A case report and literature review has found that autoimmune hemolytic anemia (AIHA), of which cold agglutinin disease (CAD) is a form, is a rare extraintestinal entity in patients with inflammatory bowel disease (IBD).

The report, published in Life, noted that AIHA was more frequent in ulcerative colitis (UC) than in Crohn’s disease (CD).

“AIHA is rare in IBD patients, and the majority of previously reported cases are diagnosed in patients with UC,” the authors wrote. “In this case-based review article we report a case of a female patient with colonic CD, Coombs positive AIHA, and pulmonary thromboembolism, and provide an extensive review of the literature on AIHA in IBD patients together with possible underlying mechanisms and therapeutic strategies used so far.”

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The patient in the case report was a 36-year-old woman with no medical history of interest who presented with diarrhea, abdominal pain, fever, and altered consciousness following a period of intentional weight loss. Extensive physical and laboratory testing revealed gallstones, reactive bone marrow with erythroid hyperplasia, positive direct Coombs test, elevated immunoglobulin G, and positive antismooth muscle and antinuclear autoantibodies, leading to a diagnosis of AIHA.   

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Treatment with corticosteroids and antibiotics was successful but she returned several months later with bloody diarrhea; further testing revealed CD. Ultimately, AIHA was established as an extraintestinal manifestation of CD.

After treatment with corticosteroids and adalimumab, she had several periods of remission and relapse until her therapy was switched to vedolizumab. Ongoing treatment with vedolizumab has resulted in complete remission of the CD and no flare-ups of AIHA since late 2019.

The authors recommend monitoring of drug trough levels and early optimization of drug dosing in patients with CD, and they note that the addition of biologic therapy to traditional treatment with corticosteroids and surgery can be helpful. Further studies to determine whether AIHA is a rare or underdiagnosed manifestation of CD are warranted.


Toplicanin A, Tonce L, Zaric V, et al. Autoimmune hemolytic anemia in inflammatory bowel disease—report of a case and review of the literature. Life. Published online November 4, 2022. doi:10.3390/life12111784