Objective: Serum from SSc patients was analyzed centrally to determine ANA patterns and extractable nuclear antigens (ENAs) between lcSSc and dcSSc and associations with organ involvement. Methods: 1145 SSc patients h...Objective: Serum from SSc patients was analyzed centrally to determine ANA patterns and extractable nuclear antigens (ENAs) between lcSSc and dcSSc and associations with organ involvement. Methods: 1145 SSc patients had ANA and ENA analyzed by indirect immunofluorescence on HEp-2 substrate at a screening serum dilution of 1/160. Most ENA antibodies [Sm. U1-RNP, Ro52, SS-A/Ro60, topoisomeraseI (Topo1), SS-B/La, chromatin, ribosomal P and Jo1] were measured by laser bead immunoassay;and RNA polymerase III (RNAP) by ELISA. Results: ANA was positive in 95% (same in lcSSc, and dcSSc). Centromere pattern was present in 34%, speckled 22%, nucleolar 18%, homogeneous and speckled (H&S) 16%, multiple nuclear dots 6%. Anti-centromere Ab (ACA) occurred in 46% of lcSSc and 11% of dcSSc (P = 0.0001). ENAs that differed between lcSSc and dcSSc subsets were Topo1 (OR 2.4, P = 0.0001) and RNAP (OR 5.6, P 0.0001) more common in dcSSc. Overall, 15% had positive Topo1;usually with a H&S pattern (67%);Topo1 was associated with ILD on CXR (OR 2.3;95% CI 1.5 - 3.5) and HRCT (OR 3.8;95% CI 1.8 - 8.2). RNAP occurred in 18.5% (35.4% in dcSSc vs. 8.9% in lcSSc). Scleroderma renal crisis (SRC) was 13 times more likely if RNAP positive;P = 0.0001. ACA was only weakly associated with sPAP > 50 mmHg (OR 1.8;95%CI 1.1 - 3.0). Conclusion: ANA homogeneous pattern alone is rare in SSc;ACA was significantly more common in lcSSc. Many ENAs are equal in lcSSc and dcSSc except RNAP and Topo1. RNAP has the highest OR of SRC. Topo1 is less strongly associated with ILD. Abstract word count: 249, Body word count 1246, Figures 2, Tables 2. Key Messages: 1) 95% of SSc has a positive ANA and ANA patterns in SSc include centromere, nucleolar, and homogeneous and speckled together;2) Most ENAs are equal in both dcSSc and lcSSc except anti RNA polymerase III and topoisomerase I;3) RNA polymerase III has the highest association (odds ratio) with scleroderma renal crisis, topoisomerase I is associated with interstitial lung disease;whereas anticentromere was not associated with elevated pulmonary arterial pressures on echocardiogram.展开更多
Aim: The aim of this study is to describe the clinical characteristics of patients with a diagnosis of systemic sclerosis who later suffer a stroke and to identify associations for this relationship. Background: Prior...Aim: The aim of this study is to describe the clinical characteristics of patients with a diagnosis of systemic sclerosis who later suffer a stroke and to identify associations for this relationship. Background: Prior studies have showed an increased risk of cardiovascular disease among patients with chronic inflammatory disorders, with chronic inflammation leading to atherosclerosis believed to be the culprit. Systemic sclerosis (SSc) is a chronic inflammatory disease characterized by diffuse fibrosis of the skin and internal organs. Previous studies have suggested a possible link between systemic sclerosis and macrovascular complications such as stroke. Methods: This is a retrospective chart review of patients treated within the University of Pennsylvania Health System from October 2015 to April 2019 with a diagnosis of SSc. Using ICD10 codes, we identified a cohort of SSc patients who suffered a stroke. Information regarding demographics and stroke risk factors were gathered from the charts of patients with a diagnosis of both SSc plus stroke and compared to a control group of randomly selected patients with SSc who never suffered a stroke. Continuous variables were conveyed using a mean plus a standard deviation. A two-sample t-test was used to compare the two groups of patients. Qualitative variables were compared using a two-tailed Fisher’s exact test. Results: Based on a large cohort of SSc patients (n = 2080) followed between October 2015 and April 2019, we identified 36 SSc patients who developed a subsequent stroke (1.7% of cohort). When looking at risk factors for stroke in SSc patients, we identified hypertension and atrial fibrillation to be associated with the diagnosis of stroke in such patients. Specifically, 28 of the 36 patients with both SSc and stroke also had a diagnosis of hypertension while in the control group, only 17 of 36 patients had hypertension. Atrial fibrillation was seen in 9 of 36 patients with both SSc and CVA while it was seen in only 2 of 36 patients in the control group. Conclusions: This case control study demonstrated that the presence of hypertension and atrial fibrillation had a statistically significant association with the diagnosis of CVA in patients with SSc.展开更多
Objective: The aim of this study was to describe the epidemiological, clinical and therapeutic features of systemic scleroderma at Cocody UTH. Methodology: We conducted a retrospective and descriptive study over a per...Objective: The aim of this study was to describe the epidemiological, clinical and therapeutic features of systemic scleroderma at Cocody UTH. Methodology: We conducted a retrospective and descriptive study over a period of 10 years (September 15, 2008 to April 15, 2019) on the files of patients hospitalized for systemic scleroderma in the rheumatology unit of the UTH of Cocody. We used the classification criteria of the American Society of Rheumatology (1980) to retain the diagnosis. Results: Nineteen patients’ files had been collected, representing a hospital frequency of 0.32%. The average age was 37.25 ± 13.82 years old. There were 15 women and 4 men. The average consultation time was 26.44 months. The mode of revelation of the disease was mostly cutaneous and articular. All patients had cutaneous sclerosis (average Rodnan score = 27.63/11.61 (min = 4, max = 49).) Scleroderma was