Consensus regarding diagnosis and management of atypical hemolytic uremic syndrome

Hajeong Lee, Eunjeong Kang, Hee Gyung Kang, Young Hoon Kim, Jin Seok Kim, Hee Jin Kim, Kyung Chul Moon, Tae Hyun Ban, Se Won Oh, Sang Kyung Jo, Heeyeon Cho, Bum Soon Choi, Junshik Hong, Hae Il Cheong, Doyeun Oh

    Research output: Contribution to journalArticlepeer-review

    6 Citations (Scopus)

    Abstract

    Thrombotic microangiopathy (TMA) is defined by specific clinical characteristics, including microangiopathic hemolytic anemia, thrombocytopenia, and pathologic evidence of endothelial cell damage, as well as the resulting ischemic end-organ injuries. A variety of clinical scenarios have features of TMA, including infection, pregnancy, malignancy, autoimmune disease, and medications. These overlapping manifestations hamper differential diagnosis of the underlying pathogenesis, despite recent advances in understanding the mechanisms of several types of TMA syndrome. Atypical hemolytic uremic syndrome (aHUS) is caused by a genetic or acquired defect in regulation of the alternative complement pathway. It is important to consider the possibility of aHUS in all patients who exhibit TMA with triggering conditions because of the incomplete genetic penetrance of aHUS. Therapeutic strategies for aHUS are based on functional restoration of the complement system. Eculizumab, a monoclonal antibody against the terminal complement component 5 inhibitor, yields good outcomes that include prevention of organ damage and premature death. However, there remain unresolved challenges in terms of treatment duration, cost, and infectious complications. A consensus regarding diagnosis and management of TMA syndrome would enhance understanding of the disease and enable treatment decision-making.

    Original languageEnglish
    Pages (from-to)25-40
    Number of pages16
    JournalKorean Journal of Internal Medicine
    Volume35
    Issue number1
    DOIs
    Publication statusPublished - 2020

    Keywords

    • Alternative
    • Atypical hemolytic uremic syndrome
    • Complement pathway
    • Diagnosis
    • Differential
    • Eculizumab
    • Thrombotic microangiopathies

    ASJC Scopus subject areas

    • Internal Medicine

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