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…
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.
In 1888, Dr. Jan Mikulicz-Radecki reported a case of chronic, bilateral, painless enlargement of the salivary and lacrimal glands that appeared to be idiopathic.1 In subsequent years, other patients with these findings were reported, and the term Mikulicz syndrome was used to describe these cases. Although Mikulicz syndrome is now known to be associated with…