diffuse in 70.59% of cases;a Raynaud’s phenomenon was seen in 47.37%. The main clinical manifestations were: cutaneous (100%), articular (89.47%), pulmonary (57.89%) and digestive (63.16%). No renal damage was found. Pulmonary fibrosis (5 cases), pulmonary arterial hypertension (3 cases) and pericardial effusion (2 cases) were sometimes founded in explorations. The positivity of antinuclear antibodies (ANA) was seen in 72% of patients and anti scl70 antibodies in 42.85%. The treatment included corticosteroids and immunosuppressants, respectively used in 84.2% and 63.16% of cases. The outcome was marked by 5 cases of death attributed to respiratory distress. Conclusion: Systemic scleroderma seems to be a very rare condition in Ivorian rheumatology milieu. The main systemic manifestations were digestive and pulmonary. Treatment was very often symptomatic sometimes associated with D-penicillamine.展开更多
Background:Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin and organs,marked changes in microvascular structure,cellular and humoral immune disorders.Renal involvement is more f...Background:Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin and organs,marked changes in microvascular structure,cellular and humoral immune disorders.Renal involvement is more frequent and mainly characterized by moderate proteinuria,elevated serum creatinine levels,and hypertension.The most common kidney involvement in SSc is scleroderma renal crisis(SRC)that is fatal without prompt intervention.Case report:A 52-year-old Caucasian male with known diffuse cutaneous systemic sclerosis was hospitalized with communityacquired pneumonia.On the fifth day after appropriate antibiotic therapy and 60 mg/day methylprednisolone,decreased urine output,arterial hypertension,decreased renal function and pulmonary edema developed.The patient was diagnosed with a scleroderma renal crisis.Emergency hemodialysis was applied to the patient,and captopril 6×25 mg/day and nifedipine 2*60 mg/day treatment were given.He received a routine hemodialysis program for about three months.The hemodialysis program was terminated when the patient’s urine quality and quantity increased.Conclusions:SRC,characterized by malignant hypertension,azotemia,microangiopathic hemolytic anemia,and kidney failure,is one of the most important complications of systemic sclerosis with a poor prognosis without prompt intervention.Steroid use is one of the important risk factors that precipitate SRC development.With angiotensin-converting enzyme inhibitors,survival increased after SRC,the need for dialysis decreased,and usually allowed the discontinuation of dialysis treatment within about 6-18 months.Suspicion of SRC in the presence of the above-mentioned findings in patients with a diagnosis or suspected systemic sclerosis can be considered the most important treatment step.展开更多
Evidence suggests that B cells may participate in the fibrotic process Based on this experimental evidence, treatment with rituximab(RTX) has been tried in systemic sclerosis(SSc) with promising results. In a randomiz...Evidence suggests that B cells may participate in the fibrotic process Based on this experimental evidence, treatment with rituximab(RTX) has been tried in systemic sclerosis(SSc) with promising results. In a randomized controlled study from our research group it was shown that treatment with 2 courses of RTX leads to a significant improvement of lung function at 1 year compared to baseline. All relevant studies have so far reported clinical and/or histologic improvement of skin fibrosis something that adds further evidence in favor of a disease modifying role of RTX in SSc. It is more than obvious that novel therapies for SSc-associated lung disease are needed. A large scale, randomized, controlled study assessing the efficacy of RTX in SSc associated interstitial lung disease is warranted. If RTX turns out to be effective it would be a major therapeutic advance in SSc since it can be administered on a long term basis due to its acceptable safety profile.展开更多
The increased awareness of systemic sclerosis(SS)and its pathogenetic background made the management of this disease more amenable than previously thought.However,scleroderma renal crisis(SRC)is a rarely seen as an as...The increased awareness of systemic sclerosis(SS)and its pathogenetic background made the management of this disease more amenable than previously thought.However,scleroderma renal crisis(SRC)is a rarely seen as an associated disorder that may involve 2%-15%of SS patients.Patients presented with earlier,rapidly progressing,diffuse cutaneous SS disease,mostly in the first 3-5 years after non-Raynaud clinical manifestations,are more vulnerable to develop SRC.SRC comprises a collection of acute,mostly symptomatic rise in blood pressure,elevation in serum creatinine concentrations,oliguria and thrombotic microangiopathy in almost 50%of cases.The advent of the antihypertensive angiotensin converting enzyme inhibitors in 1980 was associated with significant improvement in SRC prognosis.In a scleroderma patient maintained on regular dialysis;every effort should be exerted to declare any possible evidence of renal recovery.A given period of almost two years has been suggested prior to proceeding in a kidney transplant(KTx).Of note,SS patients on dialysis have the highest opportunity of renal recovery and withdrawal from dialysis as compared to other causes of end-stage renal disease(ESRD).KTx that is the best well-known therapeutic option for ESRD patients can also be offered to SS patients.Compared to other primary renal diseases,SS-related ESRD was considered for a long period of poor patient and allograft survivals.Pulmonary involvement in an SS patient is considered a strong post-transplant independent risk factor of death.Recurrence of SRC after transplantation has been observed in some patients.However,an excellent post-transplant patient and graft outcome have been recently reported.Consequently,the absence of extrarenal manifestations in an SS-induced ESRD patient can be accepted as a robust indicator for a successful KTx.展开更多
