Idiopathic Calcific Panereatitis, CRST Syndrome and Progressive Systemic Sclerosis

A 43-year old man with CRST syndrome (calcinosis, Raynaud's phenomenon, sclerodactyly and telangiectasia) and progressive systemic sclerosis presented with a four-year history of relapsing abdominal pain, the result of chronic pancreatitis, not associated with alcoholism, biliary disease, or any of the known causes of pancreatitis. He had a good response to retrograde pancreatic duct drainage but exhibited management problems and complications that may be peculiar to the systemic sclerosis patient with pancreatitis. A cause and effect relationship between progressive systemic sclerosis and pancreatic disease is not proven but we believe there is evidence to suggest such a relationship.