8,985 results match your criteria Diffuse Sclerosis


Mild motor impairment as prodromal state in amyotrophic lateral sclerosis: a new diagnostic entity.

Brain 2022 May 20. Epub 2022 May 20.

Department of Neurology, University of Miami, Miami, FL, USA.

Amyotrophic lateral sclerosis (ALS), when viewed as a biological entity rather than a clinical syndrome, likely evolves along a continuum, with the initial clinically silent phase eventually evolving into clinically manifest ALS. Since motor neuron degeneration is incremental and cumulative over time, it stands to reason that the clinical syndrome of ALS is likely preceded by a prodromal state characterized by minor motor abnormalities that are initially insufficient to permit a diagnosis of ALS. This prodromal period, however, is usually missed, given the invariably long delays between symptom onset and diagnostic evaluation. Read More

View Article and Full-Text PDF

Musculoskeletal ultrasound of the shoulder in systemic sclerosis.

J Scleroderma Relat Disord 2022 Jun 10;7(2):151-159. Epub 2022 Apr 10.

The Scleroderma Research Group, Khon Kaen University, Khon Kaen, Thailand.

Objectives: To explore shoulder findings by ultrasonography and to find factors associated with shoulder ultrasonographic abnormalities in systemic sclerosis patients.

Methods: A series of systemic sclerosis patients who attended the scleroderma clinic, Srinagarind Hospital, Faculty of Medicine, Khon Kaen University, Thailand, were prospectively evaluated for baseline characteristics, physical examination, and ultrasonography of both shoulders.

Results: Seventy-four systemic sclerosis patients were enrolled in this study. Read More

View Article and Full-Text PDF

Systemic sclerosis manifestations and clinical outcomes in Hispanics/Latinos of the American Southwest.

J Scleroderma Relat Disord 2022 Jun 10;7(2):135-143. Epub 2022 Apr 10.

Department of Internal Medicine, Division of Rheumatology, University of New Mexico Health Sciences Center, Albuquerque, NM, USA.

Objective: Certain Hispanic/Latino (Hispanic) populations have been reported to have higher rates and severity of systemic sclerosis; however, little is known of systemic sclerosis in the American Southwest. This study compared manifestations of systemic sclerosis in Hispanics with non-Hispanics of New Mexico.

Methods: This cross-sectional longitudinal study included 109 systemic sclerosis patients followed over a mean of 12. Read More

View Article and Full-Text PDF

Reliability, validity, and responsiveness to change of the Patient-Reported Outcomes Measurement Information System self-efficacy for managing chronic conditions measure in systemic sclerosis.

J Scleroderma Relat Disord 2022 Jun 20;7(2):110-116. Epub 2021 Oct 20.

Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.

Objective: The aim of this study is to examine validity, reliability, and responsiveness to change of Patient-Reported Outcomes Measurement Information System Self-Efficacy for Managing Chronic Conditions in persons with systemic sclerosis.

Methods: We conducted a post hoc analysis of the Patient-Reported Outcomes Measurement Information System Self-Efficacy measure and other quality-of-life measures from systemic sclerosis participants from a 16-week randomized control trial. The trial compared an Internet-based self-management program to a control condition where participants were provided an educational book. Read More

View Article and Full-Text PDF

Systemic sclerosis in Asians: Are there racial differences?

J Scleroderma Relat Disord 2022 Jun 3;7(2):98-109. Epub 2022 Feb 3.

Department of Rheumatology and Immunology, Singapore General Hospital, Singapore.

Systemic sclerosis is a multisystemic autoimmune disease characterized by vasculopathy and fibrosis. Racial factors exert a significant influence on the epidemiology, clinical manifestations, antibody profile, mortality and genetic factors in systemic sclerosis. In this review, we examined Asian systemic sclerosis cohorts reported in Asia and multi-racial cohort studies to evaluate the disease characteristics and outcomes of systemic sclerosis in Asians. Read More

View Article and Full-Text PDF

Utility of the ALSFRS-R for Predicting ALS and Comorbid Disease Neuropathology: The Veterans Affairs Biorepository Brain Bank.

Muscle Nerve 2022 May 18. Epub 2022 May 18.

VA Boston Healthcare System, Boston, MA.

Introduction/aims: The amyotrophic lateral sclerosis (ALS) functional rating scale-revised (ALSFRS-R) is commonly used to track ALS disease progression; however, there are gaps in the literature regarding the extent to which ALSFRS-R relates to underlying central nervous system (CNS) pathology. The current study explored the association between ALSFRS-R (total and subdomain) scores and postmortem neuropathology (both ALS-specific and comorbid disease).