Systemic sclerosis(SSc)is a rare connective tissue disorder.It is a chronic multisystem disease characterized by vascular dysfunction and progressive fibrosis of tissue,with skin hardening and thickening(known as scle...Systemic sclerosis(SSc)is a rare connective tissue disorder.It is a chronic multisystem disease characterized by vascular dysfunction and progressive fibrosis of tissue,with skin hardening and thickening(known as scleroderma)being the hallmark of the disease.It tends to affect females more than males and has a higher prevalence in African American population with earlier onset and more severe disease.While scleroderma can be a manifestation of conditions other than SSC,the presence of skin thickening of the fingers,extending proximally to metacarpophalangeal joints is sufficient to classify a patient as having SSc.SSc treatment is challenging given the heterogeneity of the disease,multiple organ involvement,different subtypes and poorly understood etiology and pathogenesis.Yet,systemic immunosuppressive therapy is often the treatment of choice.Here we present a 60-year-old white female who developed skin thickening of her fingers extending to the forearms and of her proximal thighs after being treated with pembrolizumab for metastatic non-small cell lung cancer.It was difficult to determine internal organ involvement given her history of metastatic lung cancer,but scleroderma specific autoantibodies were negative.Her symptoms improved after treatment with methotrexate and stopping pembrolizumab.This is one of the first case reports of scleroderma secondary to pembrolizumab.展开更多
Scleroderma is a rare disease with two primary forms: localized scleroderma (LS) and systemic sclerosis (SSc). Both are chronic conditions that can manifest in various patterns (subtypes) and are linked to extracutane...Scleroderma is a rare disease with two primary forms: localized scleroderma (LS) and systemic sclerosis (SSc). Both are chronic conditions that can manifest in various patterns (subtypes) and are linked to extracutaneous involvement in pediatric patients. Juvenile SSc poses a higher risk of morbidity and mortality, with patients facing life-threatening complications such as lung, heart, and visceral organ fibrosis, and vasculopathy. In contrast, mortality is extremely rare in juvenile LS, but patients are susceptible to significant morbidity, leading to severe disfigurement and functional impairment. Treatment for scleroderma aims to control inflammation and address specific issues. An early diagnosis significantly enhances the overall outcome. This study conducts a retrospective descriptive analysis aiming to document the clinical manifestations, management approaches, and outcomes of systemic sclerosis in a cohort of nine children receiving treatment for juvenile systemic sclerosis at Pediatric B department of Mohammed VI University, Hospital Center in Marrakech, Morocco.展开更多
Anti-RNA Polymerase III has been recognized as an important autoantibody in Systemic Sclerosis and it is now included in the 2013 ACR/EULAR classification criteria for Systemic Sclerosis. With this manuscript we attem...Anti-RNA Polymerase III has been recognized as an important autoantibody in Systemic Sclerosis and it is now included in the 2013 ACR/EULAR classification criteria for Systemic Sclerosis. With this manuscript we attempt to review the current data on anti-RNA polymerase II as it relates to Systemic Sclerosis.展开更多
Introduction: Cutaneous manifestations of systemic sclerosis (SSc) include skin ulceration;4% - 12% of patients with SSc develop lower extremity ulcers of various etiologies. Limited data, significant morbidity, and s...Introduction: Cutaneous manifestations of systemic sclerosis (SSc) include skin ulceration;4% - 12% of patients with SSc develop lower extremity ulcers of various etiologies. Limited data, significant morbidity, and substantial cost of wound care led us to undertake this study to describe and identify risk factors. Methods: After Institutional Review Board approval, we identified 30 patients with SSc and lower extremity ulcers over a 10-year period at a single center with an SSc clinic, which were included in a descriptive analysis. Results: Median age of onset of lower extremity ulcers was 59.5 years (range 20 - 84). Ninety percent of patients were female, 60% were Caucasian, 63% had limited SSc, 13% diffuse SSc and 23% an overlap syndrome. Immunomodulators or steroids were prescribed in 53%;hypercoagulable state identified in 16%. Ulcers were attributed to venous stasis (27%), SSc (20%), trauma (20%), arterial disease (17%), and multifactorial/unknown (17%). In patients with ulcers attributed to SSc, age at onset was lower (45.5 vs 59.5 years). Biopsies generally did not contribute to management. Multidisciplinary treatment was routine;20% required amputation, 10% endovascular intervention, 20% frequent surgical debridement, 10% hyperbaric oxygen, 26% local treatment and antibiotics and 13% received immunosuppression for wound treatment. Conclusion: Lower extremity ulcers are a serious clinical problem in patients with SSc. The clinical exam, venous dopplers, ankle-brachial indices and assessment of vascular risk factors helped define causality. In younger patients, ulcers were more frequently attributed to SSc and these patients were more likely to be on immunosuppressants/DMARDS, possibly indicating severe phenotype of SSc.展开更多
