Immunoglobin G4-related disease (IgG4-RD) is a rare fibro-inflammatory disease of unknown etiology that has been recently recognized. It can cause fibro-inflammatory masses in almost every organ of the body and is associated with dense lymphoplasmacytic infiltration of IgG4-postitive plasma cells, storiform fibrosis and elevated levels of serum IgG4.1 IgG4-RD is a systemic disease that may…
What rheumatologists need to know about identifying and diagnosing immunoglobulin G4-related disease (IgG4-RD)
Immunoglobulin G4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ, and diagnosis is challenging. ACR/EULAR classification criteria for IgG4-RD have been developed and validated in a large cohort of patients. These criteria have performed well in tests and should contribute substantially to future clinical, epidemiologic and basic science investigations.
Sarcoidosis and IgG4-related disease (IgG4-RD) are both immune-mediated, often multi-organ, diseases of uncertain etiology capable of presenting with diverse clinical manifestations. Many clinical features are common to both conditions, including hypergammaglobulinemia, the ability to form inflammatory masses and involvement of the lymph nodes, lacrimal glands, salivary glands, meninges and lungs. Although imaging modalities, such as…