IgG4-related Disease as Systemic Disease

Otologic Manifestation in IgG4-Related Systemic Disease

IgG4-related systemic disease, including autoimmune pancreatitis, is a multi-organ disorder characterized by elevated serum immunoglobulin G4 (IgG4) concentration and IgG4-positive plasma cell infiltration. We report a case of a 66-year-old woman with IgG4-related hearing disorder, presenting with fluctuating mixed hearing loss with middle ear effusion. The serum IgG4 level was elevated and pat...

Recurrent pleuritis: consider IgG4-related systemic disease.

Renal manifestations of IgG4-related systemic disease

While autoimmune pancreatitis (AIP) has been recognized since the first description by Sarles et al in 1961, its involvement of other organs has been recognized more recently. Now, some form of IgG4-related systemic disease (IgG4-RSD) has been described in nearly every organ system, including the liver, gallbladder, other gastrointestinal sites, kidney, salivary and lacrimal glands, orbit, brea...

Isolated Mass-Forming IgG4-Related Cholangitis as an Initial Clinical Presentation of Systemic IgG4-Related Disease

IgG4-related disease (IgG4-RD) may involve multiple organs. Although it usually presents as diffuse organ involvement, localized mass-forming lesions have been occasionally encountered in pancreas. However, the same pattern has been seldom reported in biliary tract. A 61-year-old male showed a hilar bile duct mass with multiple enlarged lymph nodes in imaging studies and he underwent trisection...

Ormond's Disease--IgG4-related Disease.

Ormond's disease is a relatively rare disease with unclear etiology, characterized by chronic periaortitis and retroperitoneal fibrosis. The inflammatory process affects the infrarenal part of the abdominal aorta and the iliac arteries, and the presence of infiltrates encasing the ureters and inferior vena cava. This disease is currently classed as an IgG4-related disease. In our review we anal...