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Clinical Diagnostic Criteria of IgG4-Related Sclerosing Cholangitis (IgG4-SC)

Sclerosing cholangitis (SC) is defined as a condition with progressive stenosis and destruction of the bile ducts due to diffuse inflammation and fibrosis and currently includes three categories: primary sclerosing cholangitis (PSC), secondary cholangitis, and IgG4-related sclerosing cholangitis (IgG4-SC). SC categories share similar clinical features, such as cholestasis. Patients with SC present with cholestatic symptoms, including jaundice and pruritus, and blood tests reveal elevation of cholestatic enzymes. Continue reading “Clinical Diagnostic Criteria of IgG4-Related Sclerosing Cholangitis (IgG4-SC)”

Diagnostic Criteria for IgG4-Related Kidney Disease (IgG4-RKD)

IgG4-related disease is characterized by a high level of serum IgG4 and dense infiltration of IgG4-positive plasma cells into multiple organs, with the kidney being one representative target. Although several sets of diagnostic criteria for autoimmune pancreatitis (AIP) are available and renal lesion is recognized as an extrapancreatic manifestation of AIP, it is difficult to differentiate IgG4-related tubulointerstitial nephritis (TIN) without
AIP from other types of TIN. Continue reading “Diagnostic Criteria for IgG4-Related Kidney Disease (IgG4-RKD)”

Classification Criteria for IgG4-Related Disease

Immunoglobulin G4-related disease is an immune mediated condition resulting in disease in various organs of the body such as the pancreas, kidneys, salivary glands, lung, liver, lymph nodes, biliary tract and orbits of the eyes. The disease is recognized by a characteristic pattern of pathological, serological or clinical features shared amongst the organs system that are involved. In some cases, IgG4-RD can mimic malignant, infectious or inflammatory disorders and therefore distinguishing the disease is crucial. Continue reading “Classification Criteria for IgG4-Related Disease”

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