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  1. Artikel ; Online: Gerald P Rodnan.

    Steen, Virginia D / Medsger, Thomas A

    Rheumatic diseases clinics of North America

    2023  Band 50, Heft 1, Seite(n) 25–32

    Abstract: Dr Gerald Rodnan was a man of many talents who developed a single-minded fascination with the disease systemic sclerosis. His passion and vision led to numerous important research contributions to our understanding of the natural history of this disease ... ...

    Abstract Dr Gerald Rodnan was a man of many talents who developed a single-minded fascination with the disease systemic sclerosis. His passion and vision led to numerous important research contributions to our understanding of the natural history of this disease and his extensive travel and teaching stimulated many other investigators in the United States and throughout the world to devote their careers to this uncommon but serious disorder. He indeed was a "Giant" in rheumatology and those of us who had the opportunity to train under him have been inspired to carry it forward.
    Mesh-Begriff(e) Humans ; United States ; Rheumatology ; Research Personnel
    Sprache Englisch
    Erscheinungsdatum 2023-09-07
    Erscheinungsland United States
    Dokumenttyp Journal Article ; Review
    ZDB-ID 92118-x
    ISSN 1558-3163 ; 0889-857X
    ISSN (online) 1558-3163
    ISSN 0889-857X
    DOI 10.1016/j.rdc.2023.08.002
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  2. Artikel ; Online: Mental Health Considerations in Chronic Disease: What Is the Best Approach for Supporting a Scleroderma Patient?

    Lazar, Nancy / Steen, Virginia D

    Rheumatic diseases clinics of North America

    2023  Band 49, Heft 2, Seite(n) 389–399

    Abstract: Each person who presents for scleroderma-focused care not only has their own psychosocial stressors in their day-to-day life but they also have scleroderma symptom-specific stressors as well as their own mental health reactions throughout their journey ... ...

    Abstract Each person who presents for scleroderma-focused care not only has their own psychosocial stressors in their day-to-day life but they also have scleroderma symptom-specific stressors as well as their own mental health reactions throughout their journey with this disease course. There are many actions patients can take to help and support themselves when they are faced with any of the mental health and social determinants of health stressors associated with this rare, chronic illness. Using the scleroderma specialty providers to inform, discuss, and address these areas with their patients can assist with more effective symptom and disease self-management.
    Mesh-Begriff(e) Humans ; Mental Health ; Self-Management ; Disease Management ; Chronic Disease
    Sprache Englisch
    Erscheinungsdatum 2023-03-01
    Erscheinungsland United States
    Dokumenttyp Journal Article ; Review
    ZDB-ID 92118-x
    ISSN 1558-3163 ; 0889-857X
    ISSN (online) 1558-3163
    ISSN 0889-857X
    DOI 10.1016/j.rdc.2023.01.010
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  3. Artikel: Spectrum of renal disease in scleroderma other than scleroderma renal crisis: A review of the literature.

    Farrukh, Larabe / Steen, Virginia / Shapiro, Lee / Faddoul, Geovani / Mehta, Swati

    Clinical nephrology

    2024  

    Abstract: Background: Systemic sclerosis (SSc) is a multi-system rheumatic disease characterized by vascular and fibrotic manifestations that can affect practically every organ. Scleroderma renal crisis (SRC) is the most common renal manifestation of SSc. However, ...

