17例抗高尔基体抗体阳性患者的临床特点

目的探讨抗高尔基体抗体(anti-golgi autoantibodies,AGAs)在临床就诊患者中的阳性分布及其相关临床意义。方法对2008年至2013年温州医科大学附属温岭医院用间接免疫荧光法检测AGAs阳性的临床标本进行回顾性分析,并分析AGAs在临床就诊患者中的阳性检出率及AGAs阳性患者的相关临床、实验室特征。结果 AGAs在HEp-2细胞猴肝组织冰冻切片上呈现特征性的荧光模式。在20 471例临床标本中AGAs阳性17例(0.08%),其中女性12例(70.59%),男性5例(29.41%),年龄37~75岁(平均53岁)。17例AGAs阳性者包括自身免疫性疾病(autoimmune diseases,AID)患者6例,慢性乙型肝炎病毒感染相关疾病患者4例,其他疾病患者7例。AGAs阳性滴度范围为1∶100~1∶3200,其中滴度1∶100~1∶320有14例(82.35%),主要见于非AID患者;而滴度为1∶1000~1∶3200的3例患者均见于系统性AID患者(系统性红斑狼疮、类风湿关节炎)。在部分AGAs阳性患者的临床随访中,3例系统性AID患者经11~18月随访后,AGAs持续高滴度;5例非AID临床随访中,3例AGAs转为阴性,2例AGAs未发生明显改变。结论 AGAs阳性可见于多种临床疾病患者,AGAs并非是AID的特征性自身抗体。其高滴度、持续阳性主要见于系统性AID患者,低滴度、短暂阳性主要见于非AID患者。     

Mast Cells and Lysozyme Positive Macrophages in Bronchoalveolar Lavage from Patients with Sarcoidosis

ABSTRACT In the deteriorating group of sarcoidosis patients, progress towards pulmonary fibrosis is a major problem. In order to benefit from corticosteroids, it is important for the treatment to start early. We studied a group of 45 patients with sarcoidosis. Most of them were newly detected patients and none were under or had currently received corticosteroid therapy. The patients were followed for at least six months. We found that increased amounts of polymorphonuclear neutrophils (PMN) or lysozyme-positive macrophages (Lys⁺MF) and mast cells (MC) in bronchoalveolar lavage (BAL) could implicate a bad prognosis.     

ECG Abnormalities in Patients with Sarcoidosis

The frequency and type of some abnormal ECG findings in 86 consecutive patients with intrathoracic sarcoidosis were studied and compared with 86 age- and sex-matched healthy controls. Ail ECGs from the patients and controls were mixed and evaluated blindly by one of the investigators. Ectopic beats were seen in 6 patients in the sarcoidosis group and in 3 individuals in the control group. Patients with stage m sarcoidosis had more conduction defects compared with stage I and n patients and with the controls. Repolarization disturbances were found in 17 patients and 6 controls. These disturbances were more common in patients with stage I and II man in those with stage III sarcoidosis. The total number of patients with some ECG abnormality was 27 compared to 12 controls.     

Exercise Test in Patients with Sarcoidosis

ABSTRACT The study population comprises 28 patients with sarcoidosis who all had repolarization disturbances in their exercise ECGs. None of the patients had hypertension or known cardiovascular disease, and all but two were non-smokers. The mean age was 45 years. Exercise test with β-adrenergic blockade was performed within one month of the first examination. Persisting abnormal ST-T changes in exercise ECGs after β-blockade were seen in 12 (43%) patients. No significant relationship was found between persisting ST-T changes and age, sex, chest X-ray stage, lung function or working capacity. In an earlier study, we found ST-T abnormalities in exercise ECG in 56 of 127 individuals (44%) in a consecutive 5-year study of patients with newly detected sarcoidosis. From this and the present report we postulate that, in our region, as much as 20% of the patients with newly detected sarcoidosis might have organic myocardial disease, possibly of sarcoid origin, as shown by repolarization disturbances in exercise ECG. If ECG abnormalities in the ST-T region are present in patients with sarcoidosis, exercise ECG with β-blockade is a simple way of establishing suspicion of organic myocardial lesions. If exercise ECG abnormalities in the ST-T region persist after β-blockade, careful clinical follow-up is recommended, and, in some patients early steroid therapy should be considered.     

