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.

Pyrolytic cavitation, selective adsorption and molecular recognition of a porous Eu(III) MOF.

A channel-equipped metal-organic framework (1-pyr), resulting from the pyrolysis of [(CH3)2NH2]@[Eu2L3(HCOO)] (1) (L(2-) = isophthalate), showed the preferable absorption of CO2 and the Eu(III)-based emission quenched by aromatics.

Early biochemical response to ursodeoxycholic acid and long-term prognosis of primary biliary cirrhosis: results of a 14-year cohort study.

UNLABELLED: The biochemical response to ursodeoxycholic acid (UDCA) in primary biliary cirrhosis is a strong predictor of long-term outcome and thus facilitates the rapid identification of patients needing new therapeutic approaches. Numerous criteria for predicting outcome of treatment have been studied based on biochemical response to UDCA at 1 year. We sought to determine whether an earlier biochemical response at 3 or 6 months could as efficiently identify patients at risk of poor outcome, as defined by liver-related death, liver transplantation, and complications of cirrhosis. We analyzed the prospectively collected data of 187 patients with a median follow-up of 5.8 years (range, 1.3-14 years). The survival rates without adverse outcome at 5 years and 10 years were 86% and 63%. Under UDCA therapy, laboratory liver parameters experienced the most prominent improvement in the first 3 months (P < 0.0001) and then stayed relatively stable for the following months. The Paris, Barcelona, Toronto, and Ehime definitions, but not the Rotterdam definition, applied at 3, 6, and 12 months significantly discriminated the patients in terms of long-term outcome. Compared with biochemical responses evaluated after 1 year of UDCA therapy, biochemical responses at the third month demonstrated higher positive predictive value (PPV) but lower negative predictive value (NPV) and increased negative likelihood ratio (NLR) by all definitions; biochemical responses at the sixth month showed higher or the same PPV and NPV and lower NLR by all definitions. CONCLUSION: For the previously published criteria, biochemical responses at the sixth month can be used in place of those evaluated after 1 year of UDCA therapy. Our findings justify a more rapid identification of patients who need new therapeutic approaches.

[The characteristics of peripheral lymphocytic subsets and cytokines in primary biliary and their changes to drug treatment.].

OBJECTIVE: To investigate the characteristics of peripheral lymphocytic subsets and cytokines in patients with primary biliary cirrhosis (PBC) and their changes after therapy. METHODS: Eighty two patients with untreated PBC were divided randomly into three groups. Group U (28 patients) were treated with ursodeoxycholic acid (UDCA), group UP (27 patients) were treated with UDCA and prednisonlone, while group UA (27 patients) were treated with UDCA and azatharaprine. Levels of peripheral lymphocytic subsets and cytokines were monitored at 0, 3, 6 months after therapy. Twenty healthy people were included as healthy control (HC). RESULTS: Compared with HC, the percentage of CD(4)(+)T cell (P = 0.044) increased in PBC; the levels of IFNgamma (P = 0.001), IL-2 (P = 0.000), IL-4 (P = 0.010), IL-6 (P = 0.029), tumor necrosis factoralpha (TNFalpha) (P = 0.000), IL-1beta (P = 0.008) and transforming growth factorbeta (TGFbeta) (P = 0.069) increased in PBC. The level of TNFalpha in peripheral was correlated with those of ALT (P = 0.005), AST (P = 0.002), total bilirubin (TBil) (P = 0.001), direct bilirubin (DBil) (P = 0.002), and mayo risk score (MRS) (P = 0.020). The percentage of CD(4)(+)T cell decreased in group U. In group U, the levels of IFNgamma, IL-4, IL-6 all decreased after therapy at the third month, but rebounded thereafter. In group of UP and UA, the levels of IFNgamma, IL-4, IL-6 were suppressed below the levels of baseline after therapy. Levels of TNFalpha and IL-2 decreased in all three groups after therapy. CONCLUSIONS: Levels of both Th1 and Th2 related cytokines are abnormal in the peripheral blood of patients with PBC. The abnormal levels of Th1 related cytokine indicated that PBC is a cell immunity based autoimmune disease. IFNgamma, TNFalpha are directly related to the disease. The three treatments have different effects on the adjustment of the immune system.