    Abstract Background: Systemic sclerosis (SSc) is a multi-system rheumatic disease characterized by vascular and fibrotic manifestations that can affect practically every organ. Scleroderma renal crisis (SRC) is the most common renal manifestation of SSc. However, with the use of angiotensin-converting enzyme inhibitors (ACEi), the morbidity and mortality associated with SRC has significantly reduced. Renal manifestations in SSc other than SRC have been generally under-recognized and can be left untreated, which can lead to grave consequences in this patient population. In this article, we will describe the spectrum of renal disease in SSc besides SRC.
    Materials and methods: A literature search was conducted on PubMed and Cochrane from inception to December 2022 using medical subject headings (MeSH) terms for "scleroderma", "systemic sclerosis" combined with "renal injury", and "renal dysfunction". We included case reports, case series, observational studies, and literature reviews.
    Results: The initial search revealed 393 articles. After the exclusion of duplicates and non-relevant articles, data was included from 30 articles and 45 patients. The mean age was 55.2 years, 9 males (20%) and 36 females (80%). The most reported renal manifestations included: ANCA-associated vasculitis (n = 22), penicillamine-induced renal injury (n = 8), oxalate nephropathy (n = 5), Goodpasture syndrome (n = 4), nephrotic range proteinuria (n = 2), renal artery stenosis (n = 2), membranous glomerulonephritis (n = 1), and Evans syndrome (n = 1).
    Conclusion: The spectrum of kidney involvement in SSc can range from asymptomatic reduction of the glomerular filtration rate to life-threatening scleroderma renal crisis. Therefore, it is essential that physicians closely monitor renal function in these patients for any emerging renal dysfunction.
    Sprache Englisch
    Erscheinungsdatum 2024-05-03
    Erscheinungsland Germany
    Dokumenttyp Journal Article
    ZDB-ID 185101-9
    ISSN 0301-0430
    ISSN 0301-0430
    DOI 10.5414/CN111243
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  4. Artikel ; Online: Prevalence and Characteristics of Patients With Systemic Sclerosis Fulfilling the 2019 EULAR/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus.

    Bass, Ronald D / Moore, Duncan F / Steen, Virginia D

    Arthritis care & research

    2023  Band 76, Heft 3, Seite(n) 311–317

    Abstract: Objective: There is limited literature describing the overlap of systemic sclerosis (SSc) and systemic lupus erythematosus (SLE), and the studies have employed a range of case definitions. Our study used the new EULAR/American College of Rheumatology ( ... ...

    Abstract Objective: There is limited literature describing the overlap of systemic sclerosis (SSc) and systemic lupus erythematosus (SLE), and the studies have employed a range of case definitions. Our study used the new EULAR/American College of Rheumatology (ACR) SLE classification criteria to define SSc-SLE cases among our center's SSc cohort.
    Methods: This is a single-center, retrospective study of a previously described cohort of patients with SSc. Patient data were re-abstracted to evaluate for fulfillment of the 2019 EULAR/ACR classification criteria for SLE. Demographic, laboratory, clinical features, and mortality were compared among patients with SSc-SLE and patients with SSc alone.
    Results: Among the 402 patients with SSc that were analyzed, 40 (10%) fulfilled the 2019 EULAR/ACR SLE classification criteria. Neuropsychiatric and renal involvement were rare. An initial SLE diagnosis was purported in 43% of the patients with SSc-SLE and 7% of patients with SSc alone (P < 0.001). Patients with SSc-SLE were more likely to be female, African American, and with limited cutaneous SSc. Anti-U1-RNP antibody positivity prevalence was 30% among patients with SSc-SLE and 6.6% among patients with SSc alone (P < 0.001). Death during follow-up occurred in 12 patients (30%) with SSc-SLE and in 81 patients (22%) with SSc alone, but there was no difference in survival among the groups per log rank test (P = 0.404).
    Conclusion: Ten percent of patients with SSc fulfill the 2019 EULAR/ACR classification criteria for SLE. These patients comprise a distinct demographic, serologic, and clinical phenotype but have similar severe SSc-specific end-organ damage and mortality as patients with SSc alone. Patients with SLE with Raynaud phenomenon should be evaluated for SSc-specific autoantibodies and scleroderma organ involvement.
    Mesh-Begriff(e) Humans ; Female ; United States/epidemiology ; Male ; Rheumatology ; Retrospective Studies ; Prevalence ; Lupus Erythematosus, Systemic/diagnosis ; Lupus Erythematosus, Systemic/epidemiology ; Scleroderma, Systemic/diagnosis ; Scleroderma, Systemic/epidemiology
    Sprache Englisch
    Erscheinungsdatum 2023-12-19
    Erscheinungsland United States
    Dokumenttyp Journal Article
    ZDB-ID 645059-3
    ISSN 2151-4658 ; 0893-7524 ; 2151-464X
    ISSN (online) 2151-4658
    ISSN 0893-7524 ; 2151-464X
    DOI 10.1002/acr.25235
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  5. Buch: Scleroderma

