Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK.
Division of Rheumatology, Allergy and Immunology, Department of Internal Medicine, University of Toledo Medical Center, Toledo, OH, USA.
Rheumatology (Oxford). 2021 Dec 1;60(12):5509-5516. doi: 10.1093/rheumatology/keab278.
In patients with SSc, the coexistence of ANCA-associated vasculitis (SSc-AAV) has been reported to be associated with a severe disease course, including significant pulmonary and renal involvement. The presence of ANCA is not uncommon in patients with SSc, and therefore clinicians must maintain a high index of clinical suspicion about SSc-AAV. p-ANCA and anti-myeloperoxidase antibodies are the most common antibodies observed. Patients typically present with clinical features of microscopic polyangiitis or renal-limited vasculitis. There are multiple areas of potential interaction in the pathogenesis of SSc and AAV, which can exacerbate/compound vascular disease. In addition, similar patterns of major internal organ involvement (e.g. lung and kidneys) are seen in both conditions. We highlight a diagnostic approach to SSc-AAV and the paucity of data to inform management. As such, SSc-AAV is typically treated as per isolated AAV, which can potentially be hazardous in patients with SSc (e.g. due to the association between high-dose steroid and scleroderma renal crisis). We propose that this rare clinical entity warrants rigorous investigation, including definition of a therapeutic strategy to ameliorate the potentially devastating combination of pathologies in SSc-AAV.
在硬皮病患者中，已报道抗中性粒细胞胞浆抗体（ANCA）相关血管炎（SSc-AAV）的共存与严重的疾病过程相关，包括严重的肺和肾受累。在硬皮病患者中，ANCA 的存在并不罕见，因此临床医生必须对 SSc-AAV 保持高度的临床怀疑。p-ANCA 和抗髓过氧化物酶抗体是最常见的抗体。患者通常表现为显微镜下多血管炎或肾血管炎的临床特征。在 SSc 和 AAV 的发病机制中有多个潜在的相互作用区域，这可能会加重/复合血管疾病。此外，在这两种情况下都可以看到主要内脏器官受累（如肺和肾脏）的相似模式。我们强调了 SSc-AAV 的诊断方法和缺乏数据来指导管理。因此，SSc-AAV 通常按照孤立性 AAV 进行治疗，这在硬皮病患者中可能是危险的（例如，由于大剂量类固醇与硬皮病肾危象之间的关联）。我们提出，这种罕见的临床实体需要严格的研究，包括定义治疗策略以改善 SSc-AAV 中潜在破坏性的病理组合。
