Effect of IL-17 on pulmonary artery smooth muscle cells and connective tissue disease-associated pulmonary arterial hypertension.

OBJECTIVE: To explore the role of interleukin (IL)-17 in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) and to investigate its possible mechanism on pulmonary artery smooth muscle cells (PASMCs). METHODS: Enzyme-linked immunosorbent assay (ELISA) were used to compare levels of serum IL-17 in patients with CTD-PAH and healthy controls (HCs). After treatment for 3 months, the serum IL-17 levels were tested in CTD-PAH. ELISA and immunohistochemistry were used to compare levels of serum IL-17 and numbers of pulmonary artery IL-17+ cells, respectively, in a rat model of monocrotaline-induced PAH and untreated rats. Proliferation, migration, and inflammatory factors expression of PASMCs were assessed after stimulation with different concentrations of IL-17 for various time periods. Proteins in the mitogen-activated protein kinase (MAPK) pathway were examined by western blot. RESULTS: Levels of IL-17 were upregulated in patients with CTD-PAH compared to HCs. After 3 months of treatment, serum IL-17 levels were downregulated with pulmonary artery pressure amelioration. Moreover, serum IL-17 levels and numbers of IL-17+ cells infiltrating lung arterioles were increased in PAH model rats. IL-17 could dose- and time-dependently promote proliferation and migration of PASMCs as well as time-dependently induce IL-6 and intercellular cell adhesion molecule-1 (ICAM-1) expression. The levels of MKK6 increased after IL-17 treatment. Inhibition of MAPK decreased proliferation of PASMCs. CONCLUSION: Levels of IL-17 may increase in CTD-PAH, and IL-17 promotes proliferation, migration, and secretion of IL-6 and ICAM in PASMCs, respectively, which likely involves the p-38 MAPK pathway.

Associations between sarcoidosis, autoimmune diseases, and autoantibodies: a single-center retrospective study in China.

To describe the clinical manifestations, immunological features, and risk factors in patients with sarcoidosis complicated with autoimmune diseases (ADs) as well as determine the frequency of autoantibodies and possible correlation between autoantibodies and laboratory data. Patients with pathologically confirmed sarcoidosis at Beijing Chaoyang Hospital (China) between January 2017 and October 2020 were included. Age- and sex-matched patients who visited the rheumatology outpatient clinic without systemic or ADs were included as controls. Demographic, clinical, serological, and radiological data of sarcoidosis patients were recorded and analyzed. To exclude ADs, autoantibodies, such as antinuclear antibody, extractable nuclear antigen antibodies, and anti-cyclic citrullinated peptide antibody were assessed in controls. A total of 154 sarcoidosis patients (111 females; 72.1%) with a mean ± standard deviation age of 50.7 ± 10.3 years were included. Nineteen patients (12.3%) had ADs; Hashimoto's thyroiditis (n = 6) and Sjogren's syndrome (n = 4) were common. Age, globulin, immunoglobulin G, erythrocyte sedimentation rate (ESR), and C-reactive protein were significantly different between sarcoidosis patients with and without ADs. The ESR level might be a risk factor for sarcoidosis complicated with ADs (RR = 1.053; P = 0.018). Autoantibodies were detected in 29 patients (18.8%), and the frequency was significantly higher than that in controls (18.8% vs. 3%; P = 0.001). Sarcoidosis patients were more likely to have autoantibodies despite the absence of ADs (10.4% vs. 3%; P = 0.031). Age may be a risk factor for sarcoidosis patients presenting with autoantibodies (RR = 1.077; P = 0.042). An association was identified between ADs and sarcoidosis. The inflammatory indexes, such as ESR, IgG, and CRP, were significantly different between sarcoidosis patients with and without ADs. ESR might be a risk factor for the coexistence of ADs and sarcoidosis. Sarcoidosis patients were prone to being autoantibody-positive despite the absence of ADs, and age might be a risk factor for sarcoidosis presenting with autoantibodies.

Thymic epithelial tumor complicated by immunological abnormalities: results from a single-center retrospective study in China.