Sarcoidosis in Patients with Celiac Disease

Purpose Several case reports and European studies have suggested an association between sarcoidosis and celiac disease; however, they have been inconsistent. We therefore analyzed a large cohort of celiac-disease patients to assess this association. Methods An anonymized database of patients with celiac disease was reviewed to determine the number of patients with sarcoidosis. Age- and gender-adjusted standardized morbidity ratios with corresponding 95% confidence intervals (CI) were calculated by comparing results to US-population-derived prevalence data. Results Ten patients were found to have a comorbid diagnosis of sarcoidosis, representing an age- and gender-adjusted standardized morbidity ratio of 36.8 (95% CI 26.7–50.9). Conclusions In this cohort of patients with celiac disease, there was a significantly increased risk of sarcoidosis when compared with the American white population. This further strengthens prior associations that have been made suggesting a shared mechanism behind the etiologies of celiac disease and sarcoidosis.     

Fatigue in Patients with Sarcoidosis in Denmark

Lung - Sarcoidosis is a systemic granulomatous disease of unknown etiology, primarily affecting the lungs and thoracic lymph nodes. Fatigue is a frequent and disabling symptom in sarcoidosis with a...     

Pulmonary Function in Patients with Endobronchial Sarcoidosis

ABSTRACT Sixty-four patients with clinically, radiologically and histopathologically established sarcoidosis were studied by bronchoscopy and static and dynamic spirometry. Sarcoid bronchostenosis was established by bronchoscopy in nine patients. Twenty-one had bronchial sarcoidosis without stenosis and 34 had no evidence of bronchial sarcoidosis. Patients with sarcoid bronchostenosis showed a marked impairment of lung function, mostly with an obstructive pattern. There was no improvement in lung function after bronchodilatation in these patients. Obstructive spirometry pattern was also found in three patients with bronchial sarcoidosis stage II without stenosis. None of the patients without bronchial sarcoidosis had an obstructive pattern. All six patients with restrictive spirometry pattern in the two groups without bronchostenosis had stage II sarcoidosis. At follow-up, lung function had improved in five patients, four with sarcoid bronchostenosis and one patient with stage II without bronchial sarcoidosis. Four of these patients were treated with oral corticosteroids. When clinical signs of airway obstruction are present in patients with sarcoidosis, bronchial sarcoidosis with or without bronchostenosis should be suspected. When bronchial sarcoidosis is present, oral corticosteroid treatment should be tried.     

Right Heart Involvement in Patients with Sarcoidosis

The left ventricle (LV) is affected in 20–25% of patients with sarcoidosis and its involvement is associated with morbidity and mortality. However, effects of sarcoidosis on the right ventricle (RV) are not well documented. Our aims were to investigate the prevalence of RV dysfunction in patients with sarcoidosis and determine whether it is predominantly associated with direct cardiac involvement, severity of lung disease, or pulmonary hypertension (PH). We identified 50 patients with biopsy‐proven extra‐cardiac sarcoidosis and preserved LV function, who underwent echocardiography, pulmonary function (PF) testing, and cardiovascular magnetic resonance. RV function was quantified by free wall longitudinal strain. Tricuspid valve Doppler and estimated right atrial pressure were used to estimate systolic pulmonary artery pressure. Myocardial late gadolinium enhancement was considered diagnostic for cardiac sarcoidosis and assumed to involve both ventricles. Of the 50 patients, 28 (56%) had RV dysfunction, 4 with poorly defined PF status. Of the remaining 24 patients, 16 (67%) had lung disease, 8 (33%) had PH, and 10 (42%) had LV involvement. Ten patients had greater than one of these findings, and 4 had all 3. In contrast, in 4/24 patients (17%), RV dysfunction could not be explained by these mechanisms, despite severely reduced RV strain. In conclusion, RV dysfunction is common in patients with sarcoidosis and is usually associated with either direct LV involvement, lung disease, or PH, but may occur in the absence of these mechanisms, suggesting the possibility of isolated RV involvement and underscoring the need for imaging protocols that would include RV strain analysis.     

Autoantibodies against CD4- and CDS-Positive T Lymphocytes in HIV-Infected Hemophilia Patients

The presence of IgG, IgM, C3d, or gp120 on the surface of T lymphocytes was analyzed by flow cytometry in blood samples from 73 hemophilia patients and 56 healthy controls. IgG and IgM autoantibodies against CD4+ lymphocytes were found in HIV + patients but not in HIV-patients or healthy controls (p less than 0.001). IgM autoantibodies were more frequent than IgG autoantibodies. Autoantibody formation increased with disease progression. However, within the same disease risk category, patients with autoantibodies were not "more immunologically abnormal' than patients without autoantibodies. HIV + patients who possessed autoantibodies had similar CD4+ and CD8+ lymphocyte counts as HIV + patients without autoantibodies. There was no significant difference in the number of patients with abnormal CD4/CD8 ratios, serum neopterin levels, or in vitro responses to allogeneic stimulator cells or mitogens between autoantibody-positive or -negative patients of the same risk category. Our data suggest that autoantibodies against CD4+ lymphocytes may be helpful as indicators of disease progression, however, their immunopathogenetic role remains unclear.     

