Cigarrán Secundino, Castro María Jesús, Pousa Montserrat, Paredes Sonia, Bernardo Hugo, Porteiro Montserrat
Department of Nephrology, Hospital da Costa, Burela, Spain.
Ther Apher Dial. 2010 Jun;14(3):368-72. doi: 10.1111/j.1744-9987.2010.00812.x.
Unlike Goodpasture's syndrome with diffuse alveolar hemorrhage (DAH), there are scarce reports on the use of plasmapheresis for patients with a recurrence of DAH associated with antineutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis (AAV) on hemodialysis. We report a case of a relapse of perinuclear-AAV with DAH, five months after starting hemodialysis. The patient received apheresis and induction immunosuppressive therapy, added to a short course of daily hemodialysis treatments. The DAH resolved with seven apheresis procedures and there were no adverse effects. We suggest that patients on hemodialysis with a relapse of AAV and DAH would benefit from the prompt initiation of apheresis in combination with aggressive immunosuppressive therapy. Pulmonary hemorrhage is not included in the current guidelines for therapeutic apheresis; therefore, we report this case and, if warranted, propose this condition to be included in the guidelines.
与伴有弥漫性肺泡出血(DAH)的古德帕斯丘综合征不同,关于血浆置换用于血液透析的抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎(AAV)伴DAH复发患者的报道很少。我们报告了1例血液透析开始5个月后核周型AAV伴DAH复发的病例。患者接受了单采和诱导免疫抑制治疗,并辅以短期每日血液透析治疗。经过7次单采程序,DAH得到缓解,且未出现不良反应。我们认为,血液透析的AAV和DAH复发患者将从及时开始的单采联合积极免疫抑制治疗中获益。肺出血未纳入当前治疗性单采指南;因此,我们报告此病例,并在必要时建议将此情况纳入指南。