Methods: We utilized hierarchical cluster analysis (HCA) conducted using ALSFRS-R subdomain scores to identify profiles of motor dysfunction within our sample of 93 military veterans with autopsy-confirmed ALS. Read More

View Article and Full-Text PDF

Biological and clinical insights from a randomised phase II study of an anti-oncostatin M monoclonal antibody in systemic sclerosis.

Rheumatology (Oxford) 2022 May 18. Epub 2022 May 18.

GlaxoSmithKline, Stevenage, UK.

Objectives: The cytokine oncostatin M (OSM) is implicated in the pathology of systemic sclerosis (SSc). Inhibiting OSM signalling using GSK2330811 (an anti-OSM monoclonal antibody) in patients with SSc has the potential to slow or stop the disease process.

Methods: This multicentre, randomised, double-blind, placebo-controlled study enrolled participants aged ≥18 years with active diffuse cutaneous SSc. Read More

View Article and Full-Text PDF

Orthogonal quantification of soluble inducible T-cell costimulator (ICOS) in healthy and diseased human serum.

J Pharm Anal 2022 Apr 27;12(2):317-323. Epub 2021 Jun 27.

Bioanalysis, Immunogenicity & Biomarkers, GlaxoSmithKline, Collegeville, PA, 19426, USA.

Inducible T-cell costimulator (ICOS), a homodimeric protein expressed on the surface of activated T-cells, is being investigated as a potential therapeutic target to treat various cancers. Recent studies have reported aberrant increases in the soluble form of ICOS (sICOS) in human serum in disease-state patients, primarily using commercial ELISA kits. However, results from our in-house immunoassay did not show these aberrant increases, leading us to speculate that commercial sICOS ELISAs may be prone to interference. Read More

View Article and Full-Text PDF

Nodular Lymphocyte-predominant Hodgkin Lymphoma With Nodular Sclerosis: An Underrecognized Feature Associated With Pattern D.

Am J Surg Pathol 2022 May 16. Epub 2022 May 16.

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX.

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) with unusual features, including some that can overlap morphologically with classic Hodgkin lymphoma (CHL), have been described. Herein, we describe 12 cases of NLPHL with fibrous bands and capsular fibrosis resembling, in part, nodular sclerosis (NS) CHL. Seven of 12 cases harbored Reed-Sternberg-like cells, further suggestive of CHL, but all cases lacked associated eosinophils and/or plasma cells in the background. Read More

View Article and Full-Text PDF

Erasmus Syndrome: An Underrecognized Entity.

Acta Med Port 2022 May 13. Epub 2022 May 13.

Pulmonology Department. Hospital de Santa Marta. Centro Hospitalar Universitário Lisboa Central. Lisboa. Portugal.

We present a case of a 33-year-old male who worked as a plumber and a locksmith. The patient presented with diffuse myalgia and asthenia, skin sclerosis and puffy fingers, Raynaud's phenomenon, exertional dyspnea and erectile dysfunction. The presence of specific autoantibodies enabled the diagnosis of systemic sclerosis. Read More

View Article and Full-Text PDF

Clinical, Serological, and Genetic Characteristics of a Hungarian Myositis-Scleroderma Overlap Cohort.

Biomed Res Int 2022 2;2022:6251232. Epub 2022 May 2.

Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Móricz Zs. krt. 22, 4032 Debrecen, Hungary.

Overlap myositis is a distinct subgroup of idiopathic inflammatory myositis (IIM) with various clinical phenotypes. The aim of this study was to determine the clinical, serological, and genetic features of systemic sclerosis (SSc)-IIM overlap patients. It was a retrospective study using clinical database of 39 patients, fulfilling both the criteria of SSc and IIM. Read More

View Article and Full-Text PDF

Gastric antral vascular ectasia in systemic sclerosis: a study of its epidemiology, disease characteristics and impact on survival.

Arthritis Res Ther 2022 May 10;24(1):103. Epub 2022 May 10.

Department of Medicine, The University of Melbourne at St Vincent's Hospital (Melbourne), 41 Victoria Parade, Fitzroy, Victoria, 3065, Australia.

Background: To describe the epidemiology, determinants and survival impact of gastric antral vascular ectasia (GAVE) in systemic sclerosis (SSc).

Methods: Consecutive SSc patients prospectively enrolled in the Australian Scleroderma Cohort Study (ASCS) were included. Univariable and multivariable logistic regression were used to determine the associations of GAVE with clinical manifestations and serological parameters. Read More

View Article and Full-Text PDF

Rituximab on lung, skin, and joint involvement in patients with systemic sclerosis: A case series study and review of the literature.

