What is IgG4 systemic disease?
IgG4-related disease is a fibro-inflammatory condition that can affect nearly any organ system. Common presentations include major salivary and lacrimal gland enlargement, orbital disease, autoimmune pancreatitis, retroperitoneal fibrosis and tubulointerstitial nephritis.
Which are most often affected in the IgG4-related disease?
IgG4-related disease (IgG4-RD) is a chronic, immune-mediated disorder that often manifests with multiorgan involvement and tumor-like masses most often affecting the pancreas, bile ducts, lacrimal glands, orbital tissues, salivary glands, lungs, kidneys, retroperitoneal tissues, aorta, meninges, and the thyroid gland.
Is IgG4 disease a vasculitis?
Conclusions: IgG4-RD is a distinctive, unique, and treatable cause of large-vessel vasculitis. It can also involve blood vessels secondary to perivascular tumefactive lesions. The most common manifestation of IgG4-related vasculitis is aortitis with aneurysm formation.
Is IgG4-related disease autoimmune?
IgG4-related disease is an immune-mediated condition, meaning that it involves the occurrence of disease in organs as the result of a dysregulated immune system. Increasing evidence suggests that IgG4-RD is an autoimmune condition, much like rheumatoid arthritis and systemic lupus erythematosus.
Is IgG4 an autoimmune disease?
What does IgG4 do to your body?
It has various normal functions in the body, but in IgG4-related disease, immune cells that produce IgG4, along with other related cells, accumulate abnormally in certain organs and damage them. The affected organs can enlarge and may eventually fill with scar tissue (fibrosis) and the damage can be permanent.
Is IgG4 serious?
IgG4-RD can cause many different symptoms and may affect one or several organs at the same time or at different times. Although symptoms may sometimes be mild, it can also cause severe organ damage and even death if not treated.
What kind of doctor treats IgG4-related disease?
The rheumatologist should play a leading role in the diagnosis, management and treatment of all patients with IgG4-RD. Rheumatologists are expert in the assessment of multi-organ diseases and often have a broader approach to systemic illnesses than specialists who focus on single organs.
What triggers IgG4?
IL-4 induces macrophages to produce high levels of IL-10. In turn IL-21, produced by Th2 and follicular T helper cells, is involved in the formation of germinal centers within lymphoid nodules and stimulates IgG4 production and infiltration of the affected tissues by IgG4-positive cells.
How serious is lgg4 disease?
Prognosis for IgG4-Related Disease Like most immune-mediated conditions, there is no cure, but IgG4-RD is very treatable. Much of the organ damage in this disease results from delays in diagnosis and insidious development of organ involvement and damage following treatment-induced remission.