Objective; To study the characteristics and tendency of incidence of patients with respiratory system tumors during the past 23 y in Tianjin. Methods: All data in our research was obtained from the surgical pathology...Objective; To study the characteristics and tendency of incidence of patients with respiratory system tumors during the past 23 y in Tianjin. Methods: All data in our research was obtained from the surgical pathology files of Department of Pathology of the general and the Second Hospitals of Tianjin Medical University between 1981 and 2003. All data was analyzed by Spss 11.5 statistics program. The comparisons were made by u-test, P〈0.05 was considered as significant. Results: 1. The detection rate of malignant tumors is significantly higher than that of benign tumors (U=52.68, p=0.000) in respiratory system. 2. The common sites of benign tumors are nose and pharynx, but the common sites of malignant tumors are lung and larynx. 3. The incidence of benign tumors generally peaks between the ages of 40 and 50, but the incidence of malignant tumor generally peaks between the ages of 50 and 60. 4. The commonest histological type of malignant tumors is squamous cell carcinoma, but the commonest histological type of benign tumors is papilloma. 5. The detection rate of malignant lung tumors steadily increased between 1981 and 1999 and increased sharply from 1999 to 2003, but the detection rate of malignant Nasopharyngeal tumors steadily decreased from 1981 to 2003. Between 1981 and 1997, the detection rate of malignant laryngeal tumors steadily increased, followed by a decrease between 1997 and 2003. Conclusion: The detection rate of malignant respiratory system tumors especially lung cancer is gradually increasing. Therefore early prevention and treatment are critical to patients' prognosis.展开更多
Cancer continues to be a major health problem in the world. During 2018, there were 9.6 million deaths and 18.1 million new cases registered across the globe. It is estimated that one in 5 men and one in 6 women will ...Cancer continues to be a major health problem in the world. During 2018, there were 9.6 million deaths and 18.1 million new cases registered across the globe. It is estimated that one in 5 men and one in 6 women will develop cancer during their lifetime. This disease kills one in 8 men and one in 11 women. Developing countries have seen an increase in cancer occurrence and change in type of cancers due to change in social and economic conditions. The objective of this research was to further evaluate 1659 specimens for anatomical pathology testing. The data was collected from the records of the pathological anatomy laboratory at the Mohamed V Hospital in Meknes, Morocco. It is one-year study period (January-December 2013). Results of the anatomy pathology exam showed cancer positive for 9.6% of specimens;inconclusive for 1%;and negative for 66.2%. For all specimens, the most analyzed organs are breast with 29.7%;skin with 21.3%;cervical with 2.6%. For cancer patients, the most affected organs are skin (21.3%) and breast (29.7%). For cancer patients, Neoplasm is present in 9.3% of patients. Patients with metastatic cancer represent 0.3%. Patients with non-neoplastic lesions represent 66.2%. 1% of patients represent uncertain results. Additional immunohistochemistry research is needed before conclusive recommendations are made.展开更多
Objective:To analyze the clinical and laboratory indices of patients with lupus nephritis(LN)of different pathological types and explore the related factors of LN pathological classification,it is helpful to grasp the...Objective:To analyze the clinical and laboratory indices of patients with lupus nephritis(LN)of different pathological types and explore the related factors of LN pathological classification,it is helpful to grasp the timing of renal biopsy.Methods:The clinical manifestations,laboratory parameters and renal pathological types of LN patients in recent 20 years were analyzed retrospectively by SPSS 26.0 software.Results:In this study,the first three pathological types were V,IV,V+IV;latent nephritis was common in type II and V;nephritic syndrome was common in type V;nephrotic syndrome was common in type V+IV;chronic renal insufficiency group was mostly type IV;pathological types were correlated with serum creatinine,C3,albumin and erythrocyte sedimentation rate(r=0.315,P<0.001),and serum creatinine was moderately correlated(r=0.315,P<0.001);AI,CI and SLEDAI scores were significantly different among LN patients of different pathological types.Conclusion:LN is closely related to clinical pathology,clinical manifestations,comprehensive analysis of laboratory indicators and SLEDAI score to make a preliminary prediction of LN pathological type,help to initially assess the severity of pathology,improve the timing of renal biopsy implementation,optimize the timing of treatment.展开更多
Scleroderma is a chronic connective tissue disease characterized by inflammation,vascular injury,and progressive skin fibrosis,resulting in significant aesthetic and functional impairments for patients.Current therapi...Scleroderma is a chronic connective tissue disease characterized by inflammation,vascular injury,and progressive skin fibrosis,resulting in significant aesthetic and functional impairments for patients.Current therapies are limited and insufficiently treat the cutaneous manifestations of scleroderma.Autologous fat transfer(AFT)is a surgical technique that has been utilized for many decades for facial rejuvenation.The adipose stem cells(ASCs)present in fat grafts have also shown significant promise for their anti-inflammatory and regenerative properties.Recently,AFT has been repurposed to treat the skin manifestations of systemic sclerosis and localized scleroderma.Studies suggest that AFT in scleroderma patients improves mouth and hand functions,Raynaud’s symptoms,and digital ulcerations.AFT is a safe procedure with rare postoperative complications,making it a promising intervention for the treatment of scleroderma.Further studies are required to better characterize the influence of fat grafts on the recipient site and to establish standards for fat transfer in fibrotic skin diseases.展开更多
