Joseph Adrien, Eloit Martin, Azoulay Elie, Kaplanski Gilles, Provot François, Presne Claire, Wynckel Alain, Grangé Steven, Rondeau Éric, Pène Frédéric, Delmas Yahsou, Lautrette Alexandre, Barbet Christelle, Mousson Christiane, Coindre Jean-Philippe, Perez Pierre, Jamme Matthieu, Augusto Jean-François, Poullin Pascale, Jacobs Frédéric, El Karoui Khalil, Vigneau Cécile, Ulrich Marc, Kanouni Tarik, Le Quintrec Moglie, Hamidou Mohamed, Ville Simon, Charvet-Rumpler Anne, Ojeda-Uribe Mario, Godmer Pascal, Fremeaux-Bacchi Véronique, Veyradier Agnès, Halimi Jean-Michel, Coppo Paul
INSERM, U1138, INSERM, Équipe 11 labellisée Ligue Nationale Contre le Cancer, Centre de Recherche des Cordeliers Paris France.
Centre de Référence des Microangiopathies Thrombotiques (CNR-MAT) AP-HP Paris France.
Res Pract Thromb Haemost. 2022 May 18;6(4):e12702. doi: 10.1002/rth2.12702. eCollection 2022 May.
The prevalence, prognostic role, and diagnostic value of blood pressure in immune-mediated thrombotic thrombocytopenic purpura (iTTP) and other thrombotic microangiopathies (TMAs) remain unclear.
Using a national cohort of iTTP ( = 368), Shigatoxin-induced hemolytic uremic syndrome ( = 86), atypical hemolytic uremic syndrome ( = 84), and hypertension-related thrombotic microangiopathy ( = 25), we sought to compare the cohort's blood pressure profile to assess its impact on prognosis and diagnostic performances.
Patients with iTTP had lower blood pressure than patients with other TMAs, systolic (130 [interquartile range (IQR) 118-143] vs 161 [IQR 142-180] mmHg) and diastolic (76 [IQR 69-83] vs 92 [IQR 79-105] mmHg, both < 0.001). The best threshold for iTTP diagnosis corresponded to a systolic blood pressure <150 mmHg. iTTP patients presenting with hypertension had a significantly poorer survival (hazard ratio 1.80, 95% confidence interval 1.07-3.04), and this effect remained significant after multivariable adjustment (hazard ratio = 1.14, 95% confidence interval 1.00-1.30). Addition of a blood pressure criterion modestly improved the French clinical score to predict a severe A disintegrin and metalloprotease with thrombospondin type 1 deficiency in patients with an intermediate score (i.e., either platelet count <30 × 10/L or serum creatinine <200 µM).
Elevated blood pressure at admission affects the prognosis of iTTP patients and may help discriminate them from other TMA patients. Particular attention should be paid to blood pressure and its management in these patients.
血压在免疫介导的血栓性血小板减少性紫癜（iTTP）及其他血栓性微血管病（TMA）中的患病率、预后作用及诊断价值仍不明确。
利用一个全国性队列，其中包括368例iTTP患者、86例志贺毒素诱导的溶血尿毒症综合征患者、84例非典型溶血尿毒症综合征患者以及25例高血压相关血栓性微血管病患者，我们试图比较该队列的血压情况，以评估其对预后和诊断性能的影响。
iTTP患者的血压低于其他TMA患者，收缩压（130[四分位间距（IQR）118 - 143]对161[IQR 142 - 180]mmHg）和舒张压（76[IQR 69 - 83]对92[IQR 79 - 105]mmHg，均P < 0.001）。iTTP诊断的最佳阈值对应收缩压<150 mmHg。出现高血压的iTTP患者生存率显著更低（风险比1.80，95%置信区间1.07 - 3.04），多变量调整后该效应仍显著（风险比 = 1.14，95%置信区间1.00 - 1.30）。添加血压标准适度改善了法国临床评分，以预测中间评分患者（即血小板计数<30×10⁹/L或血清肌酐<200µM）中具有血小板反应蛋白-1缺乏的严重整合素和金属蛋白酶解聚素。
入院时血压升高影响iTTP患者的预后，并可能有助于将他们与其他TMA患者区分开来。应特别关注这些患者的血压及其管理。
