Raynaud Phenomenon

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Fredrick M. Wigley - One of the best experts on this subject based on the ideXlab platform.

  • Treating Raynaud Phenomenon: Beyond staying warm.
    Cleveland Clinic Journal of Medicine, 2017
    Co-Authors: Samantha C. Shapiro, Fredrick M. Wigley
    Abstract:

    Raynaud Phenomenon is an overactive vascular response to cold and emotional stress that results in cutaneous color changes and sensory symptoms in the digits. It can be idiopathic (primary) or secondary to another condition; the latter can be more severe and more apt to lead to ischemic complications such as digital ulceration and even loss of digits. If nonpharmacologic interventions prove inadequate, then vasodilator agents are used.

  • editorial clinical trials in Raynaud s Phenomenon a spoonful of sugar pill makes the medicine go down in flames
    Arthritis & Rheumatism, 2017
    Co-Authors: James R Seibold, Fredrick M. Wigley
    Abstract:

    The peripheral vascular disease in systemic sclerosis (scleroderma) is a challenging problem because it presents with both vasospasm and an underlying structural vascular disease that leads not only to severe Raynaud Phenomenon but often critical digital ischemia with progressive tissue damage or acute ulceration. While current therapy with a calcium channel blocker alone or combined with another vasodilating agent provides some modest benefit, it does not provide adequate control of Raynaud's events in many patients with scleroderma. Vasodilating therapy alone also does not address the underlying biological process causing progressive vascular disease. This article is protected by copyright. All rights reserved.

  • management of Raynaud Phenomenon and digital ulcers in scleroderma
    Rheumatic Diseases Clinics of North America, 2015
    Co-Authors: Laura C Cappelli, Fredrick M. Wigley
    Abstract:

    Raynaud Phenomenon (RP) and associated digital ischemia can be among the most vexing clinical problems for patients with systemic sclerosis (scleroderma). Understanding the treatment approach to RP and associated ischemia and how to prevent digital ulcers is important for clinicians caring for these patients. This article reviews the management of RP and digital ischemic ulcers. The magnitude of the problem and pathophysiology of RP are first discussed, with an emphasis on recent advances in understanding of the disease process. Options for the practical pharmacologic and nonpharmacologic interventions for RP and digital ischemic ulcers are detailed.

  • a multi centre blinded randomised placebo controlled laboratory based study of mqx 503 a novel topical gel formulation of nitroglycerine in patients with Raynaud Phenomenon
    Annals of the Rheumatic Diseases, 2013
    Co-Authors: Laura K Hummers, Robert A Wise, Carin E Dugowson, Frederick J Dechow, Jeffrey Gregory, Joel E Michalek, Gayane Yenokyan, John Mcgready, Fredrick M. Wigley
    Abstract:

    Objective MQX-503 is a novel nitroglycerine preparation designed to absorb quickly and allow local vasodilatation in the skin. We examined the efficacy and tolerability of this medication in Raynaud Phenomenon (RP) in a laboratory-based study. Methods In this multi-centre, double-blind, randomised, placebo-controlled, cross-over study, subjects were treated with 0.5% or 1.25% nitroglycerine or placebo gel. Subjects received each dose twice in a randomised order. Each study session consisted of baseline laser Doppler measurements, study gel application and 5 min of cold chamber exposure (−20°C). Blood flow (BF) was measured at the end of exposure and for the next 120 min at set intervals. Other outcome measures included achievement of baseline BF; the time to achieve 50% and 70% baseline skin temperature (ST); and pain, tingling and numbness scores. Results 37 subjects completed 214 treatment periods. Time to achieve baseline BF was significantly shorter in the two treated groups (HR=1.77 and 2.02 for 0.5% and 1.25% vs placebo, respectively). The proportion of subjects achieving baseline BF was 45.8% for placebo, 66.2% for 0.5% and 69% for 1.25% (p=0.01 and p=0.002 for 0.5% and 1.25% vs placebo, respectively). No meaningful differences were seen in ST or pain/numbness/tingling scores. Treatment was well tolerated with no serious adverse events. Conclusions Treatment with MQX-503 caused a significant improvement in skin BF compared with placebo. Data from this proof of concept study suggest benefit of MQX-503 in subjects with RP.