目的评估成人胶质母细胞瘤(GBM)脑脊髓神经轴外全身转移的临床及分子病理特征。方法在“PubMed”“Embase”和“Web of Science”数据库中检索截至2023年12月31日报道的发生全身转移的成人GBM病例,收集汇总其临床特征,同时汇总分析报道...目的评估成人胶质母细胞瘤(GBM)脑脊髓神经轴外全身转移的临床及分子病理特征。方法在“PubMed”“Embase”和“Web of Science”数据库中检索截至2023年12月31日报道的发生全身转移的成人GBM病例,收集汇总其临床特征,同时汇总分析报道的分子病理结果以评估转移发生的可能机制。结果通过数据库检索纳入1928年-2023年的113篇文献,共147例全身转移病例。男性多于女性,男女比例为2.3∶1;中位年龄45岁;转移灶几乎可见于全身各个部位,其中常见的转移部位为骨、肺及淋巴结;自GBM初次诊断至转移发生的中位间隔约10个月;自转移发生至患者死亡的中位间隔约4.5个月;中位总体生存约14.8个月。多因素分析显示年龄≤40岁、全切除以及未合并颅内复发与患者生存显著正相关。约39例患者(39/147,26.5%)报道了基因检测的结果。不同的研究所检测的基因项目存在区别,而且不同病例之间检测的基因结果呈现明显异质性。从报道的数量及阳性比例来看,异柠檬酸脱氢酶(IDH)野生型(阳性率27/30)、TERT启动子突变(阳性率11/13)、PTEN突变(阳性率10/11)、TP53突变(阳性率10/13)和RB1突变(阳性率8/9)为相对常见的基因突变。结论成人GBM脑脊髓神经轴外全身转移罕见,年轻患者相对多见,颅内病变的复发或进展仍为决定患者生存的关键因素。转移的发生应为多克隆基因突变进展的结果,其中,促增殖、促侵袭类基因改变例如癌基因或抑癌基因突变以及上皮间质转化类相关基因可能发挥了重要作用。展开更多
目的观察卵巢-附件报告和数据系统超声2022版(O-RADS US v2022)及其联合恶性风险指数4(RMI4)鉴别附件良、恶性肿瘤的价值。方法回顾性分析126例手术病理诊断为附件肿瘤患者,根据O-RADS US v2022将1~3类归为良性病变、4~5类归为恶性病变,...目的观察卵巢-附件报告和数据系统超声2022版(O-RADS US v2022)及其联合恶性风险指数4(RMI4)鉴别附件良、恶性肿瘤的价值。方法回顾性分析126例手术病理诊断为附件肿瘤患者,根据O-RADS US v2022将1~3类归为良性病变、4~5类归为恶性病变,以450为RMI4分类的临界值,基于二者进行联合分类。以病理结果为金标准,绘制受试者工作特征(ROC)曲线,计算曲线下面积(AUC),评估单一O-RADS US v2022、RMI4及其联合鉴别附件良、恶性肿瘤的效能。结果126例附件肿瘤中,良性94例、恶性32例。O-RADS US v2022鉴别附件良、恶性肿瘤的敏感度、特异度、准确率及AUC分别为78.13%、80.85%和80.16%、0.795,RMI4分别为71.88%、84.04%和80.95%、0.780;二者联合的特异度及准确率(93.62%、92.06%)均高于单一O-RADS US v2022(χ^(2)=7.322、5.967,P=0.007、0.015)或RMI4(χ^(2)=4.625、5.331,P=0.032、0.021),而敏感度及AUC(87.50%、0.906)差异均无统计学意义(P均>0.05)。结论O-RADS US v2022能有效鉴别附件良、恶性肿瘤,联合RMI4可提高鉴别特异度及准确率。展开更多
文摘Objective: Serum from SSc patients was analyzed centrally to determine ANA patterns and extractable nuclear antigens (ENAs) between lcSSc and dcSSc and associations with organ involvement. Methods: 1145 SSc patients had ANA and ENA analyzed by indirect immunofluorescence on HEp-2 substrate at a screening serum dilution of 1/160. Most ENA antibodies [Sm. U1-RNP, Ro52, SS-A/Ro60, topoisomeraseI (Topo1), SS-B/La, chromatin, ribosomal P and Jo1] were measured by laser bead immunoassay;and RNA polymerase III (RNAP) by ELISA. Results: ANA was positive in 95% (same in lcSSc, and dcSSc). Centromere pattern was present in 34%, speckled 22%, nucleolar 18%, homogeneous and speckled (H&S) 16%, multiple nuclear dots 6%. Anti-centromere Ab (ACA) occurred in 46% of lcSSc and 11% of dcSSc (P = 0.0001). ENAs that differed between lcSSc and dcSSc subsets were Topo1 (OR 2.4, P = 0.0001) and RNAP (OR 5.6, P 0.0001) more common in dcSSc. Overall, 15% had positive Topo1;usually with a H&S pattern (67%);Topo1 was associated with ILD on CXR (OR 2.3;95% CI 1.5 - 3.5) and HRCT (OR 3.8;95% CI 1.8 - 8.2). RNAP occurred in 18.5% (35.4% in dcSSc vs. 8.9% in lcSSc). Scleroderma renal crisis (SRC) was 13 times more likely if RNAP positive;P = 0.0001. ACA was only weakly associated with sPAP > 50 mmHg (OR 1.8;95%CI 1.1 - 3.0). Conclusion: ANA homogeneous pattern alone is rare in SSc;ACA was significantly more common in lcSSc. Many ENAs are equal in lcSSc and dcSSc except RNAP and Topo1. RNAP has the highest OR of SRC. Topo1 is less strongly associated with ILD. Abstract word count: 249, Body word count 1246, Figures 2, Tables 2. Key Messages: 1) 95% of SSc has a positive ANA and ANA patterns in SSc include centromere, nucleolar, and homogeneous and speckled together;2) Most ENAs are equal in both dcSSc and lcSSc except anti RNA polymerase III and topoisomerase I;3) RNA polymerase III has the highest association (odds ratio) with scleroderma renal crisis, topoisomerase I is associated with interstitial lung disease;whereas anticentromere was not associated with elevated pulmonary arterial pressures on echocardiogram.
文摘Aim: The aim of this study is to describe the clinical characteristics of patients with a diagnosis of systemic sclerosis who later suffer a stroke and to identify associations for this relationship. Background: Prior studies have showed an increased risk of cardiovascular disease among patients with chronic inflammatory disorders, with chronic inflammation leading to atherosclerosis believed to be the culprit. Systemic sclerosis (SSc) is a chronic inflammatory disease characterized by diffuse fibrosis of the skin and internal organs. Previous studies have suggested a possible link between systemic sclerosis and macrovascular complications such as stroke. Methods: This is a retrospective chart review of patients treated within the University of Pennsylvania Health System from October 2015 to April 2019 with a diagnosis of SSc. Using ICD10 codes, we identified a cohort of SSc patients who suffered a stroke. Information regarding demographics and stroke risk factors were gathered from the charts of patients with a diagnosis of both SSc plus stroke and compared to a control group of randomly selected patients with SSc who never suffered a stroke. Continuous variables were conveyed using a mean plus a standard deviation. A two-sample t-test was used to compare the two groups of patients. Qualitative variables were compared using a two-tailed Fisher’s exact test. Results: Based on a large cohort of SSc patients (n = 2080) followed between October 2015 and April 2019, we identified 36 SSc patients who developed a subsequent stroke (1.7% of cohort). When looking at risk factors for stroke in SSc patients, we identified hypertension and atrial fibrillation to be associated with the diagnosis of stroke in such patients. Specifically, 28 of the 36 patients with both SSc and stroke also had a diagnosis of hypertension while in the control group, only 17 of 36 patients had hypertension. Atrial fibrillation was seen in 9 of 36 patients with both SSc and CVA while it was seen in only 2 of 36 patients in the control group. Conclusions: This case control study demonstrated that the presence of hypertension and atrial fibrillation had a statistically significant association with the diagnosis of CVA in patients with SSc.
