Anti-Ro52 Antibody as a Protective Factor for Pulmonary Fibrosis in Primary Sjögren's Syndrome

Background: Pulmonary fibrosis is common in primary Sjögren's syndrome (pSS)-related interstitial lung disease (ILD). However, research is lacking on the diagnostic immunological examination of pSS-related pulmonary fibrosis. Particularly, the value of detecting anti-Ro52 antibody in pulmonary fibrosis is unclear. Objective: To evaluate the potential diagnostic value of anti-SSA, anti-SSB, and anti-Ro52 autoantibodies as markers of pSS-related pulmonary fibrosis. Methods: One-hundred seventy-nine patients with pSS were analyzed retrospectively at our hospital. They were divided into the fibrosis and non-fibrosis groups. Pulmonary fibrosis was classified as mild, moderate, or severe based on the patients' computed tomography (CT) findings. Laboratory examinations, including anti-SSA, anti-SSB, and anti-Ro52 antibody evaluations, were performed. The influencing factors of pulmonary fibrosis were analyzed using logistic regression. Results: Chest CT revealed pulmonary fibrosis in 45 patients with pSS (25.1%). The positive rates of anti-SSA and anti-Ro52 antibodies in the fibrosis group were lower than in the non-fibrosis group (P=0.04, P=0.001). The frequency of anti-Ro52 antibody showed no significant differences between mild-to-moderate (53.8%) and severe (47.3%) pulmonary fibrosis. The anti-Ro52 antibody was identified as a potentially protective factor against pSS (P=0.041). Conclusions: Patients with pSS and pulmonary fibrosis had a low frequency of anti-SSA and anti-Ro52 antibodies. In patients with pSS and negative anti-Ro52 antibody, a chest CT is recommended to further understand the patients' condition.     

Postoperative Funktionalität und Aspekte zur Lebensqualität nach Penisbegradigung durch die Schröder-Essed-Technik

The Schröder-Essed technique is a standard technique for correction of penile curvature. Although it has been described as a simple and rapid technique offering low morbidity, no data are available on long-term follow-up and life quality of the operated patients. In a retrospective analysis we investigated functional results and life quality (LQ) using a detailed questionnaire. From January 1994 to January 1999, 40 patients (median age 26) underwent correction of penile curvature using a slightly modified Schröder-Essed technique. We used a self-established questionnaire investigating functional and cosmetic aspects and LQ. Complete follow-up data are available in 31/40 (77.5%) patients (congenital curvature, 19 patients; Peyronie's disease, 12 patients). Median follow-up is 22 months. Degree of angulation before surgery was estimated at < 45° in 5 patients, 45–90° in 22 patients, and >90° in 4 patients. In 21/31 patients (67.7%) cohabitation was not possible or discomfortable. Impaired LQ due to penile curvature was reported by 26/31 patients (83.8%) After surgery, the cosmetic result was considered as good or sufficient in 25 patients. Cohabitation was possible in 25/31 patients (80.6%). Penile shortening (> 2 cm) was reported by six patients. Significant improvement of life quality was reported by 15 patients. Among the 12 patients with Peyronie's disease, 6 mentioned postoperative impaired rigidity. In two patients with Peyronie's disease penile curvature recurred. This simple operation technique offers good functional and cosmetic results. Particularly patients with congenital curvature reported good or excellent results. Patients with Peyronie's disease have to be informed about the risk of disease recurrence. Other operation techniques should be considered for these patients.     

牛津分类与国际小儿肾脏病研究组分级在评价儿童紫癜性肾炎预后中的应用

目的探讨肾脏病理牛津分类(MESTC评分)与国际小儿肾脏病研究组(ISKDC)分级在评价儿童紫癜性肾炎临床病理表现、短期预后和临床转归中的价值。方法病例来自2018年1月至2018年12月期间于北京儿童医院肾病科住院、经肾脏活组织检查(肾活检)确诊为过敏性紫癜性肾炎的患儿。按照牛津分类与ISKDC分级对入选者肾活检标本进行病理评分。按照首发症状是否合并肾脏表现及MESTC评分、ISKDC分级分组,比较各组间患儿临床病理表现的差异。用非参数相关分析评估MESTC评分与ISKDC分级指标的相关性。采用Kaplan-Meier生存曲线及Log-rank检验比较两组患儿蛋白尿缓解的差异;单因素及多因素Cox回归方程法分析患儿蛋白尿缓解的影响因素。结果共纳入78例紫癜性肾炎患儿,男37例(47.4%),年龄(10.4±2.9)岁。依据MESTC评分与ISKDC分级分组的组间比较结果显示,内皮细胞增生(E1,P=0.008)、节段硬化(S1,P=0.015)及ISKDCⅢ级(P=0.041)组肾病水平蛋白尿患儿比例较E0、S0和ISKDCⅡ级组高;E1(P=0.015)、新月体形成(C1&C2,P=0.025)以及ISKDCⅢ级(P=0.017)组接受激素冲击治疗患儿比例高。Kaplan-Meier生存分析结果显示,C2组患儿较C0&C1组更难达到蛋白尿缓解(P=0.026)。多因素Cox回归模型分析结果提示,牛津分类评分为C2(HR=0.143,95%CI 0.020~1.046,P=0.055)可能是紫癜性肾炎患儿蛋白尿缓解的危险因素,P值为临界值。结论ISKDCⅢ级以及牛津分类中的E1、S1型紫癜性肾炎患儿更易表现为肾病水平蛋白尿。牛津分类中C2可能是患者短期蛋白尿缓解的危险因素。     

