Open AccessIgG4-related disease of pulmonary artery causing pulmonary hypertension
Author(s) -
Hui Deng,
Shunying Zhao,
Yunlong Yue,
Yong Liu,
Yali Xu,
Jin Qian,
Xiaorong Ma,
Peiliang Gao,
Xiaoyan Yao,
Xin Jiang,
Xiequn Xu,
ZhiCheng Jing,
Yong Wang,
Ling Pan,
Xinying Xue
Publication year - 2018
Publication title -
medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.59
H-Index - 148
eISSN - 1536-5964
pISSN - 0025-7974
DOI - 10.1097/md.0000000000010698
Subject(s) - medicine , pulmonary artery , thrombus , pulmonary hypertension , radiology , cyclophosphamide , rituximab , right pulmonary artery , left pulmonary artery , pulmonary angiography , cardiology , lymphoma , chemotherapy
IgG4-related disease (IgG4-RD) is recognized as an immune-mediated condition with pathology features of lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis, accompanied with or without elevated serum IgG4 concentrations. However, few of pulmonary artery IgG4-RD causing pulmonary hypertension (PH) was reported. The medical records of 3 patients with pulmonary artery IgG4-RD inducing PH were analyzed retrospectively. Imaging findings demonstrated that the lesions of 3 patients located in pulmonary artery, which were initially diagnosed as pulmonary thrombus or malignant tumor. Computed tomography pulmonary angiography (CTPA), ultrasonic cardiogram, and positron emission tomography/computed tomography (PET/CT) didn’t support the diagnosis of pulmonary thrombus or malignant tumor. Right heart catheterization (RHC) showed definite PH. Biopsy by right heart catheterization in 2 patients or pneumonectomy in 1 patient confirmed the diagnosis as IgG4-RD. Treated with glucocorticoids and cyclophosphamide or rituximab, 2 patients’ IgG4 concentrations declined sharply and the lesions shrunk gradually. Another patient treated with glucocorticoids died of heart failure. IgG4-RD involved pulmonary artery causing PH was rare. A high index of awareness of this disease is required for early diagnosis and treatment. PET/CT might be a valuable approach to distinguish pulmonary artery IgG4-RD from pulmonary thrombus and malignant tumor.