Zhou Jiamin, Liu Xueqing, Li Jian, Zhang Lu, Zhang Wen, Zhang Weihong
Department of Radiology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, No.1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China.
Department of Hematology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, 100730, No.1 Shuaifuyuan, Dongcheng District, Beijing, China.
Lung. 2025 Jan 3;203(1):20. doi: 10.1007/s00408-024-00782-3.
Immunoglobulin G4-related disease (IgG4-RD) and plasma cell-type idiopathic multicentric Castleman disease (PC-iMCD) have many overlapping features. Their differential diagnosis is challenging and crucial for clinical management due to their different prognoses and treatments. However, reports that compare these conditions are scarce, especially for patients with lung involvement. In this study, we attempted to clarify the clinical and radiologic differences in lung involvement between IgG4-RD and PC-iMCD.
Patients with IgG4-RD or PC-iMCD who exhibited lung involvement were enrolled. Clinical and chest CT findings at baseline were compared.
A total of 178 patients with IgG4-RD and 61 patients with PC-iMCD exhibited lung involvement. The IgG4-RD group consisted of older patients (P < 0.001) and had a higher male‒female ratio (P = 0.004). Patients with PC-iMCD were more inclined to present constitutional and respiratory symptoms, anemia, thrombocytosis and hypoalbuminemia (all P < 0.001). Although IgG4 levels were commonly elevated in both diseases, they were significantly greater in the IgG4-RD (median: 16,100 mg/L) than in the PC-iMCD (median: 3130 mg/L) (P < 0.001). Patients with IgG4-RD showed significantly lower levels of IgG, IgA, IgM (median: 21.59 g/L, 1.70 g/L, and 0.68 g/L, respectively) than in the PC-iMCD (median: 34.42 g/L, 4.85 g/L, and 2.11 g/L, respectively) (all P < 0.001). The levels of CRP, ESR and IL-6 were significantly greater in the PC-iMCD (median: 72.15 mg/L, 103 mm/h, and 18.30 pg/mL, respectively) than that in the IgG4-RD (median: 1.54 mg/L, 22 mm/h, and 2.85 pg/mL, respectively) (all P < 0.001). Although both nodular lesions and thickened bronchovascular bundles were common in these two diseases, PC-iMCD patients presented more extensive nodular lesions (P < 0.001), and IgG4-RD patients presented more diffuse thickened bronchovascular bundles (P < 0.001). Cysts were almost exclusively observed in PC-iMCD patients.
Compared with IgG4-RD, PC-iMCD is a more aggressive condition, associated with more common symptoms and more severe inflammation. Radiologically, extensive nodular lesions or cysts suggest a diagnosis of PC-iMCD, whereas diffuse thickened bronchovascular bundles indicate a diagnosis of IgG4-RD.
免疫球蛋白G4相关性疾病（IgG4-RD）与浆细胞型特发性多中心Castleman病（PC-iMCD）有许多重叠特征。由于它们的预后和治疗不同，其鉴别诊断具有挑战性且对临床管理至关重要。然而，比较这两种疾病的报道很少，尤其是对于有肺部受累的患者。在本研究中，我们试图阐明IgG4-RD和PC-iMCD在肺部受累方面的临床和影像学差异。
纳入有肺部受累表现的IgG4-RD或PC-iMCD患者。比较基线时的临床和胸部CT表现。
共有178例IgG4-RD患者和61例PC-iMCD患者有肺部受累。IgG4-RD组患者年龄较大（P < 0.001），男女比例较高（P = 0.004）。PC-iMCD患者更倾向于出现全身症状和呼吸道症状、贫血、血小板增多症和低白蛋白血症（均P < 0.001）。虽然两种疾病中IgG4水平通常都升高，但IgG4-RD中的IgG4水平（中位数：16,100 mg/L）显著高于PC-iMCD（中位数：3130 mg/L）（P < 0.001）。IgG4-RD患者的IgG、IgA、IgM水平（中位数分别为21.59 g/L、1.70 g/L和0.68 g/L）显著低于PC-iMCD患者（中位数分别为34.42 g/L、4.85 g/L和2.11 g/L）（均P < 0.001）。PC-iMCD患者的C反应蛋白（CRP）、红细胞沉降率（ESR）和白细胞介素6（IL-6）水平（中位数分别为72.15 mg/L、103 mm/h和18.30 pg/mL）显著高于IgG4-RD患者（中位数分别为1.54 mg/L、22 mm/h和2.85 pg/mL）（均P < 0.001）。虽然结节状病变和支气管血管束增粗在这两种疾病中都很常见，但PC-iMCD患者的结节状病变更广泛（P < 0.001），而IgG4-RD患者的支气管血管束弥漫性增粗更明显（P < 0.001）。囊肿几乎仅见于PC-iMCD患者。
与IgG4-RD相比，PC-iMCD病情更具侵袭性，症状更常见且炎症更严重。在影像学上，广泛的结节状病变或囊肿提示PC-iMCD的诊断，而弥漫性支气管血管束增粗提示IgG4-RD的诊断。
