Cronkhite-Canada syndrome: a retrospective analysis of four cases at a single medical center

Spontaneous remission of Cronkhite–Canada syndrome without immunosuppressive medication in the context of decompensated cirrhosis: A case report

“…Given the hypothesized immune dysregulation in CCS, systemic immunosuppression is considered the most effective treatment. 3 , 6 , 7 , 15 However, there is still minimal evidence to support whether the treatment has a long‐term mortality benefit particularly in patient cohorts at increased risk of side effects from corticosteroids, such as in our case. Patients who have remission induced with corticosteroids usually relapse after completion of induction dosing, meaning an extended wean or long‐term low‐dose therapy is required.…”

“…Patients who have remission induced with corticosteroids usually relapse after completion of induction dosing, meaning an extended wean or long‐term low‐dose therapy is required. 15 The symptom and mortality benefit would have to be compelling to justify use in these circumstances, and in our case, we felt that the unknown benefit was outweighed by the known risks of corticosteroid therapy. Prior to diagnosis, our patient used regular hydrocortisone at a physiologic dose (equivalent to 5 mg prednisolone) for panhypopituitarism, and at this dose, it was unlikely to have altered the course of his disease.…”

“…Given the hypothesized immune dysregulation in CCS, systemic immunosuppression is considered the most effective treatment 3,6,7,15 . However, there is still minimal evidence to support whether the treatment has a long‐term mortality benefit particularly in patient cohorts at increased risk of side effects from corticosteroids, such as in our case.…”

“…However, there is still minimal evidence to support whether the treatment has a long‐term mortality benefit particularly in patient cohorts at increased risk of side effects from corticosteroids, such as in our case. Patients who have remission induced with corticosteroids usually relapse after completion of induction dosing, meaning an extended wean or long‐term low‐dose therapy is required 15 . The symptom and mortality benefit would have to be compelling to justify use in these circumstances, and in our case, we felt that the unknown benefit was outweighed by the known risks of corticosteroid therapy.…”