ABSTRACT
A 74-year-old male patient presented with progressive anorexia, cholestatic liver function tests, and a diffuse enlarged pancreas suggestive of a pancreatic carcinoma. There was a marked elevation of total immunoglobulin G4 (IgG4) in serum. Further investigation led to the diagnosis of IgG4-related sclerosing disease with involvement of the pancreas, retroperitoneal fibrosis, and bilateral focal nephritis. To our knowledge, this is the first report on these 3 clinical entities occurring in the same patient.A short review of the literature concerning autoimmune pancreatitis and retroperitoneal fibrosis is made, with special interest to the concept of IgG4-related pathology. This systemic disease can have several clinical manifestations: IgG4-positivity not only can be found in the pancreas, but also at the level of extrahepatic biliary ducts, gallbladder, salivary glands, retroperitoneal tissue, kidneys, ureters, and lymph nodes. Although further investigation is required to determine its exact pathophysiologic role, IgG4 seems to be an important key player.
Subject(s)
Autoimmune Diseases/pathology , Immunoglobulin G/blood , Nephritis/pathology , Pancreatitis/pathology , Retroperitoneal Fibrosis/pathology , Adrenal Cortex Hormones/therapeutic use , Aged , Autoimmune Diseases/complications , Autoimmune Diseases/drug therapy , Diagnosis, Differential , Humans , Male , Nephritis/complications , Nephritis/drug therapy , Pancreas/drug effects , Pancreas/pathology , Pancreatitis/complications , Pancreatitis/drug therapy , Retroperitoneal Fibrosis/complications , Retroperitoneal Fibrosis/drug therapy , Sclerosis , Treatment OutcomeABSTRACT
A 56-year-old patient, first diagnosed with an acute cytomegalovirus infection, presented with progressive abdominal pain because of a superior mesenteric vein thrombosis for which he was treated with systemic thrombolysis and heparin in continuous infusion. As this therapy did not have the intended success after 5 days, an interventional radiological procedure was performed with local thrombolysis in the superior mesenteric artery resulting in recanalisation of the vein. Oral anticoagulation was initiated and continued for a period of 6 months. Mesenteric venous thrombosis is a relatively uncommon cause of mesenteric ischemia that can be associated with severe morbidity and significant mortality. With noninvasive techniques, it is possible to establish a diagnosis in the majority of the cases. The importance of an early diagnosis and therapy - not only with anticoagulation, but also thrombolysis in selected cases - is shown with this case and review of the literature.
