selective IgA deficiency
Introduction
Introduction to selective IgA deficiency Selective IgA deficiency refers to serum IgA below 0.05 g/L, while IgG and IgM levels are normal; it is the most common type of immunodeficiency. The swept rate in the population is about 1/700. The disease is mostly sporadic, partial familial, and involves several generations. Epidemiological surveys of six ethnic groups in different regions of China show that there are ethnic and regional differences in the disease. A gene abnormality in the heavy chain coding structure may not be the main cause; in the early stage of B cell differentiation, IgA-producing B cell maturation arrest or IgA-specific inhibition of T cell activation may cause B cell maturation to be blocked may be the cause of the disease. basic knowledge The proportion of illness: 0.001% Susceptible people: no specific population Mode of infection: non-infectious Complications: renal damage in rheumatoid arthritis systemic lupus erythematosus thyroiditis anemia
Cause
The cause of selective IgA deficiency
Causes
Heavy chain coding structure gene abnormality: heavy chain coding structural gene abnormality may not be the main cause; early stage of B cell differentiation IgA production B cell maturation arrest or IgA-specific inhibition of T cell activation, resulting in blocked B cell maturation may be pathogenic the reason.
Genetics: Selective IgA deficiency refers to serum IgA below 0.05 g/L, while IgG and IgM levels are normal; it is the most common type of immunodeficiency. The swept rate in the population is about 1/700. The disease is mostly sporadic, partial familial, and involves several generations. Epidemiological surveys of six ethnic groups in different regions of China show that there are ethnic and regional differences in the disease.
Prevention
Selective IgA deficiency prevention
There is no effective preventive measure for this disease. Early diagnosis and early treatment are the key to the prevention and treatment of this disease.
Complication
Selective IgA deficiency complications Complications Rheumatoid arthritis kidney damage Systemic lupus erythematosus Thyroiditis Anemia
Common complications of this disease are: rheumatoid arthritis, systemic lupus erythematosus, thyroiditis and pernicious anemia.
Symptom
Symptoms of selective IgA deficiency common symptoms diarrhea gastrointestinal symptoms pernicious anemia malabsorption syndrome
Most patients are asymptomatic, occasionally found at the time of examination, other cases often have different degrees of sinus and respiratory infections; if bronchial asthma is generally more serious, some patients have gastrointestinal symptoms, such as chronic diarrhea, malabsorption syndrome and intestinal tract Atrophy of the villi, infection of Giardia lamblia is rare, because the gastrointestinal tract and respiratory tract do not secrete IgA, prone to allergic reactions, in addition, rheumatoid arthritis, systemic lupus erythematosus, thyroiditis and pernicious anemia The incidence of disease has increased.
Examine
Examination of selective IgA deficiency
Serum and exocrine IgA levels are significantly reduced; a small number of patients with IgE and IgG2 are also reduced, because IgG2 can produce more antibodies to certain polysaccharide antigens, these patients are prone to recurrent sinus and lung infections, and even lead to obstructive pulmonary disease However, the number of IgA-producing B cells in the circulation is not low. After exposure to plasma IgA or gamma globulin, patients with this disease will develop anti-IgA antibodies, and then cause allergic reactions such as retransfusion or exposure to globulin. In addition, patients with this disease It also produces antibodies against thyroglobulin, gastric parietal cells, smooth muscle, collagen and food antigens. The positive rate of anti-bovine serum albumin antibodies is also increased. If IgA is detected by bovine antiserum, IgA deficiency will be masked. Therefore, other antibiotics should be used instead. Serum (such as rabbit) is more reliable.
Diagnosis
Diagnosis and identification of selective IgA deficiency
The disease can be diagnosed according to clinical and examination, without identification.
