Department of Hematology and Hemotherapy, Virgen de las Nieves University Hospital, Avda. Fuerzas Armadas Nº 2, 18012, Granada, Spain.
Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Virgen de las Nieves University Hospital, Granada, Spain.
Rheumatol Int. 2019 Feb;39(2):359-365. doi: 10.1007/s00296-018-4228-5. Epub 2018 Dec 15.
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.
血栓性微血管病(TMA)的正确诊断、分类和治疗管理仍然是临床医生面临的挑战。我们报告了一例罕见的嗜酸性肉芽肿性多血管炎(EGPA)病例,作为一名 57 岁男性补体介导 TMA 的诱因,他成功接受了皮质类固醇、环磷酰胺和治疗性血浆置换治疗。此外,我们还回顾了文献中报道的其他少数病例以及 TMA 和 EGPA 之间关联的病理生理途径。我们发现,血管炎引发的炎症、补体过度激活、嗜酸性粒细胞增多和内皮损伤之间的相互关系似乎是解释两者之间联系的关键。我们建议,了解 TMA 的多因性质对于这些患者的正确诊断和治疗至关重要。