Int J Rheum Dis 2022 May 10. Epub 2022 May 10.

Department of Rheumatology, Dokuz Eylul University School of Medicine, Izmir, Turkey.

Objectives: To evaluate the effectiveness of rituximab (RTX) in systemic sclerosis (SSc) patients.

Methods: Data were collected from patient charts before and after RTX administration for 1 year of follow-up time. An updated review of the literature was also done. Read More

View Article and Full-Text PDF

Clinical assessment of patients with systemic sclerosis: is there a place for thermography?

Arch Dermatol Res 2022 May 9. Epub 2022 May 9.

Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, 20/24 Francuska St., 40-027, Katowice, Poland.

Recurrent changes of temperature and persistence of cooling along fingers at the room temperature make hands the most frequent region of interest for thermography in systemic sclerosis (SSc). The aim of this study was to evaluate dependance of temperature in hands on a subtype of the disease, immune profile of antinuclear antibodies (ANA), and lung involvement. There were 29 patients with limited cutaneous involvement (lcSSc) and 10 patients with diffuse cutaneous disease (dcSSc) enrolled for the study. Read More

View Article and Full-Text PDF

Opercular Syndrome: A Rare Presentation of Schilder's Variant of Multiple Sclerosis.

Neurol India 2022 Mar-Apr;70(2):806-807

Department of Neurology, Institute of Medical Science, Banaras Hindu University, Varanasi, Uttar Pradesh, India.

View Article and Full-Text PDF

Different Impact of Gadopentetate and Gadobutrol on Inflammation-Promoted Retention and Toxicity of Gadolinium Within the Mouse Brain.

Invest Radiol 2022 Apr 22. Epub 2022 Apr 22.

From the Experimental and Clinical Research Center (ECRC), A Cooperation Between the Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, and Max-Delbrück-Center for Molecular Medicine (MDC) in the Helmholtz Association, Berlin.

Objectives: Using a murine model of multiple sclerosis, we previously showed that repeated administration of gadopentetate dimeglumine led to retention of gadolinium (Gd) within cerebellar structures and that this process was enhanced with inflammation. This study aimed to compare the kinetics and retention profiles of Gd in inflamed and healthy brains after application of the macrocyclic Gd-based contrast agent (GBCA) gadobutrol or the linear GBCA gadopentetate. Moreover, potential Gd-induced neurotoxicity was investigated in living hippocampal slices ex vivo. Read More

View Article and Full-Text PDF

Capillaroscopy and Immunological Profile in Systemic Sclerosis.

Life (Basel) 2022 Mar 29;12(4). Epub 2022 Mar 29.

Department of Propaedeutics of Internal Diseases, Faculty of Medicine, Medical University of Plovdiv, 4002 Plovdiv, Bulgaria.

Introduction: Data on the associations between capillaroscopic changes and diagnostic systemic-sclerosis (SSc)-related antibodies are scarce. Presence of such correlation would improve current knowledge about the disease's pathogenesis by revealing the mechanisms of microangiopathy. The microvascular pathology of SSc is a hallmark of the disease, and immunological abnormalities probably contribute to its development. Read More

View Article and Full-Text PDF

Magnetic resonance imaging criteria at onset to differentiate pediatric multiple sclerosis from acute disseminated encephalomyelitis: A nationwide cohort study.

Mult Scler Relat Disord 2022 Mar 12;62:103738. Epub 2022 Mar 12.

Department of Radiology, Diagnostic Centre, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.

Background: MRI of the nervous system is the critical in distinguishing pediatric MS from acute disseminated encephalomyelitis (ADEM). Our aim was to propose MRI criteria to distinguish MS from monophasic ADEM based on the first MRI and to validate previously proposed MRI criteria.

Methods: A neuroradiologist undertook retrospective evaluation of the MRI at the first demyelinating event in children (<18 years) with medical record-validated MS and ADEM in Denmark during 2008-15. Read More

View Article and Full-Text PDF

Current advances in the treatment of systemic sclerosis.

Curr Opin Pharmacol 2022 Apr 18;64:102211. Epub 2022 Apr 18.