文摘Objective: The aim of this study was to describe the epidemiological, clinical and therapeutic features of systemic scleroderma at Cocody UTH. Methodology: We conducted a retrospective and descriptive study over a period of 10 years (September 15, 2008 to April 15, 2019) on the files of patients hospitalized for systemic scleroderma in the rheumatology unit of the UTH of Cocody. We used the classification criteria of the American Society of Rheumatology (1980) to retain the diagnosis. Results: Nineteen patients’ files had been collected, representing a hospital frequency of 0.32%. The average age was 37.25 ± 13.82 years old. There were 15 women and 4 men. The average consultation time was 26.44 months. The mode of revelation of the disease was mostly cutaneous and articular. All patients had cutaneous sclerosis (average Rodnan score = 27.63/11.61 (min = 4, max = 49).) Scleroderma was diffuse in 70.59% of cases;a Raynaud’s phenomenon was seen in 47.37%. The main clinical manifestations were: cutaneous (100%), articular (89.47%), pulmonary (57.89%) and digestive (63.16%). No renal damage was found. Pulmonary fibrosis (5 cases), pulmonary arterial hypertension (3 cases) and pericardial effusion (2 cases) were sometimes founded in explorations. The positivity of antinuclear antibodies (ANA) was seen in 72% of patients and anti scl70 antibodies in 42.85%. The treatment included corticosteroids and immunosuppressants, respectively used in 84.2% and 63.16% of cases. The outcome was marked by 5 cases of death attributed to respiratory distress. Conclusion: Systemic scleroderma seems to be a very rare condition in Ivorian rheumatology milieu. The main systemic manifestations were digestive and pulmonary. Treatment was very often symptomatic sometimes associated with D-penicillamine.
文摘Background:Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin and organs,marked changes in microvascular structure,cellular and humoral immune disorders.Renal involvement is more frequent and mainly characterized by moderate proteinuria,elevated serum creatinine levels,and hypertension.The most common kidney involvement in SSc is scleroderma renal crisis(SRC)that is fatal without prompt intervention.Case report:A 52-year-old Caucasian male with known diffuse cutaneous systemic sclerosis was hospitalized with communityacquired pneumonia.On the fifth day after appropriate antibiotic therapy and 60 mg/day methylprednisolone,decreased urine output,arterial hypertension,decreased renal function and pulmonary edema developed.The patient was diagnosed with a scleroderma renal crisis.Emergency hemodialysis was applied to the patient,and captopril 6×25 mg/day and nifedipine 2*60 mg/day treatment were given.He received a routine hemodialysis program for about three months.The hemodialysis program was terminated when the patient’s urine quality and quantity increased.Conclusions:SRC,characterized by malignant hypertension,azotemia,microangiopathic hemolytic anemia,and kidney failure,is one of the most important complications of systemic sclerosis with a poor prognosis without prompt intervention.Steroid use is one of the important risk factors that precipitate SRC development.With angiotensin-converting enzyme inhibitors,survival increased after SRC,the need for dialysis decreased,and usually allowed the discontinuation of dialysis treatment within about 6-18 months.Suspicion of SRC in the presence of the above-mentioned findings in patients with a diagnosis or suspected systemic sclerosis can be considered the most important treatment step.
文摘Evidence suggests that B cells may participate in the fibrotic process Based on this experimental evidence, treatment with rituximab(RTX) has been tried in systemic sclerosis(SSc) with promising results. In a randomized controlled study from our research group it was shown that treatment with 2 courses of RTX leads to a significant improvement of lung function at 1 year compared to baseline. All relevant studies have so far reported clinical and/or histologic improvement of skin fibrosis something that adds further evidence in favor of a disease modifying role of RTX in SSc. It is more than obvious that novel therapies for SSc-associated lung disease are needed. A large scale, randomized, controlled study assessing the efficacy of RTX in SSc associated interstitial lung disease is warranted. If RTX turns out to be effective it would be a major therapeutic advance in SSc since it can be administered on a long term basis due to its acceptable safety profile.
文摘The increased awareness of systemic sclerosis(SS)and its pathogenetic background made the management of this disease more amenable than previously thought.However,scleroderma renal crisis(SRC)is a rarely seen as an associated disorder that may involve 2%-15%of SS patients.Patients presented with earlier,rapidly progressing,diffuse cutaneous SS disease,mostly in the first 3-5 years after non-Raynaud clinical manifestations,are more vulnerable to develop SRC.SRC comprises a collection of acute,mostly symptomatic rise in blood pressure,elevation in serum creatinine concentrations,oliguria and thrombotic microangiopathy in almost 50%of cases.The advent of the antihypertensive angiotensin converting enzyme inhibitors in 1980 was associated with significant improvement in SRC prognosis.In a scleroderma patient maintained on regular dialysis;every effort should be exerted to declare any possible evidence of renal recovery.A given period of almost two years has been suggested prior to proceeding in a kidney transplant(KTx).Of note,SS patients on dialysis have the highest opportunity of renal recovery and withdrawal from dialysis as compared to other causes of end-stage renal disease(ESRD).KTx that is the best well-known therapeutic option for ESRD patients can also be offered to SS patients.Compared to other primary renal diseases,SS-related ESRD was considered for a long period of poor patient and allograft survivals.Pulmonary involvement in an SS patient is considered a strong post-transplant independent risk factor of death.Recurrence of SRC after transplantation has been observed in some patients.However,an excellent post-transplant patient and graft outcome have been recently reported.Consequently,the absence of extrarenal manifestations in an SS-induced ESRD patient can be accepted as a robust indicator for a successful KTx.