BACKGROUND: To describe the clinical manifestations, immunological features, treatments, and outcomes of patients with thymic epithelial tumor (TET) complicated by immunological abnormalities, and to improve knowledge on immunological abnormalities in this rare disease. METHODS: Patients with pathologically confirmed TET at Beijing Chaoyang Hospital between January 2013 and May 2018 were included in this study, and clinical data were analyzed retrospectively. Immunological abnormalities were classified into two groups as follows: Good syndrome (GS) and autoimmune disease (AD). RESULTS: Fifty-nine TET patients were enrolled; twenty-two patients (37.3%) had immune dysfunction. There were no gender, age, or histological type differences between groups with or without immunological abnormalities. Six patients had GS, of whom four patients were diagnosed after thymectomy. Recurrent respiratory infections, particularly opportunistic infections, were the most common manifestation. Three GS patients developed a second cancer (50%; P=0.011). Anti-infective therapy and immunoglobulin supplements effectively treated GS. Seventeen patients developed ADs, including myasthenia gravis (MG) (n=13), Hashimoto's thyroiditis (n=4), Sjogren's syndrome (n=1), rheumatoid arthritis (n=1), pemphigus (n=1), and Evans syndrome (n=1). One patient developed both MG and GS and 4 patients presented with two ADs. Three AD cases occurred after thymectomy. Pemphigus and 80% (8/10) of MG cases were resolved following thymectomy. CONCLUSIONS: There is a strong association between immunological abnormalities and TET, which may present at any time point during the disease, even after thymectomy. In addition to infection, GS patients are more likely to develop a second cancer. Thymectomy may produce favorable outcomes for MG in this study, while surgery does not improve immunodeficiency in GS patients.

A rare case of meningoencephalitis by Listeria monocytogenes in systemic lupus erythematosus: case report and review.

Patients with systemic lupus erythematosus (SLE) have a high risk of infection. Central nervous system infection and neuropsychiatric SLE are both major causes of death. It is vital to distinguish between these two conditions to improve prognosis due to the treatment paradigms required for each condition. Here, we report one case of meningoencephalitis by Listeria monocytogenes (LM) in a patient with SLE who presented with fever and developed headache and altered in consciousness in the hospital. The cerebrospinal fluid culture was positive for LM, and magnetic resonance imaging (MRI) findings were suggestive of ependymitis and periventricular white matter lesions. Amoxicillin/sulbactam, trimethoprim-sulfamethoxazole, and rifampicin were administered for 8 weeks. The patient had a relative good recovery without serious neurological sequelae after a follow-up of nearly 2 years. MRI abnormalities also had obvious resolution.

Successful treatment of granulomatosis with polyangiitis with hydropneumothorax using corticosteroids and immunosuppressant.

Pneumothorax and pleural effusion is a rare and serious complication of granulomatosis with polyangiitis (GPA). The present study reported a case with a history of sinusitis for 20 years, dry cough for three years and exacerbated purulent nasal discharge and recurrent skin ulcers for two years. The patient experienced sudden difficulty in breathing two months prior to presentation. Lung computed tomography (CT) showed multiple bilateral lung nodules and cavitary nodules as well as right hydropneumothorax. Paranasal sinus CT showed soft tissue infiltration. The cytoplasmic pattern of anti-neutrophil cytoplasmic antibody (c-ANCA) was positive and anti-proteinase 3 (PR3) antibodies, erythrocyte sedimentation rate and C-reactive protein were elevated. After pleural drainage and methylprednisolone pulse treatment, followed by cyclophosphamide and cyclosporine, the patient's symptoms were ameliorated, lungs were re-expanded, and c-ANCA, PR3 and inflammatory markers returned to normal levels.

[Clinical Efficacy and Safety of Rituximab Combined with Fludarabine and Cyclophosphamide for Treatment of Chronic Lymphocytic Leukemia].

OBJECTIVE: To explore the clinical efficacy and safety of rituximab combined with fludarabine and cyclophosphamide for the treatment of the chronic lymphocytic leukemia (CLL). METHODS: Forty cases of CLL patients treated in our hospital from March 2010 to March 2014 years were selected and divided into the observation group (20 cases) and control group (20 cases) by random number table method. The patients in control group were treated with CHOP chemotherapy, the patients in observation group were treated with rituximab combined with fludarabine, cyclophosphamide treatment. The therapeutic efficacy of patients in 2 groups was analyzed according to the peripheral hemogram indexes, symptom and sign disappeared time as well as adverse reaction incidence. RESULTS: the remission rate in observation group was 90.00%, which was significantly higher than that in control group (70.00%) (P < 0.05); the peripheral hemogram indexes in 2 groups before treatment showed no significant difference (P > 0.05), and were significantly improved after treatment, but the white blood cell count and lymphocyte absolute number were significantly lower in observation group as compared to the control group (P < 0.05); symptom and sign disappeared time in observation group were significantly shorter as compared with the control group (P < 0.05); adverse reaction incidence in obseovation group was significantly lower as compared with control group (P < 0.05). CONCLUSION: application of rituximab combined with fludarabine and cyclophosphamide in the treatment of CLL shows the higher curative effect, can effectively improve the symptoms and reduce the incidence of adverse reactions. It is worthy to be popularized.