University of California, David Geffen School of Medicine, Los Angeles, USA. Electronic address:

Systemic sclerosis (SSc) is a rare, systemic autoimmune disease of unknown etiology. Among the systemic rheumatic diseases, SSc carries the highest mortality, in part due to the historical lack of disease modifying therapies. Recently, landmark randomized controlled trials (RCTs) have been conducted that have illustrated the heterogeneous nature of SSc and furthered our understanding of the key inflammatory and fibrotic pathways involved in SSc pathogenesis. Read More

View Article and Full-Text PDF

Connective tissue disease-related interstitial lung disease (CTD-ILD) and interstitial lung abnormality (ILA): Evolving concept of CT findings, pathology and management.

Eur J Radiol Open 2022 7;9:100419. Epub 2022 Apr 7.

Department of Radiology, Samsung Changwon Hospital, Sungkyunkwan University School of Medicine (SKKU-SOM), ChangWon, Gyeongsangnam-do, South Korea.

Connective tissue diseases (CTDs) demonstrating features of interstitial lung disease (ILD) include systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), systemic sclerosis (SSc), dermatomyositis (DM) and polymyositis (PM), ankylosing spondylitis (AS), Sjogren syndrome (SS), and mixed connective tissue disease (MCTD). On histopathology of lung biopsy in CTD-related ILDs (CTD-ILDs), multi-compartment involvement is an important clue, and when present, should bring CTD to the top of the list of etiologic differential diagnoses. Diverse histologic patterns including nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneumonia, apical fibrosis, diffuse alveolar damage, and lymphoid interstitial pneumonia can be seen on histology in patients with CTD-ILDs. Read More

View Article and Full-Text PDF

Systemic Sclerosis (SSc) After COVID-19: A Case Report.

Cureus 2022 Mar 15;14(3):e23179. Epub 2022 Mar 15.

Rheumatology, University of Toledo, Toledo, USA.

Since the start of the global pandemic caused by coronavirus disease 2019 (COVID-19), there have been numerous reports of autoimmune and rheumatological disorders developing after infection with SARS-CoV-2. To date, there has been only one reported case of systemic sclerosis (SSc) developing after SARS-CoV-2 infection. Here, we present another case of SSc developing after infection with SARS-CoV-2. Read More

View Article and Full-Text PDF

PRES-like leukoencephalopathy presenting with status epilepticus associated with Brentuximab Vedotin treatment.

Acta Biomed 2022 Mar 21;92(S4):e2021416. Epub 2022 Mar 21.

Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Italy.

Posterior Reversible Encephalopathy Syndrome (PRES) is characterized by acute neurological symptoms with typical imaging features, primarily in the territories of the brain supplied by the posterior circulation, probably due to vasogenic edema. Both clinical and imaging features are generally reversible. We report a 13-year-old girl affected by Nodular Sclerosis Classical Hodgkin Lymphoma stage IIIB into complete remission, with a recurrence and autologous bone-marrow transplantation, who has been treated with an anti-CD30 monoclonal antibody, brentuximab-vedotin. Read More

View Article and Full-Text PDF

Current perspective of progressive-fibrosing interstitial lung disease.

Authors:
Tomoo Kishaba

Respir Investig 2022 Apr 14. Epub 2022 Apr 14.

Department of Respiratory Medicine, Okinawa Chubu Hospital, Miyazato 281, Uruma City, Okinawa 〒904-2293, Japan. Electronic address:

Interstitial lung disease (ILD) is a parenchymal lung disease and restrictive disorder that presents as diffuse infiltrative shadows. The initial diagnosis of ILD is important because management strategies depend on the disease pathogenesis. Connective-tissue disease (CTD)-associated ILD including rheumatoid arthritis (RA), systemic sclerosis (SSc) requires a thorough evaluation of chronic respiratory symptoms such as non-productive cough and exertional dyspnea, as well as physical findings. Read More

View Article and Full-Text PDF

Hereditary Gynecologic Cancer Syndromes - A Narrative Review.

Onco Targets Ther 2022 8;15:381-405. Epub 2022 Apr 8.

Department of Gynecologic Oncology, Medical University Pleven, Pleven, Bulgaria.

Hereditary cancer syndromes are defined as syndromes, where the genetics of cancer are the result of low penetrant polymorphisms or of a single gene disorder inherited in a mendelian fashion. During the last decade, compelling evidence has accumulated that approximately 5-10% of all cancers could be attributed to hereditary cancer syndromes. A tremendous progress has been made over the last decade in the evaluation and management of these syndromes. Read More

View Article and Full-Text PDF

Non HCV-Related Mixed Cryoglobulinemic Vasculitis With Biopsy-Proven Renal Involvement: The Effects of Rituximab.

Front Med (Lausanne) 2022 28;9:819320. Epub 2022 Mar 28.

