Diegues Andreia, Tavares Joana, Sá Diogo, Oliveira João, Fernandes Diana, Santos Josefina, Rocha Guilherme
Internal Medicine Department, Unidade Local de Saúde do Nordeste, Portugal.
Nephrology Department, Centro Hospitalar Universitário do Porto, Portugal.
Eur J Case Rep Intern Med. 2021 Apr 22;8(4):002448. doi: 10.12890/2021_002448. eCollection 2021.
Granulomatosis with polyangiitis (GPA) is the most common antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We describe the case of a 38-year-old woman with relapsing GPA who presented with intracranial hypertension, followed by the appearance of cavitated lung nodules despite treatment with azathioprine. Clinical improvement and ANCA titre reduction were observed after rituximab treatment. We report a rare form of GPA relapse and highlight the challenge of following-up patients with GPA, in whom can be hard to distinguish relapse from the consequences of long-term immunosuppression.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare inflammatory disease with pauci-immune focal necrotising lesions that affect small and medium vessels. It has a wide clinical presentation, affecting mainly the upper and lower respiratory tract and kidneys.Granulomatosis with polyangiitis (GPA) is frequently associated with PR3-ANCA and is risk factor for relapse.Follow-up of ANCA titres, which may rise before the development of symptoms, is crucial for recurrence diagnosis. Titres can also be used to distinguish recurrence from the consequences of long-term immunosuppression.
肉芽肿性多血管炎（GPA）是最常见的抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）。我们描述了一名38岁复发性GPA女性患者的病例，该患者出现颅内高压，尽管接受了硫唑嘌呤治疗，但随后仍出现了肺空洞结节。利妥昔单抗治疗后观察到临床改善和ANCA滴度降低。我们报告了一种罕见的GPA复发形式，并强调了对GPA患者进行随访的挑战，在这类患者中，很难将复发与长期免疫抑制的后果区分开来。
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）是一种罕见的炎症性疾病，具有少免疫性局灶性坏死性病变，累及中小血管。其临床表现广泛，主要影响上下呼吸道和肾脏。肉芽肿性多血管炎（GPA）常与PR3-ANCA相关，是复发的危险因素。随访ANCA滴度对于复发诊断至关重要，其滴度可能在症状出现前升高。滴度还可用于区分复发与长期免疫抑制的后果。
