IgG4 Disease Revealed by a Type 1 Autoimmune Pancreatitis: A Case Report

Abstract

Aim:  To describe clinical, paraclinical and evolutive features of an IgG4 disease. Presentation of Case: The patient was a male, 51 years old, seen in consultation in the gastro-enterology and hepatology department of University teaching hospital “Campus” of Lome for epigastric pain of sudden onset six days before admission, with transfixing irradiation, evolving intermittently in a febrile context. On admission, the patient has a subicterus. The evolution during hospitalization was marked by the sudden onset three days later of bilateral inflammatory polyarthritis of the knees and ankles. Biological examination revealed diabetes mellitus; lipasemia was elevated to 122U/l. There was a disturbance of the renal balance; and a significant increase of plasma IgG4 at 2.380 g/l. The abdominal ultrasound showed a globally hypertrophic pancreatic gland, of heterogeneous structure, without focal lesions or necrosis; without biliary lithiasis. An abdominal CT scan showed, after injection of contrast medium, a global hypertrophy of the pancreas with a corporal-caudal predominance, giving a classic "sausage" appearance; associated with a lack of enhancement of the pancreatic parenchyma. Conclusion: Type 1 autoimmune pancreatitis is the pancreatic manifestation of IgG4 disease. It is a rare pathology of recent discovery, with a predominance in elderly and male subjects. The diagnosis must be evoked in front of a pancreatic pain associated with articular manifestations, or in case of absence of obvious etiology of a pancreatitis. This diagnosis is defined by recommandations of International Association of Pancreatology. The treatment is based on the use of corticosteroids unlike autoimmune pancreatitis type 2 which is self-limiting with a low risk of relapse and complications.