文摘Systemic sclerosis(SSc)is a rare connective tissue disorder.It is a chronic multisystem disease characterized by vascular dysfunction and progressive fibrosis of tissue,with skin hardening and thickening(known as scleroderma)being the hallmark of the disease.It tends to affect females more than males and has a higher prevalence in African American population with earlier onset and more severe disease.While scleroderma can be a manifestation of conditions other than SSC,the presence of skin thickening of the fingers,extending proximally to metacarpophalangeal joints is sufficient to classify a patient as having SSc.SSc treatment is challenging given the heterogeneity of the disease,multiple organ involvement,different subtypes and poorly understood etiology and pathogenesis.Yet,systemic immunosuppressive therapy is often the treatment of choice.Here we present a 60-year-old white female who developed skin thickening of her fingers extending to the forearms and of her proximal thighs after being treated with pembrolizumab for metastatic non-small cell lung cancer.It was difficult to determine internal organ involvement given her history of metastatic lung cancer,but scleroderma specific autoantibodies were negative.Her symptoms improved after treatment with methotrexate and stopping pembrolizumab.This is one of the first case reports of scleroderma secondary to pembrolizumab.
文摘Scleroderma is a rare disease with two primary forms: localized scleroderma (LS) and systemic sclerosis (SSc). Both are chronic conditions that can manifest in various patterns (subtypes) and are linked to extracutaneous involvement in pediatric patients. Juvenile SSc poses a higher risk of morbidity and mortality, with patients facing life-threatening complications such as lung, heart, and visceral organ fibrosis, and vasculopathy. In contrast, mortality is extremely rare in juvenile LS, but patients are susceptible to significant morbidity, leading to severe disfigurement and functional impairment. Treatment for scleroderma aims to control inflammation and address specific issues. An early diagnosis significantly enhances the overall outcome. This study conducts a retrospective descriptive analysis aiming to document the clinical manifestations, management approaches, and outcomes of systemic sclerosis in a cohort of nine children receiving treatment for juvenile systemic sclerosis at Pediatric B department of Mohammed VI University, Hospital Center in Marrakech, Morocco.
文摘Anti-RNA Polymerase III has been recognized as an important autoantibody in Systemic Sclerosis and it is now included in the 2013 ACR/EULAR classification criteria for Systemic Sclerosis. With this manuscript we attempt to review the current data on anti-RNA polymerase II as it relates to Systemic Sclerosis.
文摘Introduction: Cutaneous manifestations of systemic sclerosis (SSc) include skin ulceration;4% - 12% of patients with SSc develop lower extremity ulcers of various etiologies. Limited data, significant morbidity, and substantial cost of wound care led us to undertake this study to describe and identify risk factors. Methods: After Institutional Review Board approval, we identified 30 patients with SSc and lower extremity ulcers over a 10-year period at a single center with an SSc clinic, which were included in a descriptive analysis. Results: Median age of onset of lower extremity ulcers was 59.5 years (range 20 - 84). Ninety percent of patients were female, 60% were Caucasian, 63% had limited SSc, 13% diffuse SSc and 23% an overlap syndrome. Immunomodulators or steroids were prescribed in 53%;hypercoagulable state identified in 16%. Ulcers were attributed to venous stasis (27%), SSc (20%), trauma (20%), arterial disease (17%), and multifactorial/unknown (17%). In patients with ulcers attributed to SSc, age at onset was lower (45.5 vs 59.5 years). Biopsies generally did not contribute to management. Multidisciplinary treatment was routine;20% required amputation, 10% endovascular intervention, 20% frequent surgical debridement, 10% hyperbaric oxygen, 26% local treatment and antibiotics and 13% received immunosuppression for wound treatment. Conclusion: Lower extremity ulcers are a serious clinical problem in patients with SSc. The clinical exam, venous dopplers, ankle-brachial indices and assessment of vascular risk factors helped define causality. In younger patients, ulcers were more frequently attributed to SSc and these patients were more likely to be on immunosuppressants/DMARDS, possibly indicating severe phenotype of SSc.
文摘Objective; To study the characteristics and tendency of incidence of patients with respiratory system tumors during the past 23 y in Tianjin. Methods: All data in our research was obtained from the surgical pathology files of Department of Pathology of the general and the Second Hospitals of Tianjin Medical University between 1981 and 2003. All data was analyzed by Spss 11.5 statistics program. The comparisons were made by u-test, P〈0.05 was considered as significant. Results: 1. The detection rate of malignant tumors is significantly higher than that of benign tumors (U=52.68, p=0.000) in respiratory system. 2. The common sites of benign tumors are nose and pharynx, but the common sites of malignant tumors are lung and larynx. 3. The incidence of benign tumors generally peaks between the ages of 40 and 50, but the incidence of malignant tumor generally peaks between the ages of 50 and 60. 4. The commonest histological type of malignant tumors is squamous cell carcinoma, but the commonest histological type of benign tumors is papilloma. 5. The detection rate of malignant lung tumors steadily increased between 1981 and 1999 and increased sharply from 1999 to 2003, but the detection rate of malignant Nasopharyngeal tumors steadily decreased from 1981 to 2003. Between 1981 and 1997, the detection rate of malignant laryngeal tumors steadily increased, followed by a decrease between 1997 and 2003. Conclusion: The detection rate of malignant respiratory system tumors especially lung cancer is gradually increasing. Therefore early prevention and treatment are critical to patients' prognosis.
