scholarly journals Systemic sclerosis sine scleroderma: a case report of anterior uveitis

Reumatismo ◽  
2015 ◽  
Vol 67 (1) ◽  
Author(s):  
T. Borges ◽  
J. Vilaça ◽  
S. Ferreira ◽  
I. Chora ◽  
S. Silva ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Anterior Uveitis ◽  
Heart Diseases ◽  
Systolic Dysfunction ◽  
Skin Involvement ◽  
American College Of Rheumatology ◽  
European League Against Rheumatism ◽  
History Of ◽  
Non Ischemic Dilated Cardiomyopathy

Systemic sclerosis (SSc) sine scleroderma (ssSSc) is characterized by the absence of skin involvement, despite other manifestations of systemic sclerosis are present. It is not known whether sSSc represents a <em>forme</em> <em>fruste</em> of limited cutaneous SSc or a distinct entity, but the 2013 American College of Rheumatology/European League Against Rheumatism criteria for the classification of SSc have considered SSc without skin involvement to be a distinct subset. The authors present the case of a 70-year old female that was referred for a consultation for Raynaud’s phenomenon and a chronic anterior uveitis (CAU). She had a history of dysphagia, diffuse pulmonary emphysema and a biopsy-documented fibrosis of the upper lobes, and an idiopathic non-ischemic dilated cardiomyopathy with severe left ventricle systolic dysfunction and left bundle branch block. Anti-nuclear and anti-centromere antibodies were positive, while manometry revealed distal esophageal hypomotility. After establishing the diagnosis of ssSSc and starting immunosuppression, the ocular disease improved, while the lung and heart diseases remained stable. This case underlines that it is very important to suspect SSc when CAU is present and/or skin thickening is absent. To our knowledge, this is the first report of CAU in a patient with ssSSc.

scholarly journals Tophaceous gout of the atlantoaxial joint: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Andrew Benjamin Romero ◽  
Evan Paul Johnson ◽  
John S. Kirkpatrick
Keyword(s):  
Cervical Spine ◽  
Tissue Sample ◽  
Significant Finding ◽  
Atlantoaxial Joint ◽  
Tophaceous Gout ◽  
Case Presentation ◽  
American College Of Rheumatology ◽  
European League Against Rheumatism ◽  
History Of ◽  
Clinically Significant

Abstract Background To report the occurrence of tophaceous gout in the cervical spine and to review the literature on spinal gout. Case presentation This report details the occurrence of a large and clinically significant finding of tophaceous gout in the atlantoaxial joint of the cervical spine in an 82-year-old Caucasian man with a 40-year history of crystal-proven gout and a 3-month history of new-onset progressive myelopathy. The patient's American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria score was 15.0. Conclusion Spinal gout is more common than previously thought, and it should be considered in patients who present with symptoms of myelopathy. Diagnosis can be made without a tissue sample of the affected joint(s) with tools like the ACR/EULAR criteria and the use of the “diagnostic clinical rule” for determining the likelihood of gout. Early conservative management with neck immobilization and medical management can avoid the need for surgical intervention.

Rheumatoid Arthritis: Etiology and Pathogenesis

2014 ◽  
Author(s):  
Gary S. Firestein ◽  
Anna-Karin H. Ekwall
Keyword(s):  
Rheumatoid Arthritis ◽  
Intracellular Signaling ◽  
Cartilage Damage ◽  
Signs And Symptoms ◽  
American College Of Rheumatology ◽  
European League Against Rheumatism ◽  
Citrullinated Proteins ◽  
Definition Of

Rheumatoid arthritis (RA) is among the most common forms of chronic inflammatory arthritis. It affects approximately 1% of adults and is two to three times more prevalent in women than in men. There are no specific laboratory tests for RA; diagnosis depends on a constellation of signs and symptoms that can be supported by serology and radiographs. The disease evolves over many years as a consequence of repeated environmental stress causing inflammation and immune activation followed by a breakdown of tolerance in individuals with a specific genetic background. This review describes the definition of RA; its etiology, including genetics, infections, the role of smoking and citrullination of proteins, and epigenetic mechanisms; and its pathogenesis, including synovial histopathology, bone and cartilage damage, adaptive and innate immunity, and the role of cytokines and intracellular signaling. Tables include the 1987 American Rheumatism Association criteria for the classification of RA and the 2010 American College of Rheumatology/European League Against Rheumatism classification for RA. Figures show citrullinated proteins in airway cells, a section of a proliferative synovium from a patient with a classic RA, and scalloped regions of erosion at the junction between a proliferative inflamed rheumatoid synovium and the bone. This review contains 3 highly rendered figures, 2 tables, and 71 references.

