User:Cabculto/Acquired C1 esterase inhibitor deficiency
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Acquired Angioedema (consider title change)
[edit]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.[1]
Epidemiology
[edit]It is estimated that the worldwide incidence of AAE ranges from 1:10,000 to 1:150,000 persons.[2]
Causes
[edit]There are various disease comorbidities that are associated with acquired C1 esterase inhibitor deficiency, including:[3]
- 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.[4]
Clinical Presentation
[edit]Acquired angioedema presents as mucosal swelling of the upper respiratory tract and gastrointestinal tracts without pruritic skin lesions known as urticaria.[1]
Diagnosis
[edit]Acquired angioedema is diagnosed through supportive clinical examination in addition to necessary laboratory evaluation.[1]
The clinical history usually consists of recurrent angioedema episodes, symptom onset after age 30 years, and negative family history of hereditary angioedema.[1]
Laboratory evaluation usually consists of complement studies, genotyping, antibodies against C1INH.[1]
Complement studies are explained as follows:[1]
- 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.[1]
Treatment
[edit]Typical treatment consists of replacing the enzyme concentrate that is deficiencent in this disease process.[5]
References
[edit]- ^ a b c d e f g "UpToDate". www.uptodate.com. Retrieved 2021-11-06.
- ^ Nzeako, Ugochukwu C. (2001-11-12). "Hereditary Angioedema: A Broad Review for Clinicians". Archives of Internal Medicine. 161 (20): 2417. doi:10.1001/archinte.161.20.2417. ISSN 0003-9926.
- ^ Otani, Iris M.; Banerji, Aleena (2017-08). "Acquired C1 Inhibitor Deficiency". Immunology and Allergy Clinics of North America. 37 (3): 497–511. doi:10.1016/j.iac.2017.03.002.
{{cite journal}}: Check date values in:|date=(help) - ^ Gobert, Delphine; Paule, Romain; Ponard, Denise; Levy, Pierre; Frémeaux-Bacchi, Véronique; Bouillet, Laurence; Boccon-Gibod, Isabelle; Drouet, Christian; Gayet, Stéphane; Launay, David; Martin, Ludovic (2016-08). "A nationwide study of acquired C1-inhibitor deficiency in France: Characteristics and treatment responses in 92 patients". Medicine. 95 (33): e4363. doi:10.1097/MD.0000000000004363. ISSN 0025-7974. PMC 5370791. PMID 27537564.
{{cite journal}}: Check date values in:|date=(help)CS1 maint: PMC format (link) - ^ "UpToDate". www.uptodate.com. Retrieved 2021-11-06.