User:Cabculto/Acquired C1 esterase inhibitor deficiency

= Acquired Angioedema (consider title change) = Acquired angioedema (AAE) is a condition that presents as mucosal swelling in parts of the body that can be life threatening. The acquired form results from a deficiency of the enzyme C1 esterase inhibitor, and abbreviated as C1INH-AAE. This form of angioedema is considered acquired due to its association with immune system disorders. Typically, acquired angioedema presents later in adulthood, in contrast to hereditary angioedema, which is a similar form of angioedema, that can present in early childhood.

Epidemiology
It is estimated that the worldwide incidence of AAE ranges from 1:10,000 to 1:150,000 persons.

Causes
There are various disease comorbidities that are associated with acquired C1 esterase inhibitor deficiency, including:


 * B-cell lymphoproliferative disorders, including non-Hodgkin lymphoma and monoclonal gammopathy of undetermined significance (MGUS)
 * Autoimmune disorders
 * Human immunodeficiency virus (HIV)
 * Multiple myeloma
 * Waldeonström macroglobulinemia
 * Systemic lupus erythematosus (SLE)
 * Churg-Strauss syndrome (also known as eosinophilic granulomatosis with polyangiitis)
 * Xanthomatosis
 * Hepatitis B infection
 * Idiopathic

Through retrospective case studies performed in France, Gobert et al. found non-Hodgkin lymphoma was associated with 48% of cases in a sample size of 92 cases of acquired angioedema.

Clinical Presentation
Acquired angioedema presents as mucosal swelling of the upper respiratory tract and gastrointestinal tracts without pruritic skin lesions known as urticaria.

Diagnosis
Acquired angioedema is diagnosed through supportive clinical examination in addition to necessary laboratory evaluation.

The clinical history usually consists of recurrent angioedema episodes, symptom onset after age 30 years, and negative family history of hereditary angioedema.

Laboratory evaluation usually consists of complement studies, genotyping, antibodies against C1INH.

Complement studies are explained as follows:


 * C4 level
 * C1 INH antigenic level
 * C1 function
 * C1q level

[Consider including a table]

Furthermore, additional laboratory testing can be done to consider other causes of swelling that present similar to angioedema.

Treatment
Typical treatment consists of replacing the enzyme concentrate that is deficiencent in this disease process.