Abstract
Atypical presentation of IgG4-related autoimmune pancreatitis - be awake to early detecting subsequent renal involvement: case report and literature review
Highlights
Type 1 autoimmune pancreatitis (AIP), a distinct form of chronic pancreatic disease is mostly reported in elder men (60 years old), presenting with obstructive jaundice, weight loss, mild abdominal pain and occasionally associated with other autoimmune diseases [1,2] .Imaging of type 1 AIP may mimic pancreatic carcinom [3,4,5,6]
Type 1 AIP is considered as a prototype manifestation of IgG4-related systemic disease (IgG4-RSD) [11]; a systemic syndrome characterized by mass-forming lesions mainly in exocrine organs that consist of IgG4 positive plasma cells infiltrate and significant fibrosis [12,13,14,15]
As two years after relapse of AIP a progressive chronic renal insufficiency occurred, we underline the importance of biological screening of renal function and the usefulness of diffusion-weighted magnetic resonance imaging (DWMRI) for early detection of renal lesions during the long term follow up of such patients
Summary
Type 1 autoimmune pancreatitis (AIP), a distinct form of chronic pancreatic disease is mostly reported in elder men (60 years old), presenting with obstructive jaundice, weight loss, mild abdominal pain and occasionally associated with other autoimmune diseases [1,2] .Imaging of type 1 AIP may mimic pancreatic carcinom [3,4,5,6]. As two years after relapse of AIP a progressive chronic renal insufficiency occurred, we underline the importance of biological screening of renal function and the usefulness of diffusion-weighted magnetic resonance imaging (DWMRI) for early detection of renal lesions during the long term follow up of such patients. The physical examination marked by jaundice and the blood tests demonstrated liver cytolysis, cholostasis and elevation of bilirubine and of cancer antigen (CA) 19-9 level (Figure 1). Swelling of the pancreatic head with dilated intrahepatic bile ducts was demonstrated by abdominal CT (Figure 2). The MDZ was tapered progressively until June 2006 At this moment, renal function was normal (plasma creatinine (PCr) level at 0.9 mg/dL (79.6 mmol/L) (Figure 1). In August 2008, renal function deteriorated as demonstrated by progressive increased of PCr from 0.9 mg/dL to 1.4 mg/dL [79.6 to 123.8 mmol/L] over
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