Hereditary pancreatitis: clinical presentation, ERCP findings, and outcome of endoscopic therapy

Background: Hereditary pancreatitis (HP) is an autosomal dominant disorder characterized by the onset of recurrent attacks of acute pancreatitis in childhood and frequent progression to chronic pancreatitis. There are limited data on the role of diagnostic and therapeutic ERCP in patients with HP. The aims of this study were to describe the clinical presentation and findings on pancreatography and to evaluate the role of endoscopic and surgical intervention in patients with HP. Methods: Twenty-seven consecutive patients with HP were referred for ERCP at two centers in the midwest United States. Medical records and pancreatograms were reviewed. Results: Pancreatography was abnormal in all patients. The majority (70%) had severe (Cambridge Grade III) changes of chronic pancreatitis. Nineteen (70%) patients underwent endoscopic therapy and 8 (30%) surgical intervention. After endoscopic therapy (mean follow-up 32 months), 500/6 were symptom free, 38% had moderate improvement, and 12% had no or transient improvement in symptoms. After surgery (mean follow-up 32 months), 38% were symptom-free, 25% had moderate improvement, and 12% had transient improvement in symptoms. Two patients treated surgically died of pancreatic cancer. Conclusions: In the short-term, improving drainage of the pancreatic ductal system by endoscopic means effectively relieves pain and reduces the number of attacks of pancreatitis in the majority of patients with HP.