ArticleCEN case reports2017
Two cases of atypical hemolytic uremic syndrome (aHUS) and eosinophilic granulomatosis with polyangiitis (EGPA): a possible relationship.
Article in CEN case reports, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed.
- Vascular injury in glomerulopathies: the role of the endothelium.Frontiers in nephrology · 2024Review
- A fatal eosinophilic granulomatosis with polyangiitis case presenting intracerebral hemorrhage and thrombocytopenia.The journal of allergy and clinical immunology. Global · 2023Article
- Thrombotic microangiopathy in a patient with eosinophilic granulomatosis with polyangiitis: case-based review.Rheumatology international · 2019Review
- Eosinophilia and Kidney Disease: More than Just an Incidental Finding?Journal of clinical medicine · 2018Review
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Authors and funding
11 authors.
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Abstract
Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by hemolysis, thrombocytopenia, and renal failure. It is related to genetic mutations of the alternative complement pathway and is difficult to differentiate from other prothrombotic microangiopathies. Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss syndrome, CSS) is a systemic ANCA-associated vasculitis and a hypereosinophilic disorder where eosinophils seem to induce cell apoptosis and necrosis and therefore, vasculitis. Here, we report the case of two CSS patients with a genetic complement disorder consistent with aHUS diagnosis. Both patients showed histologic features that supported the diagnosis of CSS, and a genetic complement study confirmed the suspected aHUS diagnosis. In the case where eculizumab was administered, the global response was excellent. There is very limited understanding of the genetics and epidemiology of both, atypical HUS and EGPA, but considering our two patients we suggest that an etiopathogenic link exists among patients diagnosed with both entities.
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