摘要目的:分析弥漫性肺骨化病(DPO)的临床、影像、病理和治疗特征，提高诊治水平。方法:本研究为病例系列研究，采用非随机抽样法收集2021年12月至2023年12月南京大学医学院附属鼓楼医院呼吸与危重症医学科收治的3例DPO患者和厦门大学附属中山医院普外科收治的1例DPO患者为研究对象。分析4例DPO患者的基线临床特征、实验室检查、影像学资料、病理结果、肺功能检查、治疗方法和预后情况。同时对万方数据库、中国知网和PubMed数据库中2024年10月31日前报道的所有DPO病例进行文献检索，最终纳入125例DPO患者进行回顾性分析。结果:4例DPO患者中男3例，女1例，年龄范围为19～38岁。2例患者有咳嗽、咳痰，其中1例伴活动后胸闷、气喘，2例患者无临床症状。4例患者的血钙、血磷均正常。4例患者的胸部CT均显示双肺弥漫性高密度小结节伴钙化、散在分支状细线影，胸膜轻度增厚；其中2例患者行全身骨显像，均显示双肺放射性摄取弥漫性增高，提示骨外摄取。4例患者均经肺活检病理确诊DPO，其中1例在胸腔镜下行外科肺活检，2例行经支气管冷冻肺活检，1例行经支气管镜肺活检术，病理检查均显示肺内可见成熟骨组织，部分可见脂肪组织。肺功能检查显示3例为轻度阻塞性通气功能障碍，1例为轻度限制性通气功能障碍，3例肺弥散功能轻度下降。4例患者均以基础疾病和合并症治疗为主。截至2024年7月，随访时间范围为8～32个月，4例患者病情均基本稳定。文献报道的125例DPO患者中，男106例，女19例，男女比例5.6∶1；发病年龄(55.4±18.8)岁，年龄范围为15～91岁。40例(32.0%)无临床症状，其他患者主要表现为呼吸系统症状。胸部影像学特征表现为双肺弥漫性高密度小结节伴钙化和细线影。125例患者均通过病理检查确诊DPO，其中树突型占79.2%(99/125)，结节型占16.8%(21/125)。结论:DPO起病隐匿、进展缓慢，男性多见，临床表现无特异性。其典型影像学特征为双肺弥漫性高密度小结节伴钙化和分支状细线影，全身骨显像可见双肺局部放射性摄取增加。经支气管冷冻肺活检是可取的微创肺活检方法，病理学检查发现成熟骨组织形成可明确诊断。DPO的治疗以控制基础疾病和合并症为主，多数患者进展缓慢，预后良好。

abstractsObjective:To analyze the clinical, imaging, histopathological and therapeutic features of diffuse pulmonary ossification (DPO), thus enhancing diagnostic accuracy and clinical management.Methods:This was a case series study.Three DPO patients from the Department of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital, the Affiliated Hospital of Nanjing University Medical School, and one DPO patient from the Department of General Surgery, Zhongshan Hospital, Xiamen University between December 2021 and December 2023 were enrolled by non-random sampling.Baseline characteristics, laboratory testing, imaging findings, pathological findings, lung function, treatment process and prognosis were analyzed.Articles reporting DPO cases were searched in Wanfang, China National Knowledge Infrastructure, and PubMed databases (up to October 31, 2024) using the search terms " diffuse pulmonary ossification" or " dendriform pulmonary ossification". Finally, 125 DPO cases were retrospectively analyzed.Results:Among the four DPO patients, there were 3 males, and 1 female with an age of 19-38 years.Two presented with cough and sputum (including one with dyspnea), and the remaining two were asymptomatic.All four patients had normal serum calcium and phosphorus levels.Chest CT of four DPO patients showed diffusely distributed high-density small nodules with calcification, scattered branched linear shadows and mild pleural thickening in both lungs.Two cases showed increased pulmonary radiotracer uptake on bone scintigraphy, suggesting an extracorporeal uptake.All four patients were diagnosed with DPO through lung biopsy pathology, including one patient who underwent surgical lung biopsy under thoracoscopy, two patients who underwent transbronchial cryobiopsy, and one patient who underwent transbronchial lung biopsy.Pathological examination showed mature bone tissue in the lungs, with some showing adipose tissue.Pulmonary function tests indicated obstructive ventilatory dysfunction in three cases, restrictive dysfunction in one, and decreased diffusion capacity in three.All four patients were treated with underlying diseases and comorbidities.As of July 2024, they were followed up for 8-32 months, showing a stable disease.Among 125 DPO patients in literature review, there were 106 male and 19 female patients, with a male-female ratio of 5.6∶1.Their average age of onset was 55.4±18.8 (15-91) years old.Forty (32.0%) cases were asymptomatic, and the others mainly showed respiratory symptoms.The chest imaging characteristics were manifested as diffuse high-density small nodules in both lungs with calcification and thin line shadows.All 125 patients were diagnosed with DPO through pathological examinations, including 79.2% (99/125) of dendritic type and 16.8% (21/125) of nodule type.Conclusions:DPO is occult onset, progresses slowly, and lacks typical manifestations.It more affects men than women.Typical imaging features include diffuse high-density small nodules in both lungs with calcification and branched thin line shadows.The increase in local radio uptake in both lungs can be seen in whole-body bone imaging.Transbronchial cryobiopsy is a desirable minimally invasive lung biopsy method, and pathological examinations reveal that mature bone tissue formation can be clearly diagnosed.The treatment of DPO is mainly focused on controlling underlying diseases and comorbidities.Most patients have slow progress and a good prognosis.