    Steen, Virginia D.

    (Rheumatic disease clinics of North America ; 22,4)

    1996  

    Verfasserangabe Virginia D. Steen, guest ed
    Serientitel Rheumatic disease clinics of North America ; 22,4
    Überordnung
    Schlagwörter Scleroderma, Circumscribed ; Scleroderma, Systemic ; Sklerodermie
    Schlagwörter SSc ; Systemische Sclerose ; Systemische Sklerose ; Skleroderm ; Sklerodermia ; Scleroderma
    Sprache Englisch
    Umfang IX S., S. 647 - 931 : Ill., graph. Darst.
    Verlag Saunders
    Erscheinungsort Philadelphia u.a.
    Erscheinungsland Vereinigte Staaten
    Dokumenttyp Buch
    HBZ-ID HT007369840
    Datenquelle Katalog ZB MED Medizin, Gesundheit

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  6. Artikel ; Online: Studying the Role of C5-Inhibition Therapy in Scleroderma Renal Crisis-Induced Thrombotic Microangiopathy - A Review of Literature.

    Farrukh, Larabe / Steen, Virginia D / Shapiro, Lee / Mehta, Swati

    Seminars in arthritis and rheumatism

    2023  Band 63, Seite(n) 152256

    Abstract: Background: The pathogenesis of scleroderma renal crisis (SRC) remains poorly understood but a growing body of evidence suggests that activation of the complement system may be involved in the disease. Recent studies have shown that Eculizumab ( ... ...

    Abstract Background: The pathogenesis of scleroderma renal crisis (SRC) remains poorly understood but a growing body of evidence suggests that activation of the complement system may be involved in the disease. Recent studies have shown that Eculizumab (monoclonal antibody directed against the complement component C5) is effective in treating patients with SRC who present with symptoms of thrombotic microangiopathy (SRC-TMA).
    Objectives: In this study, we conducted a systematic review to characterize the published experience of the presentation and outcome of patients with SRC who were treated with C5 inhibitor, Eculizumab.
    Methods: A literature search was conducted from inception to December 2022 using Medical Subject Headings (MeSH) terms for 'scleroderma', 'scleroderma renal crisis, and 'Eculizumab'. We included case reports, case series, and observational studies which reported the use of Eculizumab with or without Angiotensin-converting enzyme inhibitors (ACE-I) for the treatment of scleroderma renal crisis (SRC) in patients with systemic sclerosis.
    Results: The study included 17 patients, all of whom were treated with Eculizumab. Additionally, the use of ACE-I was reported in 11/17 (64.7%) patients. Further, plasmapheresis was used in 9/17 (52.9%), steroids in 5/17 (29.4%), cyclophosphamide in 3/17 (17.6%), calcium channel blockers in 3/17 (17.6%), and Rituximab in 3/17 (17.6%) patients. Renal replacement therapy was required in 11/17 (64.7%) patients. 14/17 patients (82.3%) were reported to have clinical (renal or hematologic) improvement with Eculizumab therapy (Table 1).
    Conclusion: These findings should prompt testing on a larger cohort of SRC-TMA patients. This would help us determine whether aggressive treatment combining ACE-I and Eculizumab can target the various underlying endothelial, inflammatory, and immunologic mechanisms involved in SRC-TMA, and improve patient outcomes.
    Mesh-Begriff(e) Humans ; Angiotensin-Converting Enzyme Inhibitors/therapeutic use ; Acute Kidney Injury ; Kidney/pathology ; Thrombotic Microangiopathies/drug therapy ; Thrombotic Microangiopathies/etiology ; Scleroderma, Systemic/complications ; Scleroderma, Systemic/drug therapy ; Scleroderma, Systemic/pathology
    Chemische Substanzen Angiotensin-Converting Enzyme Inhibitors
    Sprache Englisch
    Erscheinungsdatum 2023-08-22
    Erscheinungsland United States
    Dokumenttyp Systematic Review ; Journal Article ; Review
    ZDB-ID 120247-9
    ISSN 1532-866X ; 0049-0172
    ISSN (online) 1532-866X
    ISSN 0049-0172
    DOI 10.1016/j.semarthrit.2023.152256
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  7. Artikel ; Online: Overall mortality.