Antinuclear Autoantibodies in Patients with Inflammatory Bowel Disease (High Prevalence in First-Degree Relatives)

Crohn's disease and ulcerative colitis show afamilial aggregation. The role of antinuclearautoantibodies, which occur in both diseases, remains tobe defined. In 76 patients with Crohn's disease, 61patients with ulcerative colitis, 105 first-degreerelatives of patients with Crohn's disease, 101first-degree relatives of patients with ulcerativecolitis, and 40 healthy unrelated controls antinuclearautoantibodies were detected by indirect immunofluorescence.Existence of autoantibodies was correlated with clinicalfeatures. Eighteen percent of patients with Crohn'sdisease (14/76), 43% of patients with ulcerative colitis (26/61), 13% of relatives of patientswith Crohn's disease (14/105), 24% of relatives ofulcerative colitis patients (24/101), and 2% of thehealthy controls (1/40) were positive for antinuclear autoantibodies. The difference between controlsand patients and the first-degree relatives of patientswith ulcerative colitis, respectively, was statisticallysignificant (P 0.0144). In ulcerative colitis, the existence of antinuclear autoantibodies wasnegatively correlated with immunosuppressive therapy orextraintestinal manifestations (P = 0.0004 and 0.0273,respectively). Antinuclear autoantibodies may represent a factor disposing to the developmentof ulcerative colitis.     

Heart Rate Variability in Patients with Systemic Sarcoidosis

Purpose: The identification of subjects with systemic sarcoidosis at higher risk for sudden death is an unresolved issue. An influence of the autonomic activity on the genesis of ventricular arrhythmias was postulated. Heart rate variability (HRV) analysis provides a useful method to measure autonomic activity, and is a predictor of increased risk of death in various conditions. Therefore, the aim of the study was to evaluate HRV in patients with systemic sarcoidosis. Methods : The study included 35 patients with biopsy proven systemic sarcoidosis who were not taking antiarrhythmic medications. Thallium scintigraphy was performed to all patients with systemic sarcoidosis. The cardiac sarcoidosis was accepted in 16 patients as abnormal thallium scintigraphy and normal coronary arteriography. The time‐domain analysis of HRV was expressed as the standard deviation of all normal to normal NN intervals (SDNN) detected during 24‐hour Holter monitoring. Twenty‐four healthy subjects represented a control group for HRV analysis. Results: There were no differences in age (44 ± 13 years for cardiac sarcoidosis, 42 ± 15 years for noncardiac sarcoidosis, and 40 ± 10 years for control group; P = NS), sex (the ratio of female; 63%, 68%, and 55%, respectively; P = NS), and echocardiographic ejection fraction (63 ± 10%, 67 ± 8%, and 69 ± 6%, respectively; P = NS) among study groups. The mean SDNN value of the group with cardiac sarcoidosis was significantly lower than both the group with noncardiac sarcoidosis and the control group (72 ± 32 ms vs 110 ± 46 ms and 152 ± 36 ms; P < 0.05, respectively). Conclusion: HRV is decreased in patients with systemic sarcoidosis compared to the control group. This decreasing is more obvious in patients with cardiac sarcoidosis.     

Autoantibodies against phosphatidylserine-prothrombin complex in patients with systemic sclerosis

OBJECTIVE: To determine the frequency and clinical association of antiphosphatidylserine-prothrombin complex (PS/PT) antibody (Ab) in systemic sclerosis (SSc). METHODS: Anti-PS/PT IgG Ab was examined by enzyme linked immunosorbent assay (ELISA) in 112 patients with SSc. Thirty three healthy volunteers and 30 patients with systemic lupus erythematosus (SLE) were also investigated as controls. RESULTS: Anti-PS/PT Ab was detected in 18/112 (16%) patients with SSc and 10/30 (33%) patients with SLE, whereas it was not detected in any normal controls. Anti-PS/PT Ab was more frequently detected in patients with SSc with peripheral ischaemia and lung disease (pulmonary fibrosis and pulmonary hypertension) than in patients with SSc without the Ab. However, anti-PS/PT Ab was not associated with the severity of skin sclerosis. Importantly, two patients were negative for both lupus anticoagulant and Ab against cardiolipin beta(2)-glycoprotein I complex among six anti-PS/PT Ab positive patients with SSc and a thromboembolic episode. CONCLUSIONS: Anti-PS/PT Ab is associated with thromboembolism, peripheral ischaemia, and lung involvement in some patients with SSc. Examination of this Ab may be useful to recognise the risk of thromboembolism in patients with SSc.     

