| 张皓珍,张旭辉,武妍琳,等.原发性干燥综合征继发结节性肺淀粉样变性和囊性肺病 1例病例分析和文献复习[J].安徽医药,2026,30(8):1595-1599. |
| 原发性干燥综合征继发结节性肺淀粉样变性和囊性肺病 1例病例分析和文献复习 |
| Primary Sj.gren's syndrome complicated with nodular pulmonary amyloidosis and cystic lung disease:a case analysis and literature review |
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| DOI：10.3969/j.issn.1009-6469.2026.08.022 |
| 中文关键词: 干燥综合征 肺淀粉样变 结节性肺淀粉样变性 囊性肺病 病例报告 |
| 英文关键词: Sjogren's syndrome Pulmonary amyloidosis Nodular pulmonary amyloidosis Cystic pulmonary disease Case re-port |
| 基金项目:国家自然科学基金项目( 82260889)；甘肃省自然科学基金项目( 24JRRD002)；兰州市城关区科技计划项目( 2021SHFZ0026、2024JSCX0020) |
| 作者 | 单位 | E-mail | | 张皓珍 | 甘肃中医药大学附属医院肺病科,甘肃兰州,730000 | | | 张旭辉 | 甘肃中医药大学附属医院肺病科,甘肃兰州,730000
甘肃中医药大学第三附属医院肺病科,甘肃白银 730900 | zhangxuhui20221@163.com | | 武妍琳 | 甘肃中医药大学附属医院肺病科,甘肃兰州,730000 | | | 马泉 | 甘肃中医药大学附属医院肺病科,甘肃兰州,730000 | | | 张元 | 甘肃中医药大学附属医院肺病科,甘肃兰州,730000 | | | 李红梅 | 甘肃中医药大学附属医院肺病科,甘肃兰州,730000 | |
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| 中文摘要: |
| 目的报告 1例原发性干燥综合征( pSS)继发肺淀粉样变，帮助临床医生了解该疾病临床表现的复杂性，从而提高疾病的早期诊断率，优化治疗方案，改善病人预后。方法回顾性收集 2024年 6月甘肃中医药大学附属医院住院确诊的 pSS继发结节性肺淀粉样变性 1例临床资料，总结该病人临床表现、影像学特征、病理检查结果及中西医结合治疗方案，并结合相关文献梳理该病的疾病特点与诊疗难点。结果该例病人以进行性胸闷、气短为主要表现，胸部 CT提示双肺多发混杂密度结节影及囊泡形成，经肺组织病理活检及刚果红染色明确确诊为 pSS继发结节性肺淀粉样变性。给予病人羟氯喹联合院内制剂滋阴润燥丸规律治疗 6个月后，病人胸闷、气短等临床症状显著改善，复查胸部 CT提示肺部病灶稳定，无新发结节及囊泡样病变，病情控制良好。结论 pSS继发肺淀粉样变临床少见、症状隐匿且缺乏特异性，诊断难度较大。中西医结合方案可有效改善病人临床症状、稳定肺部病灶。临床需提高对 pSS肺部罕见并发症的认知，尽早完善影像学及病理检查实现早期诊断，及时干预以延缓病情进展，优化病人预后。 |
| 英文摘要: |
| Objective To systematically explore the clinical characteristics of pulmonary amyloidosis secondary to primary Sj.gren'ssyndrome (pSS) through typical case analysis and literature review, and to deepen clinicians' understanding of the complex clinicalmanifestations of this disease, so as to improve the early diagnostic rate, optimize therapeutic regimens and ameliorate patients' progno-sis.Methods Retrospective collection of clinical data of one hospitalized patient diagnosed with nodular pulmonary amyloidosis sec-ondary to pSS in the Affiliated Hospital of Gansu University of Chinese Medicine in June 2024 were collected. The clinical manifesta-tions, imaging findings, pathological results and integrated traditional Chinese and Western medicine treatment regimens of the patientwere summarized. Combined with relevant literature, the disease characteristics and diagnostic and therapeutic challenges of this condi-tion were further analyzed.Results The patient presented with progressive chest tightness and shortness of breath as the main symp-toms. Chest CT revealed multiple mixed-density nodules and cysts in both lungs. A definite diagnosis of nodular pulmonary amyloidosissecondary to pSS was confirmed by pulmonary biopsy and Congo red staining. After six months of standardized treatment with hydroxy-chloroquine combined with the hospital-prepared traditional Chinese medicine Ziyin Runzao pill, the patient's symptoms including chest tightness and shortness of breath were markedly relieved. Re-examination by chest CT showed that the pulmonary lesions re-mained stable without newly developed nodules or cysts, indicating favorable disease control. Conclusions Pulmonary amyloidosis secondary to pSS is a rare condition with insidious and non-specific clinical symptoms, leading to great difficulties in diagnosis. The in-tegrated traditional Chinese and Western medicine therapy can effectively alleviate clinical symptoms and stabilize pulmonary lesions.Clinicians should raise awareness of this rare pulmonary complication of pSS. Early diagnosis via timely imaging and pathological exam-inations as well as prompt intervention are essential to slow disease progression and improve patients' prognosis. |
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