文摘Cancer continues to be a major health problem in the world. During 2018, there were 9.6 million deaths and 18.1 million new cases registered across the globe. It is estimated that one in 5 men and one in 6 women will develop cancer during their lifetime. This disease kills one in 8 men and one in 11 women. Developing countries have seen an increase in cancer occurrence and change in type of cancers due to change in social and economic conditions. The objective of this research was to further evaluate 1659 specimens for anatomical pathology testing. The data was collected from the records of the pathological anatomy laboratory at the Mohamed V Hospital in Meknes, Morocco. It is one-year study period (January-December 2013). Results of the anatomy pathology exam showed cancer positive for 9.6% of specimens;inconclusive for 1%;and negative for 66.2%. For all specimens, the most analyzed organs are breast with 29.7%;skin with 21.3%;cervical with 2.6%. For cancer patients, the most affected organs are skin (21.3%) and breast (29.7%). For cancer patients, Neoplasm is present in 9.3% of patients. Patients with metastatic cancer represent 0.3%. Patients with non-neoplastic lesions represent 66.2%. 1% of patients represent uncertain results. Additional immunohistochemistry research is needed before conclusive recommendations are made.
基金Shandong Province Medical and Health Science and Technology Development Plan Project(No.202203051068).
文摘Objective:To analyze the clinical and laboratory indices of patients with lupus nephritis(LN)of different pathological types and explore the related factors of LN pathological classification,it is helpful to grasp the timing of renal biopsy.Methods:The clinical manifestations,laboratory parameters and renal pathological types of LN patients in recent 20 years were analyzed retrospectively by SPSS 26.0 software.Results:In this study,the first three pathological types were V,IV,V+IV;latent nephritis was common in type II and V;nephritic syndrome was common in type V;nephrotic syndrome was common in type V+IV;chronic renal insufficiency group was mostly type IV;pathological types were correlated with serum creatinine,C3,albumin and erythrocyte sedimentation rate(r=0.315,P<0.001),and serum creatinine was moderately correlated(r=0.315,P<0.001);AI,CI and SLEDAI scores were significantly different among LN patients of different pathological types.Conclusion:LN is closely related to clinical pathology,clinical manifestations,comprehensive analysis of laboratory indicators and SLEDAI score to make a preliminary prediction of LN pathological type,help to initially assess the severity of pathology,improve the timing of renal biopsy implementation,optimize the timing of treatment.
文摘Scleroderma is a chronic connective tissue disease characterized by inflammation,vascular injury,and progressive skin fibrosis,resulting in significant aesthetic and functional impairments for patients.Current therapies are limited and insufficiently treat the cutaneous manifestations of scleroderma.Autologous fat transfer(AFT)is a surgical technique that has been utilized for many decades for facial rejuvenation.The adipose stem cells(ASCs)present in fat grafts have also shown significant promise for their anti-inflammatory and regenerative properties.Recently,AFT has been repurposed to treat the skin manifestations of systemic sclerosis and localized scleroderma.Studies suggest that AFT in scleroderma patients improves mouth and hand functions,Raynaud’s symptoms,and digital ulcerations.AFT is a safe procedure with rare postoperative complications,making it a promising intervention for the treatment of scleroderma.Further studies are required to better characterize the influence of fat grafts on the recipient site and to establish standards for fat transfer in fibrotic skin diseases.
文摘目的评估成人胶质母细胞瘤(GBM)脑脊髓神经轴外全身转移的临床及分子病理特征。方法在“PubMed”“Embase”和“Web of Science”数据库中检索截至2023年12月31日报道的发生全身转移的成人GBM病例,收集汇总其临床特征,同时汇总分析报道的分子病理结果以评估转移发生的可能机制。结果通过数据库检索纳入1928年-2023年的113篇文献,共147例全身转移病例。男性多于女性,男女比例为2.3∶1;中位年龄45岁;转移灶几乎可见于全身各个部位,其中常见的转移部位为骨、肺及淋巴结;自GBM初次诊断至转移发生的中位间隔约10个月;自转移发生至患者死亡的中位间隔约4.5个月;中位总体生存约14.8个月。多因素分析显示年龄≤40岁、全切除以及未合并颅内复发与患者生存显著正相关。约39例患者(39/147,26.5%)报道了基因检测的结果。不同的研究所检测的基因项目存在区别,而且不同病例之间检测的基因结果呈现明显异质性。从报道的数量及阳性比例来看,异柠檬酸脱氢酶(IDH)野生型(阳性率27/30)、TERT启动子突变(阳性率11/13)、PTEN突变(阳性率10/11)、TP53突变(阳性率10/13)和RB1突变(阳性率8/9)为相对常见的基因突变。结论成人GBM脑脊髓神经轴外全身转移罕见,年轻患者相对多见,颅内病变的复发或进展仍为决定患者生存的关键因素。转移的发生应为多克隆基因突变进展的结果,其中,促增殖、促侵袭类基因改变例如癌基因或抑癌基因突变以及上皮间质转化类相关基因可能发挥了重要作用。
文摘目的观察卵巢-附件报告和数据系统超声2022版(O-RADS US v2022)及其联合恶性风险指数4(RMI4)鉴别附件良、恶性肿瘤的价值。方法回顾性分析126例手术病理诊断为附件肿瘤患者,根据O-RADS US v2022将1~3类归为良性病变、4~5类归为恶性病变,以450为RMI4分类的临界值,基于二者进行联合分类。以病理结果为金标准,绘制受试者工作特征(ROC)曲线,计算曲线下面积(AUC),评估单一O-RADS US v2022、RMI4及其联合鉴别附件良、恶性肿瘤的效能。结果126例附件肿瘤中,良性94例、恶性32例。O-RADS US v2022鉴别附件良、恶性肿瘤的敏感度、特异度、准确率及AUC分别为78.13%、80.85%和80.16%、0.795,RMI4分别为71.88%、84.04%和80.95%、0.780;二者联合的特异度及准确率(93.62%、92.06%)均高于单一O-RADS US v2022(χ^(2)=7.322、5.967,P=0.007、0.015)或RMI4(χ^(2)=4.625、5.331,P=0.032、0.021),而敏感度及AUC(87.50%、0.906)差异均无统计学意义(P均>0.05)。结论O-RADS US v2022能有效鉴别附件良、恶性肿瘤,联合RMI4可提高鉴别特异度及准确率。