  • scleroderma systemic sclerosis
    Clinical Immunology (Fourth Edition), 2013
    Co-Authors: John Varga, Fredrick M. Wigley
    Abstract:

    Abstract Systemic sclerosis is an orphan disease of unknown cause and complex pathogenesis. Multiple genetic variants that are common to systemic lupus, and other autoimmune diseases have been identified. Autoimmunity and fibrosis are prominent features of the disease. Systemic sclerosis predominantly affects women, follows a chronic and unpredictable course with multiple organs affected, and lacks effective disease-modifying therapies. In addition to variable degree of skin involvement and Raynaud Phenomenon, interstitial lung disease, widespread microvascular disease, intestinal tract pathology, and cardiovascular complications are common. Late-stage disease is usually accompanied by ischemic digital ulcers, pulmonary artery hypertension, pulmonary fibrosis, and small bowel dysfunction. The diffuse cutaneous form of the disease is associated with increased mortality. Although there are no approved disease-modifying therapies, carefully tailored and individualized management of specific organ-based complications can be highly effective in improving quality of life, reducing complications, and improving outcomes.

Alex Reyentovich - One of the best experts on this subject based on the ideXlab platform.

John Varga - One of the best experts on this subject based on the ideXlab platform.

  • scleroderma systemic sclerosis
    Clinical Immunology (Fourth Edition), 2013
    Co-Authors: John Varga, Fredrick M. Wigley
    Abstract:

    Abstract Systemic sclerosis is an orphan disease of unknown cause and complex pathogenesis. Multiple genetic variants that are common to systemic lupus, and other autoimmune diseases have been identified. Autoimmunity and fibrosis are prominent features of the disease. Systemic sclerosis predominantly affects women, follows a chronic and unpredictable course with multiple organs affected, and lacks effective disease-modifying therapies. In addition to variable degree of skin involvement and Raynaud Phenomenon, interstitial lung disease, widespread microvascular disease, intestinal tract pathology, and cardiovascular complications are common. Late-stage disease is usually accompanied by ischemic digital ulcers, pulmonary artery hypertension, pulmonary fibrosis, and small bowel dysfunction. The diffuse cutaneous form of the disease is associated with increased mortality. Although there are no approved disease-modifying therapies, carefully tailored and individualized management of specific organ-based complications can be highly effective in improving quality of life, reducing complications, and improving outcomes.

  • systemic sclerosis scleroderma a treatable multisystem disease
    American Family Physician, 2008
    Co-Authors: Monique Hinchcliff, John Varga
    Abstract:

    Systemic sclerosis (systemic scleroderma) is a chronic connective tissue disease of unknown etiology that causes widespread microvascular damage and excessive deposition of collagen in the skin and internal organs. Raynaud Phenomenon and scleroderma (hardening of the skin) are hallmarks of the disease. The typical patient is a young or middle-age woman with a history of Raynaud Phenomenon who presents with skin induration and internal organ dysfunction. Clinical evaluation and laboratory testing, along with pulmonary function testing, Doppler echocardiography, and high-resolution computed tomography of the chest, establish the diagnosis and detect visceral involvement. Patients with systemic sclerosis can be classified into two distinct clinical subsets with different patterns of skin and internal organ involvement, autoantibody production, and survival. Prognosis is determined by the degree of internal organ involvement. Although no disease-modifying therapy has been proven effective, complications of systemic sclerosis are treatable, and interventions for organ-specific manifestations have improved substantially. Medications (e.g., calcium channel blockers and angiotensin-II receptor blockers for Raynaud Phenomenon, appropriate treatments for gastroesophageal reflux disease) and lifestyle modifications can help prevent complications, such as digital ulcers and Barrett esophagus. Endothelin-1 receptor blockers and phosphodiesterase-5 inhibitors improve pulmonary arterial hypertension. The risk of renal damage from scleroderma renal crisis can be lessened by early detection, prompt initiation of angiotensin-converting enzyme inhibitor therapy, and avoidance of high-dose corticosteroids. Optimal patient care includes an integrated, multidisciplinary approach to promptly and effectively recognize, evaluate, and manage complications and limit end-organ dysfunction.

