Koritala Thoyaja, Mene-Afejuku Tuoyo O, Schaefer Matthew, Dondapati Lavanya, Pleshkova Yelena, Yasmin Farah, Mushtaq Hisham Ahmed, Khedr Anwar, Adhikari Ramesh, Al Mutair Abbas, Alhumaid Saad, Rabaan Ali A, Al-Tawfiq Jaffar A, Jain Nitesh K, Khan Syed Anjum, Kashyap Rahul, Surani Salim
Hospital Medicine, Mayo Clinic, Mankato, USA.
Radiology, Mayo Clinic, Mankato, USA.
Cureus. 2021 Nov 22;13(11):e19814. doi: 10.7759/cureus.19814. eCollection 2021 Nov.
Granulomatosis with polyangiitis (GPA), formerly named Wegner's granulomatosis is an antineutrophilic cytoplasmic antibody (ANCA) associated vasculitis of the small vessels. GPA can affect several organ systems even though predominantly affects respiratory and renal systems. Pathogenesis is initiated by activation of the immune system to produce ANCA, Cytoplasmic (C-ANCA) antibody, which thereby leads to widespread necrosis and granulomatous inflammation. Multisystem involvement with varied symptomatology makes GPA diagnosis more challenging. Early diagnosis and management are vital and can alter the prognosis of the disease. We present a literature review and a clinical scenario of a 26-year-old male with a history of chronic sinusitis, testicular carcinoma in remission, recent onset of worsening cough, epistaxis, hoarseness of voice, weight loss, and dark-colored urine. Workup revealed high titers of C-ANCA, C-reactive protein, procalcitonin, CT chest evidence of mass-like consolidation, and bronchoscopy findings of friable tissue that was not amenable for biopsy. Methylprednisolone and rituximab (RTX) were administered, which resulted in marked clinical improvement. Therefore, a keen eye for details is necessary to diagnose GPA early, which can improve disease outcomes dramatically.
肉芽肿性多血管炎（GPA），以前称为韦格纳肉芽肿，是一种与抗中性粒细胞胞浆抗体（ANCA）相关的小血管血管炎。GPA可累及多个器官系统，尽管主要影响呼吸系统和肾脏系统。发病机制始于免疫系统激活产生ANCA，即胞浆型（C-ANCA）抗体，从而导致广泛的坏死和肉芽肿性炎症。多系统受累及多种症状表现使得GPA的诊断更具挑战性。早期诊断和治疗至关重要，可改变疾病的预后。我们呈现一篇文献综述以及一名26岁男性的临床病例，该患者有慢性鼻窦炎病史、睾丸癌缓解期、近期咳嗽加重、鼻出血、声音嘶哑、体重减轻及深色尿。检查发现C-ANCA、C反应蛋白、降钙素原高滴度，胸部CT有团块状实变证据，支气管镜检查发现组织脆弱不宜活检。给予甲泼尼龙和利妥昔单抗（RTX）治疗后，临床症状明显改善。因此，早期诊断GPA需要敏锐关注细节，这可显著改善疾病预后。
