Introduction
IgG4-related diseasesIt is a chronic, systemic disease closely related to IgG4 lymphocytes. It is characterized by elevated serum IgG4 levels and infiltration of various organs and tissues by IgG4-positive cells, including common lacrimal gland, pancreas and retroperitoneal space. The affected organs or tissues can cause diffuse enlargement due to chronic inflammation and fibrosis. This type of disease responds well to corticosteroid therapy.
An IgG4-related disease is a chronic, progressive autoimmune disease associated with an IgG4-related disease involving multiple organs or tissues, IgG4-related diseases. The disease has a wide range of clinical manifestations, including Mikuri's disease, autoimmune pancreatitis, interstitial nephritis and retroperitoneal fibrosis. Due to its unique clinical and pathological manifestations, IgG4-related disease-related diseases Gradually gained the attention and attention of the international community.
history
The discovery of IgG4-related disease-related diseases can be traced back to the study of autoimmune pancreatitis (AIP). Autoimmune pancreatitis was first proposed by Yoshida et al in 1995 and believes that the pathogenesis of the disease is related to autoimmune factors.
The relationship between AIP and IgG4-related disease-positive plasma cells was first introduced and proposed in 2001. AIP is often associated with other organs and tissues, such as salivary glands, bile ducts, and retroperitoneal tissues, and these organs and tissues (including AIP) Typical biopsy specimens show a large number of IgG4-related disease-positive lymphocyte infiltration.
Since then, with the deepening of research, Kamisawa was first introduced in 2003 to introduce the concept of IgG4 systemic sexually transmitted diseases, namely IgG4-related diseases, also known as lgG4 multi-organ lymphocytosis syndrome.
In 2010, the birth of the disease was announced in the journal AutoimmunRev.
Cause
The pathogenesis of IgG4-related diseases is unclear, but its characteristic pathological changes are extensive IgG4-positive lymphocyte infiltration in tissues and multiple organs, leading to probation and fibrosis. In the AIP study, CD4+ and CD8+ cells may be involved in the disease. In addition, in the study of lgG4-related lung disease, long-term exposure to asbestos was found to be a predisposing factor for the disease.
Characteristics
Clinical spectrum of IgG4-positive multi-organ lymphoproliferative syndrome
1. One or more organs or tissues have increased swelling and resemble tumorigenicity;
2. The proliferation of IgG4-positive lymphocytes leads to proliferative infiltration and hardening of lymphocytes;
3. The level of serum IgG4 cells was significantly increased (>1350 mg/L), and IgG4-positive lymphocytes infiltrated in tissues (IgG4-positive lymphocytes accounted for more than 50% of lymphocytes);
4. Good response to glucocorticoid therapy.
Clinical manifestation
Different IgG4-related disease profiles can have different clinical manifestations:
1. Mikulize disease is characterized by bilateral symmetrical salivary gland swelling, which may be associated with dry eyes, dry mouth and joint swelling and pain. It is common in middle-aged and elderly men and can coexist with other organ diseases, anti-SSA and anti-SSB antibodies. Mostly negative;
2, AIP often appears in middle-aged men, can be manifested as acute pancreatitis or chronic pancreatitis, imaging features of the intestinal pancreas enlargement;
3, retroperitoneal fibrosis is characterized by retroperitoneal fibrous tissue hyperplasia, which can lead to abdominal organ obstruction, abdominal aorta inflammation and other symptoms;
4, pituitary inflammation is mainly characterized by endocrine regulation disorder, imaging findings of diffuse swelling of the pituitary.
treatment
There is currently no uniform standard of treatment for IgG4-related diseases, but it has been found to be generally sensitive to glucocorticoid therapy during treatment.
Clinical treatment found that IgG4-related diseases responded well to hormone therapy, organ tissue swelling subsided significantly, serum IgG4 decreased, clinical signs and symptoms significantly improved; but long-term hormonal therapy adverse reactions will increase, and more recurrence after stopping the drug.
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