Hypocomplementemia Definition, Symptoms, Causes, Treatment
As the name indicates the condition, hypo means deficiency and complementemia is about complement system. Whenever there is deficiency or decreased amount of complement is observed, it is known as hypocomplementemia. In this condition, a part or component of complement is reduced in amount. This condition is associated with the immune complex diseases. Hypocomplementemia is an immunodeficiency condition which occurs due to sub-optimal functioning of complement system or absence of complement system.
There are redundancies in the immune system that is the reason various complement disorders are left undiagnosed. Only less than 10 percent are identified on time. There is a term secondary complement disorder, which occurs secondary to any other medical condition and does not occur due genetic cause. The mechanisms which may lead hypocomplementemia are as follows:
In case of c2, there are 5 mutations expected in the genes. The functions of immune system decreases and the opportunities of infection increases. Mostly mutations are responsible for deleting 28 DNA from genes.In case of c3, there are total 17 mutations expected. Mutation in c3 decreases the immune system’s ability and prevent the formation of c3 protein.Mutation in c4 is associated with systemic lupus erythematous, a protein involving in alzheimer’s disease and Abeta42.
When there is deficiency or low level of any part or component of complement system, the following symptoms appear in general:
All these diseases and infections occur due to weak immune system. Missing part or component of complement system weakens the immune system and due to this, any kind of infection may occur and it becomes difficult to treat these diseases and infections.
There are two main causes behind hypocomplementemia. It may be genetic or acquired. Most of the deficiencies of complement are autosomal recessive. Properidin deficiency show x linked inheritance. The MBL deficiency can be of both. In case of inherited hypocomplementemia, the c4 levels are low but c1 levels are normal and causes c1 inhibitor deficiency or hereditary angioedema. Due to properdin deficiency, Neisseria infections may occur. If the hypocomplementemia is acquired later in life, then it may occur in setting of infection of lining of heart, infections in bones and cryoglobunemia. Systemic lupus erythematous may occur due to low level of c3 and c4.
It is very important to manage this problem. To treat complement deficiency, immunosuppressive therapy is used which depends on the disease occurring. C1-INH concentrate is used in case to treat angioedema because angioedema occurs due to deficiency of C1-INH. Likewise pneumococcus and haemophilus are treated via immunization with the deficient complement. To treat the deficiency of C1-INH, epsilon aminocaproic acid is used. The side effects of intravascular thrombosis should be judged before starting this treatment. As mentioned above, the respective deficiencies are resolved with the concentrates and the symptoms are also get treated.
There are redundancies in the immune system that is the reason various complement disorders are left undiagnosed. Only less than 10 percent are identified on time. There is a term secondary complement disorder, which occurs secondary to any other medical condition and does not occur due genetic cause. The mechanisms which may lead hypocomplementemia are as follows:
In case of c2, there are 5 mutations expected in the genes. The functions of immune system decreases and the opportunities of infection increases. Mostly mutations are responsible for deleting 28 DNA from genes.In case of c3, there are total 17 mutations expected. Mutation in c3 decreases the immune system’s ability and prevent the formation of c3 protein.Mutation in c4 is associated with systemic lupus erythematous, a protein involving in alzheimer’s disease and Abeta42.
Hypocomplementemia Symptoms
When there is deficiency or low level of any part or component of complement system, the following symptoms appear in general:
- Patient may experience infection in lungs.
- Auto immune disorders.
- Anaphylactoid purpura.
- Dermatomyositis
- The most common one are joint problems.
- The infections may show re-occurrence.
- Angioedema.
- Glomerulonephritis.
- Vasculitis
- Glomerulonephritis.
All these diseases and infections occur due to weak immune system. Missing part or component of complement system weakens the immune system and due to this, any kind of infection may occur and it becomes difficult to treat these diseases and infections.
Hypocomplementemia Causes
There are two main causes behind hypocomplementemia. It may be genetic or acquired. Most of the deficiencies of complement are autosomal recessive. Properidin deficiency show x linked inheritance. The MBL deficiency can be of both. In case of inherited hypocomplementemia, the c4 levels are low but c1 levels are normal and causes c1 inhibitor deficiency or hereditary angioedema. Due to properdin deficiency, Neisseria infections may occur. If the hypocomplementemia is acquired later in life, then it may occur in setting of infection of lining of heart, infections in bones and cryoglobunemia. Systemic lupus erythematous may occur due to low level of c3 and c4.
Hypocomplementemia Treatment
It is very important to manage this problem. To treat complement deficiency, immunosuppressive therapy is used which depends on the disease occurring. C1-INH concentrate is used in case to treat angioedema because angioedema occurs due to deficiency of C1-INH. Likewise pneumococcus and haemophilus are treated via immunization with the deficient complement. To treat the deficiency of C1-INH, epsilon aminocaproic acid is used. The side effects of intravascular thrombosis should be judged before starting this treatment. As mentioned above, the respective deficiencies are resolved with the concentrates and the symptoms are also get treated.
Hypocomplementemia Definition, Symptoms, Causes, Treatment
Reviewed by Simon Albert
on
February 10, 2019
Rating:
Reviewed by Simon Albert
on
February 10, 2019
Rating:
