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.

The aim of this study was to investigate the possible relationships between sudden sensorineural hearing loss (SSNHL) patients with distinct audiographic data and nucleate cell count and neutrophil to lymphocyte ratio (NLR).SSNHL patients differed in audiographic curves were included, 40 with low-frequency SSNHL (LF-SSNHL), 33 with high-frequency SSNHL (HF-SSNHL), 36 with all-frequency SSNHL (AF-SSNHL), 34 with total-deafness SSNHL (TD-SSNHL), and 31 age- and sex-matched healthy controls. Peripheral venous blood samples were collected and nucleate cell such as white blood cell (WBC), neutrophil and lymphocyte, and NLR were measured. Each group was divided into recovery and unrecovery subgroup according to hearing levels after 1-month therapy, and then compared the difference of the count of WBC, neutrophil and lymphocyte, and NLR between the 2 subgroups.The WBC count of the HF-SSNHL, AF-SSNHL, and TD-SSNHL group was significantly higher than that of the control group (P = .024, P = .003, P = .008, respectively), not for LF-SSNHL group (P = .248). WBC count between LF-SSNHL and AF-SSNHL group was significantly different (P = .045). The neutrophil count of the HF-SSNHL, AF-SSNHL, and TD-SSNHL group was significantly higher than that of the control group (P = .002, P = .000, P = .000, respectively), not for LF-SSNHL group (P = .069). Neutrophil count of LF-SSNHL was significantly lower than that of AF-SSNHL and TD-SSNHL groups (P = .014, P = .013). The lymphocyte count of AF-SSNHL and TD-SSNHL group was significantly lower than that of the control group (P = .027, P = .003), not for LF-SSNHL and HF-SSNHL group (P = .119, P = .054). NLR of HF-SSNHL, AF-SSNHL, and TD-SSNHL group was significantly higher than that of the control group (P = .001, P = .000, P = .000, respectively), not for LF-SSNHL group (P = .070). NLR of LF-SSNHL was significantly lower than that of AF-SSNHL and TD-SSNHL group (P = .041, P = .005). In HF-SSNHL patients, lymphocyte count of unrecovery subgroup was significantly lower, while NLR of the unrecovery subgroup were significantly higher than those of recovery subgroup (P = .017, P = .015).The count of WBC, neutrophil, lymphocyte, and NLR may be related to SSNHL, but they were unreliable at predicting SSNHL characterized by differences in audiometric curves. Higher NLR and lower lymphocyte count maybe used to evaluate prognosis of HF-SSNHL patients.