阴虚型和湿热型原发性干燥综合征的临床特点分析

目的:探讨阴虚型和湿热型原发性干燥综合征临床特点的差异。方法:分析277例阴虚型和湿热型原发性干燥综合征患者的临床资料,统计2组在性别、年龄、病史、系统累及、实验室指标、疾病活动等方面的差异。结果:2组患者在性别、烟酒史、家族史、抗核抗体阳性率、抗SSA抗体阳性率、抗SSB抗体阳性率、免疫球蛋白(IgG、IgM、IgA)、补体(C3、C4)、细胞因子[白细胞介素(IL)-1β、IL-2R、IL-6、IL-8、IL-10、肿瘤坏死因子-α(TNF-α)]、ESDDAI评分方面比较,差异无统计学意义(P>0.05)。但阴虚组患者平均年龄大于湿热组(P<0.05),总体病程长于湿热组(P<0.05),贫血的发生率高于湿热组(P<0.05),湿热组患者泌尿系统、神经系统、消化系统受累的发生率高于阴虚组(P<0.05),阴虚组患者的中医症状分级量化表评分高于湿热组(P<0.05)。结论:阴虚型和湿热型原发性干燥综合征在临床特点上存在一定差异。     

Optical coherence tomography angiography characteristics in Waldenström macroglobulinemia retinopathy: A case report

BACKGROUNDWaldenström macroglobulinemia (WM) is a distinct clinicopathologic entity characterized by the infiltration of the bone marrow by clonal lymphoplasmacytic cells that produce monoclonal immunoglobulin M (IgM) in the blood, and patients may present with symptoms related to the infiltration of the hematopoietic tissues or the effects of monoclonal IgM in the blood. Funduscopic abnormalities were noted in some of the patients due to hyperviscosity or other retinal lesions. Optical coherence tomography angiography (OCTA) as a non-invasive imaging tool can give qualitative and quantitative information about the status of retinal and choroidal vessels, which might be useful for diagnosing patients with WM-associated retinopathy.CASE SUMMARYThe patient was a 67-year-old man who presented with sudden visual disturbance in both eyes. Ophthalmic tests showed that best corrected visual acuity (BCVA) for this patient was 20/100 in the right eye and 20/1000 in the left eye. Fundus examination, optical coherence tomography (OCT), and OCTA revealed substantial bilateral optic disc edema, dilated and tortuous retinal veins, and diffuse intraretinal blot hemorrhages and edema which were consistent with bilateral central retinal vein occlusion (CRVO). Meanwhile, remarkable bilateral serous macular detachments (SMD) were noticed on OCT. Systemic examinations showed that the patient had anemia and extremely high level of monoclonal IgM and infiltration of clonal lymphoplasmacytic cells in bone marrow. The diagnosis of WM with hyperviscosity and retinopathy was made based on the clinical manifestation and laboratory findings. He was subsequently treated with intravitreal ranibizumab injection, plasmapheresis, and bortezomib plus rituximab with dexamethasone. Six months after treatments, the central macular volume decreased by 16.1% in the right eye and 28.6% in the left eye on OCT, and the patient’s BCVA was improved to 20/60 in the right eye and 20/400 in the left eye. Very good partial response was obtained after systemic treatment.CONCLUSIONWM may affect visual function and present as bilateral CRVO. OCTA can show characteristic changes in both retina and choroid vasculatures, which might be of great value for diagnosing or following patients with WM retinopathy. Intravitreal anti-vascular endothelial growth factor treatment combined with systemic therapy might be beneficial for WM patients with retinopathy (SMD and CRVO).