Eldaabossi Safwat, Saad Mustafa, Alabdullah Mohammed, Awad Amgad, Alquraini Hussain, Moumneh Ghada, Mansour Ali
Almoosa Specialist Hospital, Al Ahsa, Saudi Arabia.
Al Azhar Faculty of Medicine, Al Azhar University, Cairo, Egypt.
Int Med Case Rep J. 2021 Dec 20;14:829-837. doi: 10.2147/IMCRJ.S340231. eCollection 2021.
Granulomatosis with polyangiitis (GPA) is an extremely rare autoimmune, necrotizing granulomatous disease of unknown etiology affecting small and medium-sized blood vessels. Chronic pulmonary aspergillosis (CPA) is a rare fungal infection with high morbidity and mortality that usually affects immunocompetent or mildly immunosuppressed patients with underlying respiratory disease. Antifungal agents (voriconazole, itraconazole) are the mainstay of therapy. Intravenous drug therapy (amphotericin B or an echinocandin), alone or in combination with azoles, is the last resort in special situations such as azole failure, resistance, or severe disease. Sometimes CPA and GPA coexist and are difficult to distinguish due to the nonspecific symptoms and similarity of clinical and radiological features, so a high degree of suspicion is required to make the correct diagnosis.
We reported that a 28-year-old man from Saudi Arabia was diagnosed with GPA. The patient had been complaining of cough, fatigue, polyarthralgia and red eyes for 40 days before he was admitted to our hospital. The diagnosis of GPA was confirmed by clinical and radiological examinations and a pathological report of a lung biopsy, and he was treated with immunosuppressive drugs. The patient's condition was complicated by chronic pulmonary aspergillosis and type 2 diabetes mellitus. Initial treatments included systemic glucocorticoids, methotrexate, followed by rituximab and voriconazole, finally intravenous cyclophosphamide and amphotericin B, with no complete remission. The thoracic surgical team postponed surgical debridement of the significant cavitary lung lesions until the active fungal infection could be brought under control.
The clinical and radiological features of GPA are similar to those of pulmonary tuberculosis, chronic pulmonary aspergillosis, and lung cancer. The lack of clear clinical symptoms of GPA requires a high degree of suspicion for early diagnosis. This case illustrates the dilemma of diagnosis and treatment of GPA and superimposed fungal infection. Secondary infection, particularly fungal infection, must be considered when GPA cannot be controlled with an immunosuppressant.
肉芽肿性多血管炎（GPA）是一种病因不明的极为罕见的自身免疫性坏死性肉芽肿疾病，累及中小血管。慢性肺曲霉病（CPA）是一种罕见的真菌感染，发病率和死亡率高，通常影响有基础呼吸系统疾病的免疫功能正常或轻度免疫抑制的患者。抗真菌药物（伏立康唑、伊曲康唑）是主要治疗手段。静脉药物治疗（两性霉素B或棘白菌素）单独或与唑类联合使用，是在唑类治疗失败、耐药或病情严重等特殊情况下的最后手段。有时CPA和GPA共存，由于症状不特异以及临床和影像学特征相似，难以区分，因此需要高度怀疑才能做出正确诊断。
我们报告了一名来自沙特阿拉伯的28岁男性被诊断为GPA。该患者在入院前40天一直抱怨咳嗽、疲劳、多关节痛和眼红。通过临床和影像学检查以及肺活检的病理报告确诊为GPA，并接受免疫抑制药物治疗。患者的病情并发慢性肺曲霉病和2型糖尿病。初始治疗包括全身糖皮质激素、甲氨蝶呤，随后是利妥昔单抗和伏立康唑，最后是静脉注射环磷酰胺和两性霉素B，但未完全缓解。胸外科团队推迟了对明显空洞性肺病变的手术清创，直到活动性真菌感染得到控制。
GPA的临床和影像学特征与肺结核、慢性肺曲霉病和肺癌相似。GPA缺乏明确的临床症状，需要高度怀疑以早期诊断。本病例说明了GPA合并真菌感染的诊断和治疗困境。当GPA不能用免疫抑制剂控制时，必须考虑继发感染，尤其是真菌感染。
