Mei Chun-Xia, Yue Guo-Long, Feng Xia, Wu Hai-Qiao, Li Jiong
Department of Respiratory, Chongqing Hospital of Traditional Chinese Medicine, Chongqing, China.
Front Immunol. 2024 Dec 20;15:1432508. doi: 10.3389/fimmu.2024.1432508. eCollection 2024.
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated chronic fibro-inflammatory condition, that can involve multiple systems. Immunoglobulin G4-related respiratory disease (IgG4-RRD) is relatively rare, with non-specific clinical symptoms. Hemoptysis is a rare clinical symptom of IgG4-RRD, and cases of massive hemoptysis in adults have not been reported. We present here a rare case of massive hemoptysis caused by IgG4-RRD in adults and review relevant literature. An 84-year-old female presented with recurrent cough and blood-streaked sputum, progressing to massive hemoptysis. Her chest CT showed patchy lesions in the lungs, initially misdiagnosed as a tumor. Ultimately, a biopsy confirmed the diagnosis of IgG4-RRD. The patient was treated with prednisone combined with leflunomide, which controlled her condition and maintained remission. However, after 13 months without hemoptysis, she experienced intermittent hemoptysis followed by a massive episode. Increasing the prednisone dose and continuing leflunomide treatment controlled the condition once again, with no recurrence in the subsequent year of follow-up. In patients with IgG4-RRD, particularly those with hemoptysis, it is essential to remain vigilant for massive hemoptysis. Similarly, in patients with lung patch lesions and no evidence of a tumor on biopsy, IgG4-RRD should not be overlooked. Early diagnosis and timely treatment can improve the patient's clinical prognosis.
免疫球蛋白G4相关疾病(IgG4-RD)是一种免疫介导的慢性纤维炎症性疾病,可累及多个系统。免疫球蛋白G4相关呼吸道疾病(IgG4-RRD)相对罕见,临床症状不具特异性。咯血是IgG4-RRD罕见的临床症状,成人大量咯血的病例尚未见报道。我们在此报告一例成人IgG4-RRD导致大量咯血的罕见病例并复习相关文献。一名84岁女性出现反复咳嗽和痰中带血,进而发展为大量咯血。她的胸部CT显示肺部有斑片状病变,最初被误诊为肿瘤。最终,活检确诊为IgG4-RRD。患者接受泼尼松联合来氟米特治疗,病情得到控制并维持缓解。然而,在无咯血13个月后,她出现间歇性咯血,随后发生大量咯血。增加泼尼松剂量并继续来氟米特治疗再次控制了病情,在随后的一年随访中未再复发。对于IgG4-RRD患者,尤其是有咯血症状的患者,必须警惕大量咯血。同样,对于肺部有斑片状病变且活检无肿瘤证据的患者,不应忽视IgG4-RRD。早期诊断和及时治疗可改善患者的临床预后。