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Article ; Online: Gerald P Rodnan.

Steen, Virginia D / Medsger, Thomas A

Rheumatic diseases clinics of North America

2023 Volume 50, Issue 1, Page(s) 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 term(s)	Humans ; United States ; Rheumatology ; Research Personnel
Language	English
Publishing date	2023-09-07
Publishing country	United States
Document type	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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Article ; 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 Volume 49, Issue 2, Page(s) 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 term(s)	Humans ; Mental Health ; Self-Management ; Disease Management ; Chronic Disease
Language	English
Publishing date	2023-03-01
Publishing country	United States
Document type	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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Article ; 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 Volume 76, Issue 3, Page(s) 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 term(s)	Humans ; Female ; United States/epidemiology ; Male ; Rheumatology ; Retrospective Studies ; Prevalence ; Lupus Erythematosus, Systemic/diagnosis ; Lupus Erythematosus, Systemic/epidemiology ; Scleroderma, Systemic/diagnosis ; Scleroderma, Systemic/epidemiology
Language	English
Publishing date	2023-12-19
Publishing country	United States
Document type	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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Zs.A 24: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG)

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Article ; 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 Volume 63, Page(s) 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 term(s)	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
Chemical Substances	Angiotensin-Converting Enzyme Inhibitors
Language	English
Publishing date	2023-08-22
Publishing country	United States
Document type	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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Article ; Online: Racial Disparities in Systemic Sclerosis.

Moore, Duncan F / Steen, Virginia D

Rheumatic diseases clinics of North America

2020 Volume 46, Issue 4, Page(s) 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 term(s)	Ethnic Groups ; Health Status Disparities ; Healthcare Disparities ; Humans ; Quality of Life ; Racism ; Scleroderma, Systemic/epidemiology ; Scleroderma, Systemic/ethnology ; Scleroderma, Systemic/therapy ; Socioeconomic Factors
Language	English
Publishing date	2020-09-08
Publishing country	United States
Document type	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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Article ; Online: Overall mortality.

Moore, Duncan F / Steen, Virginia D

Journal of scleroderma and related disorders

2020 Volume 6, Issue 1, Page(s) 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.
Language	English
Publishing date	2020-06-08
Publishing country	England
Document type	Journal Article
ISSN	2397-1991
ISSN (online)	2397-1991
DOI	10.1177/2397198320924873
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Book: Scleroderma

Steen, Virginia D.

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

1996

Author's details	Virginia D. Steen, guest ed
Series title	Rheumatic disease clinics of North America ; 22,4
Collection
Keywords	Scleroderma, Circumscribed ; Scleroderma, Systemic ; Sklerodermie
Subject	SSc ; Systemische Sclerose ; Systemische Sklerose ; Skleroderm ; Sklerodermia ; Scleroderma
Language	English
Size	IX S., S. 647 - 931 : Ill., graph. Darst.
Publisher	Saunders
Publishing place	Philadelphia u.a.
Publishing country	United States
Document type	Book
HBZ-ID	HT007369840
Database	Catalogue ZB MED Medicine, Health

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Se 363 -22,4-	verfügbar in Königswinter	Königswinter

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Article ; Online: Scleroderma renal crisis.

Hudson, Marie / Ghossein, Cybele / Steen, Virginia

Presse medicale (Paris, France : 1983)

2021 Volume 50, Issue 1, Page(s) 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 term(s)	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
Chemical Substances	Angiotensin-Converting Enzyme Inhibitors
Language	English
Publishing date	2021-02-03
Publishing country	France
Document type	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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Zs.A 794: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG)
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Article ; Online: Kidney involvement in systemic sclerosis.

Steen, Virginia D

Presse medicale (Paris, France : 1983)

2014 Volume 43, Issue 10 Pt 2, Page(s) 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 term(s)	Humans ; Kidney Diseases/diagnosis ; Kidney Diseases/etiology ; Prognosis ; Risk Factors ; Scleroderma, Systemic/complications
Language	English
Publishing date	2014-10
Publishing country	France
Document type	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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Zs.A 794: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG)
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Article ; Online: Not all that is 'full house' is systemic lupus erythematosus: a case of membranous nephropathy due to syphilis infection.

Scaperotti, Moira Marie / Kwon, DongHyang / Kallakury, Bhaskar V / Steen, Virginia

BMJ case reports

2021 Volume 14, Issue 8

Abstract: We describe an unusual case of membranous nephropathy precipitated by syphilis infection in a patient without systemic lupus erythematosus (SLE). A previously healthy 20-year-old man presented with leg and facial swelling. Laboratory investigation ... ...

Abstract	We describe an unusual case of membranous nephropathy precipitated by syphilis infection in a patient without systemic lupus erythematosus (SLE). A previously healthy 20-year-old man presented with leg and facial swelling. Laboratory investigation revealed nephrotic range proteinuria, acute kidney injury, hypocomplementaemia and a highly positive rapid plasma reagin. Kidney biopsy showed membranous nephropathy with 'full-house' immunofluorescence (IgG, IgA, IgM, C1q and C3), mimicking lupus nephritis class Vb. However, the patient had no features of SLE and had negative antinuclear and anti-double-stranded DNA antibodies. He was treated with high-dose methylprednisolone and mycophenolate mofetil for lupus nephritis and with penicillin for syphilis. After 2 months of therapy, his proteinuria resolved, and his renal function and C4 level normalised. This case illustrates that syphilis infection can be a mimicker of lupus nephritis. A literature review suggests that ful-house nephropathy may occur independently of lupus nephritis and may or may not develop into SLE.
MeSH term(s)	Adult ; Antibodies, Antinuclear ; Glomerulonephritis, Membranous/diagnosis ; Glomerulonephritis, Membranous/drug therapy ; Humans ; Lupus Erythematosus, Systemic/complications ; Lupus Erythematosus, Systemic/diagnosis ; Lupus Erythematosus, Systemic/drug therapy ; Lupus Nephritis/complications ; Lupus Nephritis/diagnosis ; Lupus Nephritis/drug therapy ; Male ; Syphilis ; Young Adult
Chemical Substances	Antibodies, Antinuclear
Language	English
Publishing date	2021-08-19
Publishing country	England
Document type	Case Reports ; Journal Article
ISSN	1757-790X
ISSN (online)	1757-790X
DOI	10.1136/bcr-2021-244466
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