The natural course of autoimmune pancreatitis.
OBJECTIVE
Since autoimmune pancreatitis (AIP) responds dramatically to steroid therapy, most AIP patients are promptly treated with steroids when the diagnosis of AIP is made. Therefore, the natural course of AIP is unclear. This study aimed to evaluate the clinical course of AIP patients without steroid therapy and assess the indications for steroid therapy in these patients.


METHODS
Clinical features were retrospectively assessed in 12 patients who were followed for more than 6 months after the diagnosis of AIP without steroids.


RESULTS
Six patients were later treated with steroids due to exacerbation of AIP. Five of them developed obstructive jaundice due to bile duct stenosis. Segmental enlargement progressed to diffuse enlargement in 4 patients. Serum IgG and/or IgG4 levels increased with AIP progression. In 4 patients, swelling of the salivary glands preceded AIP. Radiological and clinical features responded well to steroid therapy. Spontaneous improvement occurred in 3 patients. Four asymptomatic patients with segmental pancreatic enlargement have demonstrated no changes without steroid therapy until now.


CONCLUSIONS
About half of the segmental AIP cases progressed and needed steroid therapy, which was effective. Asymptomatic segmental AIP cases without biliary lesions may be followed without steroid therapy with periodic laboratory and imaging studies.
Kamisawa
Terumi
T
Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan. kamisawa@cick.jp
Anjiki
Hajime
H
Takuma
Kensuke
K
Egawa
Naoto
N
Kubota
Naoko
N
D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000150:complications* / Q000473:pathology* / Q000628:therapy; D015331:Cohort Studies; D018450:Disease Progression; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008297:Male; D008875:Middle Aged; D010180:Pancreatectomy; D010195:Pancreatitis / Q000150:complications* / Q000473:pathology* / Q000628:therapy; D012189:Retrospective Studies
D000893:Anti-Inflammatory Agents; D005938:Glucocorticoids