Primary Antiphospholipid Syndrome-Associated Diffuse Alveolar Hemorrhage
Primary Antiphospholipid Syndrome-Associated Diffuse Alveolar Hemorrhage
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DOI：
10.1002/acr.22109
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发表时间：
2014-02-01
影响因子：
4.7
通讯作者：
Specks, Ulrich
中科院分区：
文献类型：
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作者：
Cartin-Ceba, Rodrigo;Peikert, Tobias;Specks, Ulrich
目的弥漫性肺泡出血（DAH）是原发性抗磷脂综合征（APS）的罕见并发症。我们的目的是描述单中心原发性 APS 相关 DAH 的临床特征、治疗和结果。方法我们对 Mayo Clinic 15 年来评估的所有原发性 APS 相关 DAH 成人进行回顾性评价。 DAH 被定义为双侧肺部浸润和支气管肺泡灌洗 (BAL) 液，记录逐渐血性回归和/或存在 >20% 的含铁血黄素巨噬细胞。排除其他原因导致 DAH 的患者。结果 确定了 18 名患者（中位年龄 43 岁）。 3 名患者的外科肺活检样本中存在毛细血管炎。 BAL 分类细胞计数显示主要是中性粒细胞。所有患者最初均接受糖皮质激素治疗。 8 例患者使用了环磷酰胺（CYC）； 3 名仅接受 CYC 治疗的患者和 1 名接受利妥昔单抗 (RTX) 联合治疗的患者实现了完全缓解。 9 名患者使用了 RTX； 2 名患者仅使用 RTX 获得缓解，而 3 名患者需要与 CYC 或吗替麦考酚酯 (MMF) 联合治疗。在接受 MMF、硫唑嘌呤或血浆置换单一治疗时，没有患者获得完全缓解。 5例患者接受静脉丙种球蛋白治疗；没有患者能够控制疾病。 6 名患者死亡，均因与未控制的 DAH 或其治疗相关的并发症而死亡。结论我们提出了文献中报道的最大的原发性 APS 相关 DAH 病例系列。 DAH 的预后非常差，治疗选择也很有限。 CYC 或 RTX 的免疫抑制与诱导缓解的可能性最高有关，应尽早考虑。
ObjectiveDiffuse alveolar hemorrhage (DAH) is an uncommon complication of primary antiphospholipid syndrome (APS). We aimed to describe the clinical characteristics, treatment, and outcomes of primary APS-associated DAH in a single center.MethodsWe conducted a retrospective review of all adults with primary APS-associated DAH evaluated at Mayo Clinic over a 15-year period. DAH was defined as bilateral pulmonary infiltrates and bronchoalveolar lavage (BAL) fluid documenting progressively bloody returns and/or the presence of >20% hemosiderin-laden macrophages. Patients with other causes of DAH were excluded.ResultsEighteen patients were identified (median age 43 years). Capillaritis was present in surgical lung biopsy samples of 3 patients. BAL differential cell counts revealed predominantly neutrophils. All patients were treated initially with glucocorticoids. Cyclophosphamide (CYC) was used in 8 patients; complete remission was achieved in 3 patients treated with CYC alone and in 1 patient receiving combination therapy with rituximab (RTX). RTX was used in 9 patients; 2 patients achieved remission with RTX alone, whereas 3 patients required combination therapy with CYC or mycophenolate mofetil (MMF). No patient achieved complete remission while receiving single therapy with MMF, azathioprine, or plasma exchange. Intravenous gamma globulin therapy was administered in 5 patients; no patient achieved control of the disease. Six patients died, all because of complications related to uncontrolled DAH or its therapy.ConclusionWe present the largest case series of primary APS-associated DAH reported in the literature. DAH carries a very poor prognosis and therapeutic options are limited. Immunosuppression with either CYC or RTX is associated with the highest likelihood of remission induction and should be considered early.