Refractory Chronic Pouchitis and Autoimmune Hemolytic Anemia Successfully Treated with Vedolizumab

Pouchitis is the most common adverse event among patients with ulcerative colitis (UC) who underwent restorative proctocolectomy with ileal pouch-anal anastomosis. Although initial acute episodes typically respond to antibiotics, patients may become antibiotic dependent or develop refractory disease [1]. Immunosuppressants, anti-TNFs, topical therapy, and surgery are therapeutic options when refractoriness is established but final decision is still based on a case-by-case basis [2]. Recently, few case reports of successful use of Vedolizumab and Ustekinumab in refractory pouchitis have been published [3, 4]. Autoimmune hemolytic anemia (AIHA) is a rare extraintestinal manifestation of UC, usually responding to proctocolectomy [5], with no previous reports of AIHA starting after surgery. We report the singular case of a patient with chronic pouchitis (CP) and AIHA, refractory to antibiotics, steroids, and anti-TNF therapy, who achieved endoscopic remission and subsequent anemia resolution after starting Vedolizumab. A 20-year-old woman was diagnosed with extensive severe acute UC at the age of 16. She underwent an ileal pouch-anal anastomosis due to refractoriness to steroids and Infliximab. One year later, a relapsing CP was diagnosed based on disabling symptoms, elevated inflammatory markers, pouchoscopy, and histologic findings. Initially she was treated with antibiotics (ciprofloxacin plus metronidazole) and oral budesonide with partial clinical response. Complementary study excluded infectious pouchitis, Crohn’s disease, vasculitis, ischemia, and autoimmune pouchitis. Simultaneously she was diagnosed with a severe symptomatic AIHA with the lowest hemoglobin level of 5 g/dL, which required therapy with high doses of steroids. AIHA diagnosis was established according to the increased total bilirubin levels (3 times the upper limit of normal) with predominance of unconjugated bilirubin, increased lactate dehydrogenase (2.5 times higher than the normal range), consumption of haptoglobin (< 10 mg/dL), increased reticulocyte count, positive Coombs test (direct and indirect), and increased erythroblasts count with normal myeloid-erythroid ratio