Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition of unknown etiology, with presumed autoimmune mechanisms. It is characterized by high levels of IgG4 and variable clinical manifestations. It can involve one or multiple organs. Herein, we reported the case of a 62-year-old man with three organs involvement. He initially presented with recurrent jaundice. Laboratory analysis revealed cholestasis, high gamma-globulin levels, renal failure, and proteinuria. Abdominal Magnetic Resonance Imaging (MRI) showed segmental strictures of the left intrahepatic bile ducts and the wirsung duct with an increased volume of the pancreas and diffuse bilateral enlargement of the kidneys. Laboratory tests revealed high IgG4 levels (770 mg/dL). Based on the biological and radiological findings, we have suggested the diagnosis of systemic IgG4-related disease involving bile ducts, the pancreas, and probably the kidneys. Renal biopsy revealed lymphoplasmacytic infiltrate and fibrosis, but no IgG4-positive cell. The patient received corticosteroid therapy with a complete resolution of all symptoms and a rapid normalization of all blood tests. The present case underlines the complexity of IgG4-RD because of its variable clinical presentation. The diagnosis is challenging and should be carefully assessed for possible multi-organ involvement.
Background: The aim of our study was to evaluate the frequency and risk factors of clinical postoperative recurrence in Tunisian patients with Crohn’s disease (CD). Methods: Clinical data of 86 patients with CD who underwent ileocolonic resection at University Hospital of Sahloul in Tunisia were retrospectively reviewed. Continuous data are expressed as median (interquartile range), and categorical data as frequencies and percentages. Multivariate Cox proportional hazard regression analysis was conducted to identify the risk factors of postoperative clinical recurrence. Results: A total of 86 patients with CD were included in this study. During follow-up, 21 patients (24.4%) had clinical recurrence. The cumulative clinical recurrence rate was 9.3% at 1 year and 20.9% at 5 years. In univariate analysis, predictive factors of postoperative clinical recurrence were active preoperative smoking ( p = 0.008), ileal location of the disease ( p = 0.01), active CD [Crohn’s Disease Activity Index (CDAI) > 150] ( p = 0.04), duration of disease before first surgery <9.5 months ( p = 0.027), and limited resection margins (<2 cm) from macroscopically diseased bowel ( p = 0.005). In multivariate analysis, only smoking ( p = 0.012), duration of disease before first surgery <9.5 months ( p = 0.048), and limited resection margins (<2 cm) from macroscopically diseased bowel ( p = 0.046) were confirmed to be independent factors of clinical relapse. Conclusion: Smoking, duration of disease before first surgery <9.5 months, and limited resection margins (<2 cm) from macroscopically diseased bowel were independent risk factors for clinical recurrence. Based on these factors, patients could be stratified in order to guide postoperative therapeutic options.
We report a case of hematochezia with hemodynamic instability due to jejunal varices in a cirrhotic patient with no prior history of surgery. The patient was managed with coil embolization via the portal vein. After which, the patient did not present any hemorrhage recurrence and maintained a stable hemoglobin level.
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