Antimitochondrial antibody-negative primary biliary cirrhosis: a subset of primary biliary cirrhosis.

OBJECTIVE: Antimitochondrial antibodies (AMA) are the hallmark in primary biliary cirrhosis (PBC); nevertheless, it has long been recognized that 5-10% patients with typical features compatible with PBC do not have detectable AMA, and they were referred to as 'AMA-negative PBC'. This study aimed to evaluate whether AMA-negative/positive PBC represents different clinical entities. METHODS: We compared the clinical, laboratory, percentage of regulatory T cells (Tregs) in peripheral blood, liver biopsy features and response to treatment of the two groups of patients. The first group was comprised of 12 patients with 'AMA-negative PBC'. The second was made up of another 12 PBC patients with positive AMA. RESULTS: Antimitochondrial antibodies-negative/positive patients were remarkably similar in terms of clinical manifestations, liver biochemistries and histological findings. The frequency of anti-nuclear antibodies, anti-smooth-muscle antibody, anti-gp210 and anti-sp100 antibody showed no significant difference between the two groups. A significantly lower mean percentage of CD4+CD25(high) T cells was observed in peripheral blood mononuclear cells of AMA-negative/positive PBC patients compared with that of the 12 control subjects (5.8+/-1.8 and 5.4+/-1.4% vs. 7.6+/-1.7% respectively; P=0.014 and 0.004). However, no difference could be found between AMA-negative and AMA-positive PBC patients (P=0.599). After 1 year treatment with ursodeoxycholic acid, the two groups showed similar response. CONCLUSION: Antimitochondrial antibody-negative/positive PBC patients are similar in clinical, laboratory, percentage of Treg in peripheral blood, liver biopsy features and response to treatment. This suggests that AMA-negative PBC may be a variant of AMA-positive PBC rather than a separate clinical entity.

[Characteristics of airway involvement in relapsing polychondritis].

OBJECTIVE: To investigate the characteristics of airway involvement in relapsing polychondritis (RP). METHODS: The clinical data, including clinical manifestations, respiratory function test, computerized tomography (CT), and bronchoscopy of 38 out of the 56 RP patients who had airway involvement, 20 males and 18 females, with the mean onset age of 45 +/- 11 (27 - 71), and 3 RP patients without airway involvement were retrospectively analyzed. Three patients out of the 16 RP patients who did not have respiratory involvement but underwent respiratory function test, CT, and bronchoscopy were used as controls. RESULTS: The symptoms of airway involvement included cough, expectoration, hoarseness, feeling of suffocation, asthma, and dyspnea. Obstructive disturbance of ventilation was found in 10 and mixed disturbance of ventilation was seen in 2 of the 18 RP patients with airway involvement. The forced expiratory volume in one second, ratio of forced expiratory volume in one second to forced vital capacity, peak expiratory flow, FEF50/FIF50, and maximal mild-expiratory flow of the patients with airway involvement were 1.4 L +/- 0.23 L, 46.0 +/- 4.86, 3.3 L/s +/- 0.67 L/s, 0.3 +/- 0.08, and 0.4 L/s +/- 0.18 L/s respectively, all significantly lower than those of the RP patients without airway involvement (2.5 L +/- 0.09 L, 83.7 +/- 2.24, 6.9 L/s +/- 0.52 L/s, 1.3 +/- 0.51, and 2.8 L/s +/- 0.73 L/s, all P = 0.01). Flow volume loop showed remarkable decrease of PEF and formation of a plateau in the expiratory phase in the RP patients with airway involvement. CT performed in 27 RP patients with airway involvement showed trachea stenosis in 11, and thickened airway wall in 8 of them. Bronchoscopy performed in 23 patients with airway involvement showed inflammation in 16, destruction of tracheobronchial cartilage in 6, collapsed tracheobronchial wall in 7, tracheal stenosis in 15, left major bronchial stenosis in 13, and right major bronchial stenosis in 12 of them. CONCLUSION: Respiratory function test is sensitive in early detection of airway involvement in RP. Bronchoscopy and CT are useful in evaluation of the severity of airway involvement in patients with RP.

