Atypical hemolytic uremic syndrome and complement blockade: established and emerging uses of complement inhibition
| Autor: | Ira B Kurtz, Andrae Vandross, Ramy M Hanna, Richard M. Burwick, Marina Barsoum |
|---|---|
| Rok vydání: | 2019 |
| Předmět: |
Thrombotic microangiopathy
business.industry 030232 urology & nephrology Diagnostic dilemma 030204 cardiovascular system & hematology urologic and male genital diseases medicine.disease Hemolysis Complement inhibition Complement system Blockade Complement (complexity) 03 medical and health sciences 0302 clinical medicine Complement Inactivating Agents Nephrology hemic and lymphatic diseases Immunology Atypical hemolytic uremic syndrome Internal Medicine Medicine Humans business Complement Activation Atypical Hemolytic Uremic Syndrome |
| Zdroj: | Current opinion in nephrology and hypertension. 28(3) |
| ISSN: | 1473-6543 |
| Popis: | Atypical hemolytic uremic syndrome (aHUS) is a diagnosis that has captured the interest of specialists across multiple fields. The hallmark features of aHUS are microangiopathic hemolysis and thrombocytopenia, which creates a diagnostic dilemma because of the occurrence of these findings in a wide variety of clinical disorders.In most of the instances, aHUS is a diagnosis of exclusion after ruling out causes such as Shigella toxin, acquired or genetic a disintegrin and metalloproteinase thrombospondin motif 13 deficiency (thrombotic thrombocytopenic purpura), and vitamin B12 deficiency. In the purest sense, aHUS is a genetic condition that is activated (or unmasked) by an environmental exposure. However, it is now evident that complement activation is a feature of many diseases. Variants in complement regulatory genes predispose to microangiopathic hemolysis in many rheumatologic, oncologic, and drug-induced vascular, obstetric, peritransplant, and infectious syndromes.Many 'hemolysis syndromes' overlap clinically with aHUS, and we review the literature on the treatment of these conditions with complement inhibition. New reports on the treatment of C3 glomerulopathy, Shiga toxin-related classic hemolytic uremic syndrome, and medication-related thrombotic microangiopathy will be reviewed as well. |
| Databáze: | OpenAIRE |
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