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Abstract：
BACKGROUND: Cryoglobulinemia with pulmonary involvement is rare, and its characteristics, radiological findings, and outcomes are still poorly understood.
METHODS: Ten patients with pulmonary involvement of 491 cryoglobulinemia patients at Peking Union Medical College Hospital were enrolled in this retrospective study. We analyzed the characteristics, radiological features and management of pulmonary involvement patients, and compared with those of non-pulmonary involvement with cryoglobulinemia.
RESULTS: The 10 patients with pulmonary involvement (2 males; median age, 53 years) included three patients with type I cryoglobulinemia and seven patients with mixed cryoglobulinemia. All of 10 patients were IgM isotype cryoglobulinemia. All type I patients were secondary to B-cell non-Hodgkin lymphoma. Four mixed patients were essential, and the remaining patients were secondary to infections (n = 2) and systemic lupus erythematosus (n = 1), respectively. Six patients had additional affected organs, including skin (60%), kidney (50%), peripheral nerves (30%), joints (20%), and heart (20%). The pulmonary symptoms included dyspnea (50%), dry cough (30%), chest tightness (30%), and hemoptysis (10%). Chest computed tomography (CT) showed diffuse ground-glass opacity (80%), nodules (40%), pleural effusions (30%), and reticulation (20%). Two patients experienced life-threatening diffuse alveolar hemorrhage. Five patients received corticosteroid-based regimens, and four received rituximab-based regimens. All patients on rituximab-based regimens achieved clinical remission. The estimated two-year overall survival (OS) was 40%. Patients with pulmonary involvement had significantly worse OS and progression-free survival than non-pulmonary involvement patients of cryoglobulinemia (P < 0.0001).
CONCLUSIONS: A diagnosis of pulmonary involvement should be highly suspected for patients with cryoglobulinemia and chest CT-indicated infiltrates without other explanations. Patients with pulmonary involvement had a poor prognosis. Rituximab-based treatment may improve the outcome.
METHODS: Ten patients with pulmonary involvement of 491 cryoglobulinemia patients at Peking Union Medical College Hospital were enrolled in this retrospective study. We analyzed the characteristics, radiological features and management of pulmonary involvement patients, and compared with those of non-pulmonary involvement with cryoglobulinemia.
RESULTS: The 10 patients with pulmonary involvement (2 males; median age, 53 years) included three patients with type I cryoglobulinemia and seven patients with mixed cryoglobulinemia. All of 10 patients were IgM isotype cryoglobulinemia. All type I patients were secondary to B-cell non-Hodgkin lymphoma. Four mixed patients were essential, and the remaining patients were secondary to infections (n = 2) and systemic lupus erythematosus (n = 1), respectively. Six patients had additional affected organs, including skin (60%), kidney (50%), peripheral nerves (30%), joints (20%), and heart (20%). The pulmonary symptoms included dyspnea (50%), dry cough (30%), chest tightness (30%), and hemoptysis (10%). Chest computed tomography (CT) showed diffuse ground-glass opacity (80%), nodules (40%), pleural effusions (30%), and reticulation (20%). Two patients experienced life-threatening diffuse alveolar hemorrhage. Five patients received corticosteroid-based regimens, and four received rituximab-based regimens. All patients on rituximab-based regimens achieved clinical remission. The estimated two-year overall survival (OS) was 40%. Patients with pulmonary involvement had significantly worse OS and progression-free survival than non-pulmonary involvement patients of cryoglobulinemia (P < 0.0001).
CONCLUSIONS: A diagnosis of pulmonary involvement should be highly suspected for patients with cryoglobulinemia and chest CT-indicated infiltrates without other explanations. Patients with pulmonary involvement had a poor prognosis. Rituximab-based treatment may improve the outcome.
摘要：
背景：冷球蛋白血症伴肺部受累是罕见的，及其特点，放射学发现，结果仍然知之甚少。
方法：本回顾性研究纳入北京协和医院491例冷球蛋白血症患者的10例肺部受累患者。我们分析了特点，肺部受累患者的放射学特征和管理，并与非肺部受累的冷球蛋白血症进行比较。
结果：10例肺部受累患者（2例男性；中位年龄，53年）包括3例I型冷球蛋白血症患者和7例混合性冷球蛋白血症患者。10例患者均为IgM同种型冷球蛋白血症。所有I型患者均继发于B细胞非霍奇金淋巴瘤。四个混合患者是必不可少的，其余患者继发于感染（n=2）和系统性红斑狼疮（n=1），分别。六名患者有其他受影响的器官，包括皮肤（60%），肾脏（50%），周围神经（30%），接头（20%），心脏（20%）。肺部症状包括呼吸困难（50%），干咳（30%），胸闷（30%），咯血（10%）。胸部计算机断层扫描（CT）显示弥漫性毛玻璃混浊（80％），结节（40%），胸腔积液（30%），和网状(20%)。两名患者经历了危及生命的弥漫性肺泡出血。五名患者接受了基于皮质类固醇的方案，4人接受了以利妥昔单抗为基础的治疗方案.所有以利妥昔单抗为基础的患者均达到临床缓解。估计的两年总生存率（OS）为40％。与冷球蛋白血症的非肺部受累患者相比，肺部受累患者的OS和无进展生存期明显更差（P<0.0001）。
结论：对于冷球蛋白血症和胸部CT显示的浸润而没有其他解释的患者，应高度怀疑肺部受累的诊断。肺部受累患者预后不良。基于利妥昔单抗的治疗可以改善结果。
方法：本回顾性研究纳入北京协和医院491例冷球蛋白血症患者的10例肺部受累患者。我们分析了特点，肺部受累患者的放射学特征和管理，并与非肺部受累的冷球蛋白血症进行比较。
结果：10例肺部受累患者（2例男性；中位年龄，53年）包括3例I型冷球蛋白血症患者和7例混合性冷球蛋白血症患者。10例患者均为IgM同种型冷球蛋白血症。所有I型患者均继发于B细胞非霍奇金淋巴瘤。四个混合患者是必不可少的，其余患者继发于感染（n=2）和系统性红斑狼疮（n=1），分别。六名患者有其他受影响的器官，包括皮肤（60%），肾脏（50%），周围神经（30%），接头（20%），心脏（20%）。肺部症状包括呼吸困难（50%），干咳（30%），胸闷（30%），咯血（10%）。胸部计算机断层扫描（CT）显示弥漫性毛玻璃混浊（80％），结节（40%），胸腔积液（30%），和网状(20%)。两名患者经历了危及生命的弥漫性肺泡出血。五名患者接受了基于皮质类固醇的方案，4人接受了以利妥昔单抗为基础的治疗方案.所有以利妥昔单抗为基础的患者均达到临床缓解。估计的两年总生存率（OS）为40％。与冷球蛋白血症的非肺部受累患者相比，肺部受累患者的OS和无进展生存期明显更差（P<0.0001）。
结论：对于冷球蛋白血症和胸部CT显示的浸润而没有其他解释的患者，应高度怀疑肺部受累的诊断。肺部受累患者预后不良。基于利妥昔单抗的治疗可以改善结果。