Characterization and Outcomes of SARS-CoV-2 Infection in Patients with Sarcoidosis

To analyze the clinical characteristics and outcomes of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in patients with sarcoidosis from a large multicenter cohort from Southern Europe and to identify the risk factors associated with a more complicated infection. We searched for patients with sarcoidosis presenting with SARS-CoV-2 infection (defined according to the European Centre for Disease Prevention and Control guidelines) among those included in the SarcoGEAS Registry, a nationwide, multicenter registry of patients fulfilling the American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and Other Granulomatous Disorders 1999 classification criteria for sarcoidosis. A 2:1 age-sex-matched subset of patients with sarcoidosis without SARS-CoV-2 infection was selected as control population. Forty-five patients with SARS-CoV-2 infection were identified (28 women, mean age 55 years). Thirty-six patients presented a symptomatic SARS-CoV-2 infection and 14 were hospitalized (12 required supplemental oxygen, 2 intensive care unit admission and 1 mechanical ventilation). Four patients died due to progressive respiratory failure. Patients who required hospital admission had an older mean age (64.9 vs. 51.0 years, p = 0.006), a higher frequency of baseline comorbidities including cardiovascular disease (64% vs. 23%, p = 0.016), diabetes mellitus (43% vs. 13%, p = 0.049) and chronic liver/kidney diseases (36% vs. 0%, p = 0.002) and presented more frequently fever (79% vs. 35%, p = 0.011) and dyspnea (50% vs. 3%, p = 0.001) in comparison with patients managed at home. Age- and sex-adjusted multivariate analysis identified the age at diagnosis of SARS-Cov-2 infection as the only independent variable associated with hospitalization (adjusted odds ratio 1.18, 95% conficence interval 1.04–1.35). A baseline moderate/severe pulmonary impairment in function tests was associated with a higher rate of hospitalization but the difference was not statistically significant (50% vs. 23%, p = 0.219). A close monitoring of SARS-CoV-2 infection in elderly patients with sarcoidosis, especially in those with baseline cardiopulmonary diseases and chronic liver or renal failure, is recommended. The low frequency of severe pulmonary involvement in patients with sarcoidosis from Southern Europe may explain the weak prognostic role of baseline lung impairment in our study, in contrast to studies from other geographical areas.     

Pancreatic Autoantibodies in Greek Patients with Inflammatory Bowel Disease

Pancreatic autoantibodies (PAbs) have been suggested as a specific but not sensitive marker for Crohn's disease (CD). The aim of this study was to assess the value of detecting PAbs in Greek patients with ulcerative colitis (UC) and CD. Sera were collected from 150 patients with IBD (73 with UC and 77 with CD), 31 cases with non-IBD intestinal inflammation, 16 cases with other autoimmune diseases, and 104 healthy controls. Determination of PAbs was performed by a standard indirect immunofluorescence technique. PAbs were detected in 18 of 73 (24.7%) samples from UC patients and in 32 of 77 (41.6%) samples from CD patients. The prevalence of positive PAbs was significantly higher in CD than in UC (P= 0.04). None of the 104 samples from healthy controls and the 31 cases with non-IBD intestinal inflammation had detectable PAbs. One patient with Sjogren's syndrome was PAbs positive. No association of PAbs with IBD activity, IBD localization, or medical treatment was found. Patients with stenotic CD had a significantly higher prevalence of PAbs positivity (60%) compared with patients with inflammatory (28.6%) and fistulizing (41.2%) disease (P= 0.02). The prevalence of PAbs in Greek CD patients was found to be similar to that in previous reports. In contrast to these studies we found also increased prevalence of PAbs in UC patients. These findings suggest that PAbs should be considered as a specific marker for IBD rather than for CD.     

38例胸内结节病的临床分析

目的提高对结节病的认识及诊断水平，减少误诊。方法回顾性分析了自1990-2005年38例住院患者诊断为胸内结节病的临床特点、确诊途径以及误诊情况。结果38例胸内结节病中Ⅰ期14例，Ⅱ期19例。Ⅲ期5例。临床表现以咳嗽、胸闷最多见。确诊途径以纵隔镜、胸腔镜及手术等检查阳性率最高。另外浅表淋巴结活检阳性率亦较高。25例患者被误诊，误诊为肺结核16例，肺癌5例，淋巴瘤1例，关节病2例．眼部疾病1例。结论结节病临床表现多样．易误诊或漏诊。确诊途径中以操作方便，创伤小且阳性率较高的浅表淋巴结活检推荐为首选；本病与结核、淋巴瘤及肺癌等鉴别困难。末明确诊断前应慎重治疗．