Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Chest. 2023 Aug;164(2):418-428. doi: 10.1016/j.chest.2023.03.022. Epub 2023 Mar 22.
Previous studies about multicentric Castleman disease-associated pulmonary manifestations have been limited by small cohorts and not following the Castleman Disease Collaborative Network classification criteria of multicentric Castleman disease. The pulmonary manifestations in idiopathic multicentric Castleman disease-not otherwise specified (iMCD-NOS), a distinct clinical phenotype in the classification criteria, have not been reported.
Which pulmonary abnormalities in iMCD-NOS are advanced manifestations and which are reversible after effective treatment?
Patients diagnosed with iMCD-NOS with pulmonary involvement were enrolled. The baseline CT scan was evaluated for the presence and anatomic locations of pulmonary abnormalities. Patients were further divided into different subgroups according to baseline CT scan manifestations. Follow-up CT scan was reviewed to assess the changes in pulmonary lesions among patients without and with treatment.
Of 162 patients with iMCD-NOS, 58 individuals (35.8%) with pulmonary involvement were identified. Pulmonary manifestations included nodules (96.6%), cysts (65.5%), consolidation (22.4%), interstitial thickening (50.0%-87.9%), and ground-glass opacities (55.2%). Patients (n = 58) were further classified into nodule (n = 15), cyst (n = 33), and consolidation (n = 10) subgroups. Patients in the consolidation (median, 67 months) and cyst (median, 23 months) subgroups had a longer duration of symptoms before the baseline CT examination than those in the nodule subgroup (median, 12 months) (P = .016). During follow-up, the evolution of pulmonary lesions from nodules to cysts was observed in two patients without treatment. After treatment, pulmonary lesions, except for cysts, improved in most patients. Moreover, nodules or cysts progressed into consolidation in two patients.
Pulmonary involvement is not rare in iMCD-NOS. Chest CT scan examination is very essential in finding potential pulmonary abnormalities. Pulmonary manifestations follow a unique pattern with evolution from nodules to cysts or consolidation, the latter of which can also form in cystic areas. Timely diagnosis of pulmonary involvement is crucial because of possible reversibility after treatment.
以前关于多发性 Castleman 病相关肺部表现的研究受到小队列和不符合 Castleman 疾病协作网络多发性 Castleman 病分类标准的限制。分类标准中特有的一种临床表型特发性多发性 Castleman 病-未特指（iMCD-NOS）的肺部表现尚未报道。
iMCD-NOS 中的哪些肺部异常是进展性表现，哪些在有效治疗后是可逆的？
招募了诊断为 iMCD-NOS 且有肺部受累的患者。评估基线 CT 扫描是否存在肺部异常及其解剖部位。根据基线 CT 扫描表现，患者进一步分为不同亚组。对无治疗和有治疗患者的随访 CT 扫描进行评估，以评估肺部病变的变化。
在 162 例 iMCD-NOS 患者中，发现 58 例（35.8%）有肺部受累。肺部表现包括结节（96.6%）、囊肿（65.5%）、实变（22.4%）、间质增厚（50.0%-87.9%）和磨玻璃影（55.2%）。患者（n=58）进一步分为结节亚组（n=15）、囊肿亚组（n=33）和实变亚组（n=10）。与结节亚组（中位，12 个月）相比，实变（中位，67 个月）和囊肿（中位，23 个月）亚组的基线 CT 检查前症状持续时间更长（P=.016）。在随访期间，两名未接受治疗的患者的肺部病变从结节进展为囊肿。经过治疗，除囊肿外，大多数患者的肺部病变均有所改善。此外，两名患者的结节或囊肿进展为实变。
iMCD-NOS 肺部受累并不少见。胸部 CT 扫描检查对于发现潜在的肺部异常非常重要。肺部表现具有独特的模式，从结节发展为囊肿或实变，后者也可以在囊性区域形成。及时诊断肺部受累至关重要，因为治疗后可能会逆转。
