Introduction:

Idiopathic autoimmune hepatitis (IAH) is a chronic disease that occurs predominately in women, has episodic activity and remission, and favors hepatic fibrosis. Forty percent of patients have family histories of autoimmune diseases. It is apparently mediated by antigen-antibody interaction, but its causes are unknown. IAH is frequently associated with hepatobiliary cancer, less frequently with lymphomas, skin cancer and colon cancer and very rarely with hereditary colon cancer syndrome. This case debuted IAH and rectal bleeding caused by familial adenomatous polyposis (FAP) and adenocarcinoma of the sigmoid colon.

Clinical case:

The patient was a 51-year-old woman who had had IAH for one year which had been managed with prednisolone and azathioprine. A total colonoscopy, performed because of anemia, found multiple polyps that measured 5 and 10 mm and one ulcerated 30 mm sessile polyp in the sigmoid colon. A multiple endoscopic polypectomy revealed a tubulovillous adenoma with high and low grade dysplasia in several polyps and a low grade adenocarcinoma in the sigmoid polyp. Tests and examinations for metastasis were negative. Following presurgical nutritional repletion, a subtotal colectomy was performed and an ileal pouch-anal anastomosis with protective ileostomy was created. The pathology of the surgical specimen showed low grade adenocarcinoma of the colon and tubular and tubulovillous adenomas with high and low grade dysplasia.

Discussion and conclusions:

Association of IAH with familial adenomatous polyposis (FAP) and colorectal cancer (CC) occurs infrequently although associations of IAH with hepatobiliary cancer associated with cirrhosis, lymphomas, skin cancer and other autoimmune disorders are well-known. The prognosis is bad and no clear correlation with immune modulators can be established.

        · resumo em Espanhol     · texto em Espanhol | Inglês     · Inglês ( pdf ) | Espanhol ( pdf )