    Moore, Duncan F / Steen, Virginia D

    Journal of scleroderma and related disorders

    2020  Band 6, Heft 1, Seite(n) 3–10

    Abstract: Systemic sclerosis is a highly morbid, complex autoimmune disease that is variable both in its phenotype and the attendant mortality driven by such manifestations. This review article synthesizes mortality data from the best available meta-analyses, ... ...

    Abstract Systemic sclerosis is a highly morbid, complex autoimmune disease that is variable both in its phenotype and the attendant mortality driven by such manifestations. This review article synthesizes mortality data from the best available meta-analyses, subgroup analyses of single cohorts, and subjective comparisons of individual cohort studies, which in aggregate suggest that mortality in systemic sclerosis has been gradually improving over the past several decades. This review also summarizes the literature describing various risk factors for mortality in systemic sclerosis.
    Sprache Englisch
    Erscheinungsdatum 2020-06-08
    Erscheinungsland England
    Dokumenttyp Journal Article
    ISSN 2397-1991
    ISSN (online) 2397-1991
    DOI 10.1177/2397198320924873
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  8. Artikel ; Online: Racial Disparities in Systemic Sclerosis.

    Moore, Duncan F / Steen, Virginia D

    Rheumatic diseases clinics of North America

    2020  Band 46, Heft 4, Seite(n) 705–712

    Abstract: Racial and ethnic disparities in systemic sclerosis are abundant. The incidence, severity of end-organ manifestations, functional impairment, quality of life, and mortality of systemic sclerosis vary by ethnic group. This article summarizes such ... ...

    Abstract Racial and ethnic disparities in systemic sclerosis are abundant. The incidence, severity of end-organ manifestations, functional impairment, quality of life, and mortality of systemic sclerosis vary by ethnic group. This article summarizes such disparities and explores the role of socioeconomic status in their development and persistence.
    Mesh-Begriff(e) Ethnic Groups ; Health Status Disparities ; Healthcare Disparities ; Humans ; Quality of Life ; Racism ; Scleroderma, Systemic/epidemiology ; Scleroderma, Systemic/ethnology ; Scleroderma, Systemic/therapy ; Socioeconomic Factors
    Sprache Englisch
    Erscheinungsdatum 2020-09-08
    Erscheinungsland United States
    Dokumenttyp Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 92118-x
    ISSN 1558-3163 ; 0889-857X
    ISSN (online) 1558-3163
    ISSN 0889-857X
    DOI 10.1016/j.rdc.2020.07.009
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  9. Artikel ; Online: Scleroderma renal crisis.

    Hudson, Marie / Ghossein, Cybele / Steen, Virginia

    Presse medicale (Paris, France : 1983)

    2021  Band 50, Heft 1, Seite(n) 104063

    Abstract: Scleroderma renal crisis (SRC) is a rare but life-threatening complication of systemic sclerosis (SSc) characterized by malignant hypertension and acute kidney injury. Historically, SRC was the leading cause of death in SSc. However, with the advent of ... ...

