Pinto Rodrigo Ambar, Corrêa Neto Isaac José Felippe, Nahas Sérgio Carlos, Bustamante Lopes Leonardo Alfonso, Sobrado Júnior Carlos Walter, Cecconello Ivan
Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, Disciplina de Cirurgia do Aparelho Digestivo Coloproctologia, Departamento de Gastroenterologia, São Paulo, SP, Brasil.
Arq Gastroenterol. 2018 Nov;55Suppl 1(Suppl 1):47-51. doi: 10.1590/S0004-2803.201800000-49. Epub 2018 Oct 4.
Scleroderma or progressive systemic sclerosis is characterized by a chronic inflammatory process with proliferation of fibrous connective tissue and excessive deposition of collagen and extracellular matrix in the skin, smooth muscle, and viscera. The smooth muscle most involved in scleroderma is that of the esophagus, and dysphagia is the most commonly reported symptom. However, the internal anal sphincter may also be impaired by degeneration and fibrosis, leading to concomitant anal incontinence in scleroderma patients. These patients may neglect to complain about it, except when actively questioned.
To assess anorectal function and anatomy of female scleroderma patients with symptoms of anal incontinence through Cleveland Clinic Florida Fecal Incontinence Score (CCFIS), anorectal manometry and endoanal ultrasound at the outpatient clinic of colorectal and anal physiology, Clinics Hospital, University of São Paulo Medical School (HC-FMUSP).
Female scleroderma patients were prospectively assessed and questioned as to symptoms of anal incontinence. The anorectal manometry and endoanal ultrasound results were correlated with clinical data and symptoms.
In total, 13 women were evaluated. Their mean age was 55.77 years (±16.14; 27-72 years) and their mean disease duration was 10.23 years (±6.23; 2-23 years). All had symptoms of fecal incontinence ranging from 1 to 15. Seven (53.8%) patients had fecal incontinence score no higher than 7; three (23.1%) between 8 and 13; and three (23.1%) 14 or higher, corresponding to mild, moderate, and severe incontinence, respectively. Ten (76.92%) patients had hypotonia of the internal anal sphincter. Three-dimensional endoanal ultrasound showed tapering associated with muscle atrophy of the internal sphincter in six cases and previous muscle defects in three cases.
A functional and anatomical impairment of the sphincter is an important factor to assess in patients with progressive systemic sclerosis and it should not be underestimated.
硬皮病或进行性系统性硬化症的特征是慢性炎症过程，伴有纤维结缔组织增生以及皮肤、平滑肌和内脏中胶原蛋白和细胞外基质的过度沉积。硬皮病中受累最严重的平滑肌是食管平滑肌，吞咽困难是最常报告的症状。然而，内括约肌也可能因变性和纤维化而受损，导致硬皮病患者并发肛门失禁。这些患者可能不会主动提及此事，除非被主动询问。
通过圣保罗大学医学院临床医院（HC-FMUSP）结直肠和肛门生理学门诊的克利夫兰诊所佛罗里达粪便失禁评分（CCFIS）、肛门直肠测压和腔内超声，评估有肛门失禁症状的女性硬皮病患者的肛门直肠功能和解剖结构。
对女性硬皮病患者进行前瞻性评估，并询问其肛门失禁症状。将肛门直肠测压和腔内超声结果与临床数据及症状相关联。
总共评估了13名女性。她们的平均年龄为55.77岁（±16.14；27 - 72岁），平均病程为10.23年（±6.23；2 - 23年）。所有患者的粪便失禁症状评分为1至15分。7名（53.8%）患者的粪便失禁评分不高于7分；3名（23.1%）在8至13分之间；3名（23.1%）为14分或更高，分别对应轻度、中度和重度失禁。10名（76.92%）患者存在内括约肌低张。三维腔内超声显示6例内括约肌逐渐变细并伴有肌肉萎缩，3例有既往肌肉缺陷。
括约肌的功能和解剖结构受损是进行性系统性硬化症患者评估中的一个重要因素，不应被低估。