  • intravenous iloprost infusion in patients with Raynaud Phenomenon secondary to systemic sclerosis a multicenter placebo controlled double blind study
    Annals of Internal Medicine, 1994
    Co-Authors: Fredrick M. Wigley, Robert A Wise, James R Seibold, Deborah A Mccloskey, Gregory Kujala, Thomas A Medsger, Virginia Steen, John Varga, Sergio A Jimenez, Maureen Mayes
    Abstract:

    Objective: To evaluate the efficacy and safety of iloprost, a prostacyclin analog, administered intravenously in patients with Raynaud Phenomenon secondary to systemic sclerosis. Design: Multicente...

James R Seibold - One of the best experts on this subject based on the ideXlab platform.

  • editorial clinical trials in Raynaud s Phenomenon a spoonful of sugar pill makes the medicine go down in flames
    Arthritis & Rheumatism, 2017
    Co-Authors: James R Seibold, Fredrick M. Wigley
    Abstract:

    The peripheral vascular disease in systemic sclerosis (scleroderma) is a challenging problem because it presents with both vasospasm and an underlying structural vascular disease that leads not only to severe Raynaud Phenomenon but often critical digital ischemia with progressive tissue damage or acute ulceration. While current therapy with a calcium channel blocker alone or combined with another vasodilating agent provides some modest benefit, it does not provide adequate control of Raynaud's events in many patients with scleroderma. Vasodilating therapy alone also does not address the underlying biological process causing progressive vascular disease. This article is protected by copyright. All rights reserved.

  • rapidly progressive fatal interstitial lung disease in a patient with systemic sclerosis
    Nature Reviews Rheumatology, 2009
    Co-Authors: Kristine Phillips, Cathryn Byrnedugan, Eric Batterson, James R Seibold
    Abstract:

    Interstitial lung disease is a common manifestation of systemic sclerosis, and often leads to death in these patients. These authors describe the case of a previously healthy woman with acute systemic sclerosis who experienced an unusually fulminant course of interstitial lung disease, which did not respond to treatment with intravenous cyclophosphamide. Background. A 36-year-old woman developed new-onset Raynaud Phenomenon and rapidly progressive dyspnea over a 2-week period. A lung biopsy demonstrated pauci-inflammatory nonspecific pneumonitis, which proved refractory to systemic corticosteroid and intravenous cyclophosphamide therapy. Her preterminal course in an intensive care unit was typified by sequential organ failure. Postmortem examination showed extensive organ fibrosis, including severe diffuse alveolar damage and parenchymal fibrosis, and a notable lack of potentially treatable tissue inflammation. Investigations. Chest radiography, physical examination, screening for autoantibodies, measurement of serum creatinine, creatine phosphokinase, and brain natriuretic peptide levels, cardiac examination, pulmonary function tests, electrocardiography, transthoracic Doppler echocardiography, right heart catheterization, high-resolution thoracic CT, pulmonary ventilation/perfusion scan, lung biopsy. Diagnosis. Interstitial lung disease associated with diffuse systemic sclerosis. Management. Treatment with oxygen, oral and intravenous corticosteroids, mycophenolate mofetil and intravenous cyclophosphamide.

  • intravenous iloprost infusion in patients with Raynaud Phenomenon secondary to systemic sclerosis a multicenter placebo controlled double blind study
    Annals of Internal Medicine, 1994
    Co-Authors: Fredrick M. Wigley, Robert A Wise, James R Seibold, Deborah A Mccloskey, Gregory Kujala, Thomas A Medsger, Virginia Steen, John Varga, Sergio A Jimenez, Maureen Mayes
    Abstract:

    Objective: To evaluate the efficacy and safety of iloprost, a prostacyclin analog, administered intravenously in patients with Raynaud Phenomenon secondary to systemic sclerosis. Design: Multicente...

Todd S. Cutler - One of the best experts on this subject based on the ideXlab platform.

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