Abstract
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.
Highlights
In recent decades, immunoglobulin G4-related diseases (IgG4-RD) have attracted increasing attention
The current best diagnosis of IgG4-RD depends on the comprehensive and organ-specific diagnostic criteria of subjects suffering from IgG4-related pancreatitis, sclerosing cholangitis and kidney disease [17]
Patients, who could not respond to these comprehensive diagnostic (CD) criteria, could be diagnosed by applying organ-specific criteria, including those for IgG4-pancreatitis (AIP), IgG4-sclerosing cholangitis (IgG4-SC), and IgG4-kidneys related Disease (IgG4-RKD) (Table 2) [19,20,21]
Summary
Immunoglobulin G4-related diseases (IgG4-RD) have attracted increasing attention They are a group of immune-mediated diseases with a variable spectrum of clinical manifestations and common serological markers. This entity is characterized by tissue infiltration by IgG4+ plasma cells and elevated serum IgG4 levels. IgG4-RD is considered a rare disease and, currently, little is known about its epidemiology. Only the criteria for IgG4-related pancreatitis, cholangitis, nephritis, and glandular disease are available. These criteria are not applicable for the diagnosis of another organ involvement [2,3]. We present the following case in accordance with the CARE reporting checklist
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