da Silva Felipe Souza, de Pinho Costa João Victor, Júnior Carlos Alberto Dos Santos, Santos Érika Emmylaine Dos, Júnior Ailton José de Castro, Oliveira Ana Cecília de Sena, Santos Flávia Patrícia Sena Teixeira, Kakehasi Adriana Maria, Calderaro Débora Cerqueira
Faculdade de Medicina da Universidade Federal de Minas, Avenida Professor Alfredo Balena, 190, Belo Horizonte, Minas Gerais, 30130-100, Brazil.
Hospital das Clínicas da Universidade Federal de Minas Gerais, Belo Horizonte, Brazil.
Adv Rheumatol. 2025 Mar 4;65(1):10. doi: 10.1186/s42358-025-00442-x.
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.
CARE guidelines and the PRISMA statement were applied.
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.
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.
非肝硬化性特发性门静脉高压（NCIPH）与系统性硬化症（SSc）的重叠情况罕见。本文报告了1例患有SSc并发展为NCIPH的患者，并对先前报道的病例进行了系统综述。
应用CARE指南和PRISMA声明。
我们报告了1例52岁女性患者，2015年出现弥漫性皮肤型硬皮病（SSc），接受口服泼尼松龙和每月静脉注射环磷酰胺治疗。3个月后，她发生了硬皮病肾危象，需要进行18个月的血液透析。自2017年以来，她未接受针对SSc的免疫抑制治疗，皮肤受累情况改善，并且患有稳定的Kdigo 3期慢性肾脏病。2019年，她出现腹水。在检查过程中，诊断为NCIPH导致中小食管静脉曲张和侧支循环形成。目前，该患者正在接受食管静脉曲张的预防性内镜套扎治疗，病情稳定。在系统综述中，纳入了18篇报告20例与SSc相关的NCIPH病例的论文。17例（81%）患者为女性，平均（标准差）年龄为56.71（12.97）岁。SSc的分类为（N = 15）：10例局限性，4例弥漫性，1例无硬皮病。NCIPH的临床表现为食管和/或胃静脉曲张[19例（90.5%）]、腹水[10例（47.6%）]和上消化道出血[9例（42.8%）]。NCIPH的治疗方法包括利尿剂[n = 9例（42.8%）]、内镜下食管静脉曲张硬化术或套扎术[n = 7例（35%）]以及β受体阻滞剂[n = 4例（19%）]。9例患者症状有所缓解或病情稳定。尽管有7例患者死亡，但仅1例归因于肝脏疾病。
NCIPH在SSc患者中鲜有报道。SSc患者中NCIPH的预后良好。由于同时报告这两种疾病发生情况的病例较少，发生NCIPH风险的SSc患者的特征仍不清楚。