Nephrology and Dialysis Unit (The European Rare Kidney Disease Reference Network, The European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases, and the European Reference Network That Aims at Improving the Care of Patients With Rare Immunological Disorders), Center of Research of Immunopathology and Rare Diseases- Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, Department of Clinical and Biological Sciences, University of Turin and S. Giovanni Bosco Hub Hospital, Turin, Italy.

In the countries where HCV infection is still endemic, about 90% of subjects with mixed cryoglobulinemia had previously been infected with HCV and about 80% are RNA positive. Remarkable results in severe HCV-related cryoglobulinemic vasculitis have been obtained with Rituximab. Details of the clinical characteristics and effective treatment of non HCV-related cryogloulinemic syndromes are presently lacking. Read More

View Article and Full-Text PDF

Using magnetic resonance imaging to map the hidden burden of muscle involvement in systemic sclerosis.

Arthritis Res Ther 2022 Apr 11;24(1):84. Epub 2022 Apr 11.

Department of Medicine, The University of Melbourne at St Vincent's Hospital Melbourne, 41 Victoria Pde, Fitzroy, VIC, 3065, Australia.

Background: Skeletal muscle can be directly affected by systemic sclerosis (SSc); however, a significant burden of SSc-associated myopathy is undetected because clinical parameters such as weakness and creatine kinase (CK) are unreliable biomarkers of muscle involvement. This study presents qualitative and quantitative magnetic resonance imaging (MRI) findings that quantify the prevalence of myopathy and evaluate any association between skeletal and cardiac muscle involvement in SSc.

Methods: Thirty-two patients with SSc who fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism classification criteria underwent skeletal muscle MRI in addition to cardiac MRI. Read More

View Article and Full-Text PDF

Surgery for intractable epilepsy after severe encephalopathy with reversible splenial lesion and new onset hippocampal lesion associated with parechovirus.

Brain Dev 2022 Jun 4;44(6):410-414. Epub 2022 Apr 4.

Department of Neurosurgery, Epilepsy Center, Juntendo University, Tokyo, Japan. Electronic address:

We describe a case of severe encephalopathy with reversible splenial lesion associated with parechovirus, followed by intractable temporal lobe epilepsy (TLE), which was improved by epilepsy surgery. A 3-year-old girl was admitted because of fever, consciousness disturbance and generalized tonic clonic seizure. Her seizure lasted for four hours. Read More

View Article and Full-Text PDF

Scleroderma renal crisis following Covid-19 infection.

J Scleroderma Relat Disord 2021 Oct 28;6(3):320-321. Epub 2021 May 28.

Rheumatology Unit, Faculty of Medicine, Bnai Zion Medical Center, Technion, Haifa, Israel.

Systemic sclerosis (SSc) is an autoimmune disease in which environmental exposure to substances and agents may trigger disease onset or exacerbation. The most fatal complication of SSc is scleroderma renal crisis (SRC), the incidence of which is 2-3%. SRC usually occurs in the first 5 years from disease onset in diffuse-SSc patients with anti-topoisomerase 1 (ATA) or RNA polymerase 3 antibodies [1]. Read More

View Article and Full-Text PDF
October 2021

Ascending aorta aneurysm in scleroderma.

J Scleroderma Relat Disord 2021 Oct 21;6(3):322-326. Epub 2021 Jun 21.

Department of Rheumatology and clinical Immunology, Sir Ganga Ram Hospital, New Delhi, India.

Ascending aorta aneurysm in scleroderma can be ascribed to its macrovascular involvement which is very less elucidated. We here describe a 56-year-old female with rapidly progressive diffuse cutaneous scleroderma. She presented with skin thickening involving all four limbs, thorax and abdomen. Read More

View Article and Full-Text PDF
October 2021

Mycophenolate in scleroderma-associated interstitial lung disease: Real-world data from rheumatology and pulmonology clinics in South Asia.

J Scleroderma Relat Disord 2021 Oct 30;6(3):271-276. Epub 2021 Jun 30.

Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India.

Introduction: There is a paucity of real-world data on mycophenolate mofetil/mycophenolate sodium in systemic sclerosis-related interstitial lung disease.

Aim: To study the efficacy of mycophenolate mofetil/ mycophenolate sodium in systemic sclerosis-related interstitial lung disease.

Methods: In this single-centre study, clinical, laboratory and imaging details of consecutive patients with systemic sclerosis-related interstitial lung disease receiving mycophenolate mofetil/mycophenolate sodium from rheumatology and pulmonology clinics between January 2008 and March 2017 were retrospectively retrieved. Read More

View Article and Full-Text PDF
October 2021