patients randomly allocated to one of three groups: the first group received 40 µg/mL ozone therapy, the second group received 20 µg/mL ozone therapy, and the control group received oxygen. Functional mobility was assessed through the Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC), knee flexion range of motion (FROM), the Timed Up and Go (TUG) test, and the six-minute walk test (6MWT). Pain intensity was measured using the Visual Analog Scale (VAS) and the pain subscale of the WOMAC. Intra-articular injections were administered weekly for four consecutive weeks, with assessments conducted pre-treatment, and at two weeks, one month, and two months post-intervention. One-way ANOVA was employed for normally distributed quantitative data, while the Kruskal-Wallis test was utilized for non-normally distributed data. Qualitative variables were analyzed using the Chi-squared or Fisher's exact test, as appropriate.</p><p><strong>Results: </strong>The groups receiving intra-articular ozone therapy exhibited notable reductions in mean VAS scores and improvement in functional mobility variables when compared to the control group (p < 0.05). However, post-hoc analysis indicated no statistically significant differences between the 40 µg/mL and 20 µg/mL ozone therapy groups regarding these parameters (VAS, FROM, TUG, 6MWT, or WOMAC scores) (p > 0.05).</p><p><strong>Conclusion: </strong>Both 20 µg/mL and 40 µg/mL doses of intra-articular ozone therapy prove to be effective in reducing pain and enhancing functional mobility in patients with knee osteoarthritis (KOA). Nevertheless, there was no significant difference in the efficacy between the two ozone concentrations.</p><p><strong>Trial registration: </strong>The trial is registered on us ClinicalTrials.gov in 2024-05-01 with the following ID code NCT06088706.</p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"65 1","pages":"11"},"PeriodicalIF":2.0,"publicationDate":"2025-03-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143574511","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Non-cirrhotic Idiopathic portal hypertension in systemic sclerosis patients: report of one case and a systematic review of previous case reports.","authors":"Felipe Souza da Silva, João Victor de Pinho Costa, Carlos Alberto Dos Santos Júnior, Érika Emmylaine Dos Santos, Ailton José de Castro Júnior, Ana Cecília de Sena Oliveira, Flávia Patrícia Sena Teixeira Santos, Adriana Maria Kakehasi, Débora Cerqueira Calderaro","doi":"10.1186/s42358-025-00442-x","DOIUrl":"https://doi.org/10.1186/s42358-025-00442-x","url":null,"abstract":"<p><strong>Background: </strong>The overlap of non-cirrhotic idiopathic portal hypertension (NCIPH) and systemic sclerosis (SSc) is rare. This article reports one case of a patient with SSc developing NCIPH and presents a systematic review of previously reported cases.</p><p><strong>Methods: </strong>CARE guidelines and the PRISMA statement were applied.</p><p><strong>Results: </strong>We report the case of a 52 year-old woman, presenting, in 2015, diffuse cutaneous scleroderma (SSc), treated with oral prednisolone and monthly intravenous cyclophosphamide. Three months later, she developed a scleroderma renal crisis, requiring hemodialysis for 18 months. Since 2017 she has not been on immunosuppressive treatment for SSc, the cutaneous involvement improved, and she has a stable Kdigo 3 chronic kidney disease. In 2019, she developed ascites. During investigation, NCIPH leading to small and medium esophageal varices and collateral circulation was diagnosed. Currently, the patient is undergoing prophylactic endoscopic band ligation of the esophageal varices and presents a stable condition. In the systematic review, 18 papers reporting 20 cases of NCIPH associated with SSc were included. Seventeen (81%) patients were women, with [Mean (SD)]: 56.71 (12.97) years. Classification of SSc was (N = 15): 10 limited, 4 diffuse, and 1 sin scleroderma. Clinical presentation of NCIPH was esophageal and/or gastric varices [19 (90,5%)], ascites [10 (47,6%)], and upper gastrointestinal bleeding [9 (42,8%)]. NCIPH was treated with diuretics [n = 9 (42,8%)], endoscopic esophageal varices sclerosis or band ligation [n = 7 (35%)], and beta-blockers [n = 4 (19%)]. Recovery of symptoms, or stabilization of clinical condition was reported in nine patients. Despite the death of seven patients, only one was attributed to the hepatic condition.</p><p><strong>Conclusions: </strong>NCIPH has been rarely reported in SSc patients. NCIPH prognosis in SSc is good. Due to the scarcity of cases reporting the occurrence of both diseases, the characteristics of SSc patients at risk of developing NCIPH remain unclear.</p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"65 1","pages":"10"},"PeriodicalIF":2.0,"publicationDate":"2025-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143558028","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}