2nd Department of Internal Medicine, Ivan Horbachevsky Ternopil National Medical University, Majdan Voli, 1, Ternopil, 46001, Ukraine.
Department of Morphology, Clinical Pathology and Forensic Medicine, Shupyk National Healthcare University of Ukraine, Kyiv, Ukraine.
Rheumatol Int. 2024 Jul;44(7):1369-1379. doi: 10.1007/s00296-024-05586-5. Epub 2024 Apr 17.
Granulomatosis with polyangiitis is a systemic vasculitis. While the classic triad typically comprises otorhinolaryngologic, pulmonary, and renal manifestations, it is essential to recognize that granulomatosis with polyangiitis can affect any organ. Furthermore, reports have documented less common sites of involvement, such as the gastrointestinal tract. In this case-based review, we focus on a case of granulomatosis with polyangiitis presenting with intestinal perforation and the added challenge of concurrent pancytopenia.A 25-year-old female was diagnosed with granulomatosis with polyangiitis, with her clinical course progressing from joint pain to severe multi-organ involvement, including gastrointestinal complications. Treatment challenges emerged with the development of pancytopenia. While this may not directly result from granulomatosis with polyangiitis, it introduced an additional layer of complexity and delayed the induction of remission with immunosuppressants. Despite initial stabilization, an unexpected jejunal perforation occurred, requiring surgical intervention and subsequent postoperative care. The case underscores the complex nature of granulomatosis with polyangiitis and its potential complications. A literature search yielded discrete relevant cases in the context of our patient's intricate presentation, which has been summarized.We highlight the complexities in diagnosing and managing granulomatosis with polyangiitis-related complications, especially in uncommon presentations, and emphasize the importance of a personalized approach to patient care in these circumstances.
肉芽肿性多血管炎是一种系统性血管炎。虽然经典三联征通常包括耳鼻喉、肺部和肾脏表现，但必须认识到，肉芽肿性多血管炎可影响任何器官。此外，有报道记录了较少见的受累部位，如胃肠道。在本次基于病例的回顾中，我们重点关注一例以肠穿孔为表现并伴有同时发生全血细胞减少症的罕见病例。
一位 25 岁女性被诊断为肉芽肿性多血管炎，其临床病程从关节痛进展为严重多器官受累，包括胃肠道并发症。全血细胞减少症的出现带来了治疗挑战。虽然这可能不是直接由肉芽肿性多血管炎引起的，但它增加了复杂性，并延迟了免疫抑制剂诱导缓解。尽管最初病情稳定，但意外发生了空肠穿孔，需要手术干预和随后的术后护理。
该病例突出了肉芽肿性多血管炎的复杂性及其潜在并发症。文献检索在我们患者复杂的表现背景下找到了一些离散的相关病例，这些病例已被总结。
我们强调了诊断和管理与肉芽肿性多血管炎相关并发症的复杂性，特别是在不常见的表现中，并强调在这些情况下对患者护理采取个性化方法的重要性。