Prevalence of Systemic Sclerosis in Primary Biliary Cholangitis Using the New ACR/EULAR Classification Criteria

2016 ◽  
Vol 44 (1) ◽  
pp. 33-39 ◽  
Author(s):  
Boyang Zheng ◽  
Catherine Vincent ◽  
Marvin J. Fritzler ◽  
Jean-Luc Senécal ◽  
Martial Koenig ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Tertiary Care ◽  
Classification Criteria ◽  
Primary Biliary Cholangitis ◽  
American College Of Rheumatology ◽  
Acr Criteria ◽  
European League Against Rheumatism ◽  
High Prevalence ◽  
Established Disease ◽  
Secondary Objective

Objective.Systemic sclerosis (SSc) is a well-established disease associated with primary biliary cholangitis (PBC). However, the original 1980 American College of Rheumatology (ACR) criteria have poor sensitivity, especially for the detection of earlier SSc in previous studies. The objective was to evaluate the prevalence of SSc in patients with PBC using more sensitive 2001 LeRoy and Medsger criteria and the 2013 ACR/European League Against Rheumatism (EULAR) classification criteria. The secondary objective was to evaluate the frequency of individual clinical features.Methods.One hundred consecutive patients with PBC without previously diagnosed SSc were recruited between 2005 and 2007 from a tertiary care gastroenterology clinic. All patients underwent a complete clinical examination, determination of SSc-specific antibodies, and a nailfold capillary microscopy. Fulfillment of the 3 different criteria sets was analyzed, along with individual disease features.Results.Of 100 patients with PBC, 1% met the ACR 1980 criteria, 22% met the 2001 LeRoy and Medsger criteria for early SSc, and 17% the 2013 ACR/EULAR criteria. Raynaud phenomenon, SSc-related antibodies, and SSc capillaroscopic patterns were the most prevalent findings, with the highest sensitivities to help guide future screening.Conclusion.Our data show a high prevalence of SSc in patients with PBC with probable underestimation by previous studies using the original ACR criteria. Comorbid SSc should be actively searched for based on newly described criteria to improve detection and increase benefits of earlier treatment.

scholarly journals Diagnostic Classification of Patients with Dilated Cardiomyopathy Using Ventricular Strain Analysis Algorithm

2021 ◽  
Vol 2021 ◽  
pp. 1-9
Author(s):  
Mingliang Li ◽  
Yidong Chen ◽  
Yujie Mao ◽  
Mingfeng Jiang ◽  
Yujun Liu ◽  
...  
Keyword(s):  
Left Ventricle ◽  
Dilated Cardiomyopathy ◽  
Heart Diseases ◽  
Systolic Dysfunction ◽  
Strain Analysis ◽  
Parameter Extraction ◽  
Mr Image ◽  
Normal People ◽  
Complex Methods

Dilated cardiomyopathy (DCM) is a cardiomyopathy with left ventricle or double ventricle enlargement and systolic dysfunction. It is an important cause of sudden cardiac death and heart failure and is the leading indication for cardiac transplantation. Major heart diseases like heart muscle damage and valvular problems are diagnosed using cardiac MRI. However, it takes time for cardiologists to measure DCM-related parameters to decide whether patients have this disease. We have presented a method for automatic ventricular segmentation, parameter extraction, and diagnosing DCM. In this paper, left ventricle and right ventricle are segmented by parasternal short-axis cardiac MR image sequence; then, related parameters are extracted in the end-diastole and end-systole of the heart. Machine learning classifiers use extracted parameters as input to predict normal people and patients with DCM, among which Random forest classifier gives the highest accuracy. The results show that the proposed system can be effectively utilized to detect and diagnose DCM automatically. The experimental results suggest the capabilities and advantages of the proposed method to diagnose DCM. A small amount of sample input can generate results comparable to more complex methods.

Incidence and prevalence of systemic sclerosis in Valcamonica, Italy, during an 18-year period

2019 ◽  
Vol 5 (1) ◽  
pp. 51-56 ◽  
Author(s):  
Paolo Airò ◽  
Francesca Regola ◽  
Maria-Grazia Lazzaroni ◽  
Angela Tincani ◽  
Flora Inverardi ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
International Classification Of Diseases ◽  
Alpine Valley ◽  
Tertiary Referral Center ◽  
American College Of Rheumatology ◽  
Clinical Databases ◽  
European League Against Rheumatism ◽  
Classification Of Diseases ◽  
Rheumatology Unit ◽  
Capture Recapture

Objectives: To investigate the epidemiology of systemic sclerosis in Valcamonica, an Italian Alpine valley, during an 18-year-long period. Methods: Patients with systemic sclerosis living in Valcamonica between 1999 and 2016 were identified by capture/recapture method using: (1) clinical databases of the only secondary Rheumatology Unit present in the valley and of the tertiary referral center for this area; (2) administrative data, extracting records with the International Classification of Diseases, 10th Revision, code for systemic sclerosis. Patients were included in the analysis when either the 1980 American Rheumatism Association classification criteria for systemic sclerosis or the 2013 American College of Rheumatology/European League Against Rheumatism criteria were satisfied. To study temporal changes, mean yearly incidence during three different 6-year interval was calculated. Prevalence rates were estimated at four different time points. Results: General population with age over 14 years living in Valcamonica varied during the evaluated period between 85,168 and 91,245 inhabitants. A total of 65 patients with systemic sclerosis were identified (female 84.6%, limited cutaneous systemic sclerosis: 84.6%; anticentromere: 64.6%). Systemic sclerosis incidence and prevalence increased during the study period (p = 0.029 and p < 0.0001, respectively). The increase of incidence was accounted for by cases satisfying only the 2013 criteria, with limited cutaneous systemic sclerosis, and with anticentromere, whereas the incidence of systemic sclerosis cases classified according to the 1980 criteria did not significantly increase. The prevalence at 31 December 2016 was 58.6 (95% confidence interval, 44.8–76.6) per 100,000 persons aged >14 years. Survival at 10 years after systemic sclerosis diagnosis was 83.0% (standard error, 5.6). Conclusion: Systemic sclerosis incidence and prevalence increased over time in this area, due to the increased recruitment of patients with milder forms of the disease.

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