[Clinical analysis of pneumomediastinum complicated in polymyositis and dermatomyositis].

OBJECTIVE: To analyze the clinical features of pneumomediastinum complicated in polymyositis and dermatomyositis (PM/DM) and to study the pathogenesis thereof. METHODS: The clinical data of 4 patients with pneumomediastinum complicated in dermatomyositis out of 447 PM/DM patients hospitalized in Peking Union Medical College (PUMC) Hospital Jan 1989 to June 2005, were analyzed. The records of patients with PM/DM available in English throughout the world were reviewed to collect those with pneumomediastinum as a complication. And the data of these patients were analyzed, focusing mainly on the age, gender, peak of serum creatine kinase (CK), presence of pneumomediastinum, cutaneous vasculopathy, chest radiographic changes, tracheal cannula, management, and outcome. RESULTS: Among the 447 patients with PM/DM hospitalized in PUMC Hospital, 134 males and 313 females, aged 42 +/- 17, pneumomediastinum was observed as a complication in four patients, 3 males and 1 female, aged 12 - 43, with a prevalence rate of 0.9%. Together with 17 cases reported in the English literatures there were 21 patients with pneumomediastinum complicated in polymyositis and dermatomyositis (PM/DM in all. Only one of the literatures reported a prevalence rate as high as 8.3% (4/48), and other literatures were merely case reports. Compared with the PM/DM patients without pneumomediastinum the mean age of the PM/DM patients with pneumomediastinum was significantly younger (34:42, P < 0.01), the male: female ratio significantly higher (13:8 to 132:311, P < 0.01), the morbidity rates of interstitial lung disease and of cutaneous vasculopathy significantly higher (18/21 to 134/443, and 12/21 to 44/443, both P < 0.01). Although statistic analysis could not be undertaken because of the peak of CK not being provided in details in the literatures, the CK levels of the patients with pneumomediastinum were mostly normal or mildly higher with a peak lower than 500 U/L Three of the 4 patients with pneumomediastinum hospitalized in PUMC Hospital and 5 of the 443 patients (1.1%) without this complication received tracheal cannula. There was a significant association of pneumomediastinum with tracheal cannula (P = 0.000). CONCLUSION: Vasculopathy is strongly suspected as being responsible for the pneumomediastinum in DM, and male gender, younger age, interstitial lung disease, and tracheal cannula may be the risk factors of this pneumomediastinum complicated in PM/DM.

[Room-temperature solid-state synthesis of complexes of copper acetate and multi-nitrogen heterocyclic ligands].

At room temperature, sufficiently grinding the mixtures of copper acetate with 1,2,4-triazole and benzotriazole separately resulted in the proceeding of the solid state reaction. It was found that the acetate acid flowed during the grinding. The heterocycle ligand 1,2,4-triazole or benzotriazole replaced the acetate and coordinates to Cu (II) to form a mixed ligands complex. The elementary analysis results show that the compositions of products were consistent with Cu (C2H2N3)(Ac).H2O and Cu (C6H4N3)(Ac).H2O, respectively. Infrared spectra of both complexes have exhibited the characteristics of C=N vibrations [Cu(C2H2N3)(Ac).H2O, 1,513 cm-1; Cu(C6H4N3)(Ac).H2O, 1,446 cm-1] for heterocycle ligands, C=O [Cu(C2H2N3)(Ac).H2O, 1,570 and 1,406 cm-1; Cu(C6H4N3)(Ac).H2O, 1,604 and 1,422 cm-1] for acetate and O-H (-3,400 cm-1) for water, respectively. The solid state reaction provides a way which is rapid, mild and in the absence of solvent for synthesized new complexes.