Published online Aug 7, 2021. doi: 10.3748/wjg.v27.i29.4929
Peer-review started: February 25, 2021
First decision: April 18, 2021
Revised: April 29, 2021
Accepted: July 13, 2021
Article in press: July 13, 2021
Published online: August 7, 2021
Processing time: 159 Days and 17.7 Hours
Autoimmune enteropathy (AIE) and primary biliary cholangitis (PBC) are both immune-mediated diseases. AIE or PBC complicated with ulcerative colitis (UC) are rare. There are no cases of AIE and PBC diagnosed after proctocolectomy for UC reported before, and the pathogenesis of these comorbidities has not been revealed.
A middle-aged woman diagnosed with UC underwent subtotal colectomy and ileostomy due to the steroid-resistant refractory disease, and a restorative proctectomy with ileal pouch-anal anastomosis and proximal neoileostomy was postponed due to active residual rectal inflammation in January 2016. A few months after the neoileostomy, she began to suffer from recurrent episodes of watery diarrhea. She was diagnosed with postcolectomy enteritis and stoma closure acquired a good therapeutic effect. However, her symptoms of diarrhea relapsed in 2019, with different histological features of endoscopic biopsies compared with 2016, which showed apoptotic bodies, a lack of goblet and Paneth cells, and villous blunting. A diagnosis of AIE was established, and the patient’s stool volume decreased dramatically with the treatment of methylprednisolone 60 mg/d for 1 wk and tacrolimus 3 mg/d for 4 d. Meanwhile, her constantly evaluated cholestatic enzymes and high titers of antimitochondrial antibodies indicated the diagnosis of PBC, and treatment with ursodeoxycholic acid (16 mg/kg per day) achieved satisfactory results.
Some immune-mediated diseases may be promoted by operation due to microbial alterations in UC patients. Continuous follow-up is essential for UC patients with postoperative complications.
Core Tip: This is the first case report of autoimmune enteropathy and primary biliary cholangitis complicated with ulcerative colitis, and the female patient in this case suffered from a tortuous process of diagnosis and treatment. It is speculated that a microbial shift and subsequent immune response are involved in the pathogenesis of these comorbidities, and surgery may facilitate the progression of coexisting diseases. Understanding the progression of these diseases may help with their early recognition and treatment.