Roshniben Patel, MD, Simon Go, MD, Akhila Mohan, MD, & Maria Pardi, MD | Issue: March 2022 |
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)
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…
SAN DIEGO—Fibrosis affects all organ systems, but isn’t always systemic sclerosis. Experts on less common forms discussed patient presentations, diagnosis and treatment at the 2017 ACR/ARHP Annual Meeting in San Diego on Nov. 6. Retroperitoneal Fibrosis Formerly called Ormond’s disease, retroperitoneal fibrosis (RPF) is usually an IgG4-related disease, but has some unique characteristics, said John…