Thrombotic microangiopathy in a patient with eosinophilic granulomatosis with polyangiitis: case-based review.
ANCA-associated vasculitis
Atypical haemolytic uremic syndrome
Complement-mediated diseases
Eosinophilic granulomatosis with polyangiitis
Thrombotic microangiopathy
Journal
Rheumatology international
ISSN: 1437-160X
Titre abrégé: Rheumatol Int
Pays: Germany
ID NLM: 8206885
Informations de publication
Date de publication:
02 2019
02 2019
Historique:
received:
25
07
2018
accepted:
12
12
2018
pubmed:
17
12
2018
medline:
31
12
2019
entrez:
17
12
2018
Statut:
ppublish
Résumé
The correct diagnosis, classification and therapeutic management of thrombotic microangiopathies (TMA) continue to be a challenge for the clinician. We report a rare case of eosinophilic granulomatosis with polyangiitis (EGPA) as a trigger for complement-mediated TMA in a 57-year-old man who was successfully treated with corticoids, cyclophosphamide and therapeutic plasma exchange. Additionally, we review few other cases reported in the literature and the pathophysiological pathway of association between TMA and EGPA. We found that the mutual relationships between the inflammation triggered by vasculitis, the exacerbated complement activation, together with hypereosinophilia and endothelial damage seem to be the key in explaining the connection between both entities. We suggest that an understanding of the multi-causal nature of TMAs is crucial for the correct diagnosis and treatment of these patients.
Identifiants
pubmed: 30554307
doi: 10.1007/s00296-018-4228-5
pii: 10.1007/s00296-018-4228-5
doi:
Types de publication
Case Reports
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
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