Duarte Maria, Faria Luciana, Patronillo Catarina, da Costa Fernandes Sónia, Seara Vera
Internal Medicine Department, Centro Hospitalar Póvoa de Varzim - Vila do Conde, Portugal.
Eur J Case Rep Intern Med. 2021 Oct 29;8(10):002976. doi: 10.12890/2021_002976. eCollection 2021.
Good's syndrome is a rare adult-onset combined immunodeficiency. The association of hypogammaglobulinaemia with a history of recurrent infectious or autoimmune manifestations in a middle-aged patient with evidence of a mediastinal mass should lead to the clinical suspicion of Good's syndrome. The mortality rate associated with infectious complications is high. Thus, although it is rare, the disease should be diagnosed early so that proper treatment can be started. Thymectomy and immunoglobulin replacement are the main therapeutic strategies. We describe the case of a patient with a history of thymoma and recurrent respiratory infections, with a late diagnosis of Good's syndrome in the context of severe organizing pneumonia secondary to COVID-19.
Infection is the leading cause of mortality in patients with Good's syndrome.Early recognition of this rare disease and substitutive therapy with immunoglobulin may have an important impact on prognosis.Due to its rarity, data on Good's syndrome are scarce and its association with severe COVID-19 onset is still unclear.To the best of our knowledge, this is the fifth case report of COVID-19 in a patient with Good's syndrome, the third with a favourable outcome.
古德综合征是一种罕见的成人起病的联合免疫缺陷病。在一名有纵隔肿块证据的中年患者中,低丙种球蛋白血症与反复感染或自身免疫表现病史相关,应引起对古德综合征的临床怀疑。与感染并发症相关的死亡率很高。因此,尽管该病罕见,但应尽早诊断以便开始适当治疗。胸腺切除术和免疫球蛋白替代是主要的治疗策略。我们描述了一名有胸腺瘤病史和反复呼吸道感染的患者的病例,该患者在继发于新型冠状病毒肺炎的严重机化性肺炎背景下被晚期诊断为古德综合征。
感染是古德综合征患者死亡的主要原因。早期识别这种罕见疾病并使用免疫球蛋白进行替代治疗可能对预后有重要影响。由于其罕见性,关于古德综合征的数据稀少,其与新型冠状病毒肺炎严重发病的关联仍不清楚。据我们所知,这是第五例关于古德综合征患者感染新型冠状病毒肺炎的病例报告,第三例有良好结局。