Icahn School of Medicine at Mount Sinai-Elmhurst Hospital, Queens, NY 11373, USA.
Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, USA.
Medicina (Kaunas). 2019 Jul 16;55(7):378. doi: 10.3390/medicina55070378.
Granulomatosis with polyangiitis (GPA) is a systemic granulomatous inflammatory disease characterized by small-to-medium vessel vasculitis due to Central Anti-Neutrophil Cytoplasmic Antibody (C-ANCA). GPA commonly involves the lungs and the kidneys. Among the pulmonary manifestations, diffuse alveolar hemorrhage (DHA) is a rare presentation of GPA that can present with hemoptysis leading to acute onset of anemia and hemodynamic instability. An active diagnostic workup including serologic titer of C-ANCA, imaging, intensive care, and aggressive immunosuppression is the key to DAH management. We report a case of DAH secondary to GPA that presented with hemoptysis leading to severe anemia, initially resuscitated symptomatically and started on plasmapheresis with pulse steroids and cyclophosphamide. Timely diagnosis and management led to a remarkable recovery of the pulmonary symptoms and imaging findings of DAH.
肉芽肿性多血管炎（GPA）是一种系统性肉芽肿性炎症性疾病，其特征是由于抗中性粒细胞胞浆抗体（C-ANCA）导致的中小血管血管炎。GPA 通常累及肺部和肾脏。在肺部表现中，弥漫性肺泡出血（DHA）是 GPA 的一种罕见表现，可表现为咯血，导致急性贫血和血流动力学不稳定。积极的诊断性检查包括 C-ANCA 的血清滴度、影像学、重症监护和强化免疫抑制是 DHA 管理的关键。我们报告了一例 GPA 继发的 DHA 病例，该病例表现为咯血导致严重贫血，最初通过对症复苏和血浆置换、脉冲类固醇和环磷酰胺进行治疗。及时的诊断和管理导致 DHA 的肺部症状和影像学表现显著恢复。