    Abstract Scleroderma renal crisis (SRC) is a rare but life-threatening complication of systemic sclerosis (SSc) characterized by malignant hypertension and acute kidney injury. Historically, SRC was the leading cause of death in SSc. However, with the advent of angiotensin converting enzyme (ACE) inhibitors, mortality rates have decreased significantly. Nevertheless, one-year outcomes remain poor, with over 30% mortality and 25% of patients remaining dialysis-dependent. There is an urgent need to improve early recognition and treatment, and to identify novel treatments to improve outcomes of SRC. In this chapter, the clinical features, classification, pathophysiology, differential diagnosis, management and outcomes of SRC are presented. Specific issues relating to pregnancy, prophylactic ACE inhibition and management of essential hypertension are also discussed.
    Mesh-Begriff(e) Acute Kidney Injury/etiology ; Acute Kidney Injury/mortality ; Acute Kidney Injury/pathology ; Acute Kidney Injury/therapy ; Angiotensin-Converting Enzyme Inhibitors/therapeutic use ; Diagnosis, Differential ; Female ; Humans ; Hypertension, Malignant/etiology ; Hypertension, Malignant/mortality ; Hypertension, Malignant/pathology ; Hypertension, Malignant/therapy ; Pregnancy ; Pregnancy Complications/etiology ; Renal Dialysis ; Scleroderma, Systemic/complications
    Chemische Substanzen Angiotensin-Converting Enzyme Inhibitors
    Sprache Englisch
    Erscheinungsdatum 2021-02-03
    Erscheinungsland France
    Dokumenttyp Journal Article ; Review
    ZDB-ID 120943-7
    ISSN 2213-0276 ; 0032-7867 ; 0755-4982 ; 0301-1518
    ISSN (online) 2213-0276
    ISSN 0032-7867 ; 0755-4982 ; 0301-1518
    DOI 10.1016/j.lpm.2021.104063
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  10. Artikel ; Online: Kidney involvement in systemic sclerosis.

    Steen, Virginia D

    Presse medicale (Paris, France : 1983)

    2014  Band 43, Heft 10 Pt 2, Seite(n) e305–14

    Abstract: Kidney involvement in systemic sclerosis (SSc) is primarily manifested by scleroderma renal crisis (SRC). Formerly, it was the most severe complication in scleroderma and was the most frequent cause of death in these patients. More than 30years ago, with ...

    Abstract Kidney involvement in systemic sclerosis (SSc) is primarily manifested by scleroderma renal crisis (SRC). Formerly, it was the most severe complication in scleroderma and was the most frequent cause of death in these patients. More than 30years ago, with the development of angiotensin converting enzyme (ACE) inhibitors, SRC became a very treatable complication of scleroderma. Although there are still many patients who do not survive and have poor outcomes, early diagnosis of renal crisis and prompt therapeutic intervention can achieve excellent outcomes. Renal abnormalities independent of renal crisis have been noted, but can usually be attributed to other problems. Further understanding of the pathogenesis of renal disease in scleroderma may lead to additional improvement in the therapy of renal crisis and perhaps the disease in general. This chapter reviews the pathogenesis, clinical setting, and therapy of this serious complication of SSc.
    Mesh-Begriff(e) Humans ; Kidney Diseases/diagnosis ; Kidney Diseases/etiology ; Prognosis ; Risk Factors ; Scleroderma, Systemic/complications
    Sprache Englisch
    Erscheinungsdatum 2014-10
    Erscheinungsland France
    Dokumenttyp Journal Article ; Review
    ZDB-ID 120943-7
    ISSN 2213-0276 ; 0032-7867 ; 0755-4982 ; 0301-1518
    ISSN (online) 2213-0276
    ISSN 0032-7867 ; 0755-4982 ; 0301-1518
    DOI 10.1016/j.lpm.2014.02.031
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