Thompson Gwen, Klecka Mary, Roden Anja C, Specks Ulrich, Cartin-Ceba Rodrigo
Department of Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Division of Anatomic Pathology, Mayo Clinic, Rochester, Minnesota, USA.
Respirology. 2016 May;21(4):734-8. doi: 10.1111/resp.12738. Epub 2016 Jan 26.
Pulmonary capillaritis is generally secondary to a systemic autoimmune process. Little is known regarding other causes of pulmonary capillaritis, particularly isolated pulmonary capillaritis (IPC). IPC is defined as pulmonary capillaritis in the absence of evidence of a systemic disease. We aim to describe the aetiology of biopsy-proven pulmonary capillaritis to add to the existing knowledge on aetiologies of pulmonary capillaritis and provide a more detailed description of IPC cases.
A retrospective cohort of biopsy-proven pulmonary capillaritis was conducted at the Mayo Clinic, Rochester over a 17-year period. Histologic slides were re-reviewed by a lung pathologist. Medical records were reviewed to identify a possible aetiology of the pulmonary capillaritis. A subset analysis of patients identified with IPC was then performed.
Thirty-six cases of biopsy-proven pulmonary capillaritis were identified. The majority of cases were secondary to a systemic autoimmune disease, most commonly granulomatosis with polyangiitis. There were four cases of IPC in this cohort. Median follow-up was 116.5 months with no evidence of systemic disease development. No risk factors for IPC were identified. All patients presented sub-acutely with haemoptysis and diffuse alveolar haemorrhage with a delay in diagnosis. After initiation of immunosuppression, most patients obtained remission with a benign clinical course.
Pulmonary capillaritis is most commonly secondary to systemic autoimmune disorders, predominantly ANCA-associated vasculitis. IPC is a rare form of pulmonary capillaritis with very few cases described in the literature, the availability of effective treatment makes this rare disease important to recognize.
肺毛细血管炎通常继发于全身性自身免疫过程。关于肺毛细血管炎的其他病因，尤其是孤立性肺毛细血管炎（IPC），人们了解甚少。IPC被定义为在无全身性疾病证据情况下的肺毛细血管炎。我们旨在描述经活检证实的肺毛细血管炎的病因，以增加对肺毛细血管炎病因的现有认识，并提供更详细的IPC病例描述。
在罗切斯特的梅奥诊所对一组经活检证实的肺毛细血管炎患者进行了为期17年的回顾性队列研究。肺病理学家对组织学切片进行了重新评估。查阅病历以确定肺毛细血管炎的可能病因。然后对确诊为IPC的患者进行了亚组分析。
共确定了36例经活检证实的肺毛细血管炎病例。大多数病例继发于全身性自身免疫性疾病，最常见的是肉芽肿性多血管炎。该队列中有4例IPC病例。中位随访时间为116.5个月，无全身性疾病发展的证据。未发现IPC的危险因素。所有患者均亚急性起病，伴有咯血和弥漫性肺泡出血，诊断延迟。开始免疫抑制治疗后，大多数患者病情缓解，临床过程良好。
肺毛细血管炎最常见继发于全身性自身免疫性疾病，主要是抗中性粒细胞胞浆抗体相关血管炎。IPC是肺毛细血管炎的一种罕见形式，文献中描述的病例很少，有效的治疗方法使得识别这种罕见疾病很重要。
