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  1. No Access

    Chapter

    Managing the Ischemic Finger in Scleroderma

    The patient is a 48-year-old Caucasian woman with limited scleroderma. The features of her disease include stiff puffy fingers, gastrointestinal reflux disease (GERD), severe Raynaud’s phenomenon (RP), and sic...

    Fredrick M. Wigley, Thomas Reifsnyder in Scleroderma (2024)

  2. No Access

    Book

    Raynaud’s Phenomenon

    From Pathogenesis to Management

    Fredrick M. Wigley, Ariane L. Herrick (2024)

  3. No Access

    Chapter

    Drug Therapy

    Raynaud’s phenomenon (RP) may warrant pharmacological treatment if there are significant symptoms not controlled by nonpharmacological treatment or complications. The principles of RP treatment are to decrease...

    Ariane L. Herrick, Fredrick M. Wigley, Janet Pope in Raynaud’s Phenomenon (2024)

  4. No Access

    Chapter

    Practical Approaches to Treatment: Case Studies

    This Chapter presents several clinical situations to illustrate the approach to the management of Raynaud’s phenomenon and its complications. The management of several cases of scleroderma with recurrent epis...

    Fredrick M. Wigley in Raynaud’s Phenomenon (2024)

  5. No Access

    Chapter

    Overlooked Manifestations

    Systemic sclerosis (SSc) is a heterogeneous disease with well-described traditional clinical manifestations, such as Raynaud’s phenomenon, dermal sclerosis, esophageal dysmotility, calcinosis, and telangiectas...

    Ami A. Shah, Fredrick M. Wigley in Scleroderma (2024)

  6. No Access

    Article

    Assessment of right ventricular reserve utilizing exercise provocation in systemic sclerosis

    Right ventricular (RV) capacity to adapt to increased afterload is the main determinant of outcome in pulmonary hypertension, a common morbidity seen in systemic sclerosis (SSc). We hypothesized that supine bi...

    Monica Mukherjee, Valentina Mercurio in The International Journal of Cardiovascula… (2021)

  7. Article

    Open Access

    Linaclotide for the treatment of refractory lower bowel manifestations of systemic sclerosis

    Lower gastrointestinal (GI) tract involvement can affect up to 50% of systemic sclerosis (SSc) patients, and may result in malabsorption, pseudo-obstruction, hospitalization, and death. We report our experienc...

    Eric J. Dein, Fredrick M. Wigley, Zsuzsanna H. McMahan in BMC Gastroenterology (2021)

  8. No Access

    Article

    Vascular biomarkers and digital ulcerations in systemic sclerosis: results from a randomized controlled trial of oral treprostinil (DISTOL-1)

    Although there is abundant evidence of vascular perturbation from studies of peripheral blood in systemic sclerosis (SSc), there are few data about the ability to use biomarkers of vascular injury and growth f...

    Christopher A. Mecoli, Jamie Perin, Jennifer E. Van Eyk, Jie Zhu in Clinical Rheumatology (2020)

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    Article

    Vascular complications in systemic sclerosis: a prospective cohort study

    Two major complications in scleroderma patients that cause substantial morbidity and mortality are ischemic digital lesions (DL) and pulmonary hypertension (PH). The clinician’s ability to predict which patien...

    Christopher A. Mecoli, Ami A. Shah, Francesco Boin in Clinical Rheumatology (2018)

  10. No Access

    Book

    Scleroderma

    From Pathogenesis to Comprehensive Management

    John Varga, Christopher P. Denton, Fredrick M. Wigley (2017)

  11. Article

    Open Access

    Frequency of circulating topoisomerase-I-specific CD4 T cells predicts presence and progression of interstitial lung disease in scleroderma

    Scleroderma is an antigen-driven T cell-mediated autoimmune disease. Presence of anti-topoisomerase-I antibodies is associated with pulmonary fibrosis and predicts increased mortality. Characterization of auto...

    Andrea Fava, Raffaello Cimbro, Fredrick M. Wigley in Arthritis Research & Therapy (2016)

  12. Article

    Open Access

    Genetic susceptibility loci of idiopathic interstitial pneumonia do not represent risk for systemic sclerosis: a case control study in Caucasian patients

    Systemic sclerosis (SSc)-related interstitial lung disease (ILD) has phenotypic similarities to lung involvement in idiopathic interstitial pneumonia (IIP). We aimed to assess whether genetic susceptibility lo...

    Minghua Wu, Shervin Assassi, Gloria A. Salazar in Arthritis Research & Therapy (2016)

  13. Article

    Open Access

    Biomarkers of pulmonary hypertension in patients with scleroderma: a case–control study

    Significant pulmonary vascular disease is a leading cause of death in patients with scleroderma, and early detection and early medical intervention are important, as they may delay disease progression and impr...

    Zsuzsanna McMahan, Florian Schoenhoff, Jennifer E. Van Eyk in Arthritis Research & Therapy (2015)

  14. No Access

    Book

    Raynaud’s Phenomenon

    A Guide to Pathogenesis and Treatment

    Fredrick M. Wigley, Ariane L. Herrick (2015)

  15. No Access

    Chapter

    Ischemic Digital Ulcers

    Systemic sclerosis (scleroderma) is unique among the rheumatic diseases because a widespread obliterative vasculopathy exists involving the peripheral arteries and microcirculation. Various forms of digital ul...

    Fredrick M. Wigley in A Visual Guide to Scleroderma and Approach to Treatment (2014)

  16. No Access

    Article

    Integrin-modulating therapy prevents fibrosis and autoimmunity in mouse models of scleroderma

    Failure of integrin-mediated cell-matrix attachment is sufficient to initiate dermal fibrosis and autoimmunity in mouse models of scleroderma; integrin-modulating therapies prevent the recruitment and activati...

    Elizabeth E. Gerber, Elena M. Gallo, Stefani C. Fontana, Elaine C. Davis in Nature (2013)

  17. No Access

    Book

    Scleroderma

    From Pathogenesis to Comprehensive Management

    John Varga, Christopher P. Denton, Fredrick M. Wigley (2012)

  18. No Access

    Chapter

    Raynaud’s Phenomenon and Sjögren’s Syndrome

    In 1862, Maurice Raynaud published his medical school thesis in which he argued that some people have transient digital ischemia when exposed to cold temperatures. It is now recognized that skin blood flow in ...

    Fredrick M. Wigley in Sjögren’s Syndrome (2012)

  19. Article

    Correction: Corrigendum: Genome-wide association study of systemic sclerosis identifies CD247 as a new susceptibility locus

    Nat. Genet. 42, 426–429 (2010); published online 11 April 2010; corrected after print 23 March 2011 In the version of this article initially published, incorrect affiliations were published for Lorenzo Beretta...

    Timothy R D J Radstake, Olga Gorlova, Blanca Rueda, Jose-Ezequiel Martin in Nature Genetics (2011)

  20. No Access

    Chapter

    Painful Digital Ulcers in a Scleroderma Patient with Raynaud’s Phenomenon

    Raynaud’s phenomenon (RP) is seen in over 95% of patients with SSc and is often the first symptom of the disease. Scleroderma vascular disease is secondary to an obliterative vasculopathy causing not only vaso...

    Fredrick M. Wigley, Peter K. Wung M.D., MHS. in Case Studies in Systemic Sclerosis (2011)

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