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Thrombotic microangiopathy in a patient with eosinophilic granulomatosis with polyangiitis: case-based review

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Abstract

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.

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Authors and Affiliations

  1. Department of Hematology and Hemotherapy, Virgen de las Nieves University Hospital, Avda. Fuerzas Armadas Nº 2, 18012, Granada, Spain

    Jon Badiola

  2. Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Virgen de las Nieves University Hospital, Granada, Spain

    Nuria Navarrete-Navarrete & José Mario Sabio

Authors
  1. Jon Badiola
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  2. Nuria Navarrete-Navarrete
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  3. José Mario Sabio
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All the authors have contributed to patient management and clinical procedures, have drafted and revised the manuscript and have approved the final version.

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Correspondence to Jon Badiola.

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JB, NNN and JMS declare that they have no conflict of interest.

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Informed consent was obtained from the patient to publish this case report.

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Badiola, J., Navarrete-Navarrete, N. & Sabio, J.M. Thrombotic microangiopathy in a patient with eosinophilic granulomatosis with polyangiitis: case-based review. Rheumatol Int 39, 359–365 (2019). https://doi.org/10.1007/s00296-018-4228-5

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  • DOI: https://doi.org/10.1007/s00296-018-4228-5

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