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
{"title":"系统性硬化症患者的非肝硬化特发性门静脉高压症：1例报告及对以往病例报告的系统回顾。","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":null,"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":2.1000,"publicationDate":"2025-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"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\":null,\"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\":2.1000,\"publicationDate\":\"2025-03-04\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Advances in Rheumatology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1186/s42358-025-00442-x\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"RHEUMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Advances in Rheumatology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1186/s42358-025-00442-x","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"RHEUMATOLOGY","Score":null,"Total":0}
Non-cirrhotic Idiopathic portal hypertension in systemic sclerosis patients: report of one case and a systematic review of previous case reports.
Background: 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.
Methods: CARE guidelines and the PRISMA statement were applied.
Results: 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.
Conclusions: 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.
期刊介绍：
Formerly named Revista Brasileira de Reumatologia, the journal is celebrating its 60th year of publication.
Advances in Rheumatology is an international, open access journal publishing pre-clinical, translational and clinical studies on all aspects of paediatric and adult rheumatic diseases, including degenerative, inflammatory and autoimmune conditions. The journal is the official publication of the Brazilian Society of Rheumatology and welcomes original research (including systematic reviews and meta-analyses), literature reviews, guidelines and letters arising from published material.
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