Segraves Justin M, Iyer Vivek N
Department of Internal Medicine, Mayo Clinic, Rochester, MN, United States.
Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, United States.
Respir Med Case Rep. 2017 Mar 4;21:12-15. doi: 10.1016/j.rmcr.2017.03.001. eCollection 2017.
Microscopic polyangiitis is an uncommon systemic vasculitis of varying severity that is associated with myeloperoxidase (MPO) and perinuclear antineutrophil cytoplasmic (p-ANCA) antibodies. The most commonly affected organs are the lungs and kidneys. We report on a very unusual case of microscopic polyangiitis presenting with severe mesenteric ischemia in addition to diffuse alveolar hemorrhage and acute renal failure. The patient was initially diagnosed with acute pancreatitis at an outside facility given his severe abdominal pain and elevated pancreatic enzymes. Further investigations after transfer to our facility determined that the patient was actually suffering from a severe exacerbation of previously diagnosed microscopic polyangiitis. He quickly developed diffuse alveolar hemorrhage (DAH) necessitating intubation and acute kidney injury (AKI) requiring dialysis. He subsequently developed mesenteric ischemia and bowel necrosis resulting in emergent laparotomy and extensive small bowel resection. Physicians need to be aware that microscopic polyangiitis can very rarely present with severe involvement of the abdominal viscera and mesenteric vessels. Severe disease necessitates the use of high dose IV steroids, rituximab or cyclophosphamide, and plasma exchange (PLEX).
显微镜下多血管炎是一种罕见的系统性血管炎，严重程度不一，与髓过氧化物酶（MPO）和核周抗中性粒细胞胞浆抗体（p-ANCA）相关。最常受累的器官是肺和肾。我们报告了一例非常罕见的显微镜下多血管炎病例，除弥漫性肺泡出血和急性肾衰竭外，还伴有严重的肠系膜缺血。该患者最初因严重腹痛和胰腺酶升高，在外院被诊断为急性胰腺炎。转至我院后进一步检查发现，该患者实际上是先前诊断的显微镜下多血管炎严重加重。他很快出现弥漫性肺泡出血（DAH），需要插管，以及急性肾损伤（AKI），需要透析。随后，他发展为肠系膜缺血和肠坏死，导致急诊剖腹手术和广泛小肠切除。医生需要意识到，显微镜下多血管炎极少会严重累及腹部脏器和肠系膜血管。严重疾病需要使用大剂量静脉类固醇、利妥昔单抗或环磷酰胺，以及血浆置换（PLEX）。
