Primary sclerosing cholangitis (PSC) is a cholangiopathy-usually associated with
inflammatory bowel disease-that leads to
cirrhosis and
liver failure. Based on a multitude of clinical trials, there is general consensus that PSC progression is unchanged by current
therapies, including
steroids. However, there are scattered reports in the literature of PSC patients responsive to
steroids. Recently, several
steroid-responsive PSC mimics have been described, most notably
immunoglobulin G4-related
sclerosing cholangitis. Following these discoveries, many assume that cases in the literature previously reported as
steroid-responsive PSC would now be classified as one of these mimics. We reviewed liver biopsies and the medical histories of patients diagnosed with PSC with documented response to
steroids. We identified 3 cases of
steroid-responsive PSC in patients with
inflammatory bowel disease that do not fit criteria of known PSC mimics. All 3 were adults (age range = 18-44 years) with
inflammatory bowel disease, and included 2 males and 1 female. All 3 patients had abnormal liver function tests that normalized on
prednisone. Histologically, these 3 cases share a common feature, hepatic fibroinflammatory nodules in a
collagen-rich background. They lacked clinical, serologic, and histologic features of
immunoglobulin G4-related
sclerosing cholangitis. These cases suggest that fibroinflammatory nodules may identify a unique subset of PSC patients who are responsive to
steroids.