D-penicillamine in systemic sclerosis? Yes!

Medsger, Thomas A.; Lucas, Mary; Wildy, Kathryn S.; Baker, Carol J.

doi:10.1080/030097401316909503

Cited by 16 publications

(2 citation statements)

References 4 publications

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“…Multiple open label trials have consistently shown improvement of skin score of SSc patients treated with D-Pen [115,116]. Furthermore, the observation of recurrence of diffuse SSc skin changes following discontinuation and subsequent improvement of the skin after resuming D-Pen, support its effectiveness [117]. In contrast with the results of these studies, a controlled study evaluating low dose vs. high dose D-Pen did not show statistical differences between the groups.…”

Section: Treatment Of Rapidly Progressive Sscmentioning

confidence: 99%

Treatment of rapidly progressive systemic sclerosis: current and futures perspectives

Mendoza

Mansoor

Jimenez

2015

Expert Opinion on Orphan Drugs

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Introduction Systemic Sclerosis (SSc) is a systemic autoimmune disease characterized by severe and often progressive cutaneous, pulmonary, cardiac and gastrointestinal tract fibrosis, cellular and humoral immunologic alterations, and pronounced fibroproliferative vasculopathy. There is no effective SSc disease modifying therapy. Patients with rapidly progressive SSc have poor prognosis with frequent disability and very high mortality. Areas Covered This paper reviews currently available therapeutic approaches for rapidly progressive SSc and discuss novel drugs under study for SSc disease modification. Expert Opinion The extent, severity, and rate of progression of SSc skin and internal organ involvement determines the optimal therapeutic interventions for SSc. Cyclophosphamide for progressive SSc-associated interstitial lung disease and mycophenolate for rapidly progressive cutaneous involvement have shown effectiveness. Methotrexate has been used for less severe skin progression and for patients unable to tolerate mycophenolate. Rituximab was shown to induce improvement in SSc-cutaneous and lung involvement. Autologous bone marrow transplantation is reserved for selected cases in whom poor survival risk outweighs the high mortality rate of the procedure. Novel agents capable of modulating fibrotic and inflammatory pathways involved in SSc pathogenesis, including tocilizumab, pirfenidone, tyrosine kinase inhibitors, lipid lysophosphatidic acid 1, and NOX4 inhibitors are currently under development for the treatment of rapidly progressive SSc.

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Section: Treatment Of Rapidly Progressive Sscmentioning

confidence: 99%

Treatment of rapidly progressive systemic sclerosis: current and futures perspectives

Mendoza

Mansoor

Jimenez

2015

Expert Opinion on Orphan Drugs

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“…In addition, D-penicillamine inhibits collagen biosynthesis and have immunosuppressive activity. [ 22 ] As scleroderma can give rise to a variety of oral problems, so maintenance of existing dentition is important. A small mouth in scleroderma makes lip, mouth movements, and oral hygiene difficult.…”

Section: Discussionmentioning

confidence: 99%

Progressive systemic sclerosis with intraoral manifestations: A case report and review

et al. 2016

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The word scleroderma comes from two Greek words, “sclero” meaning hard and “derma” meaning skin. Scleroderma or progressive systemic sclerosis (PSS), a rare condition, was first characterized as a single condition in 1752 by Curzio of Naples. It generally affects woman between 30 and 50 years of age and has a low prevalence. Scleroderma is a disease of the immune system, blood vessels, and connective tissue. Dermal manifestations include stiff, tight, and shiny skin usually of the hands and feet due to swelling and thickening of the connective tissue as they become fibrotic or scarred. Other symptoms include difficulty in swallowing, bloating, abdominal pain, tiredness, lack of energy, weight loss, aching muscles, joints, and bones. The vital organs that may get involved are lungs, heart, and kidneys. We present a case report of PSS in a 45-year-old female patient with characteristic systemic and oral manifestations.

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Systemic Sclerosis (Systemic Scleroderma)

Orteu

Denton

2016

Rook's Textbook of Dermatology, Ninth Edition

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Systemic sclerosis is a multisystem autoimmune disease that causes fibrosis in the skin and internal organs with associated vascular and inflammatory manifestations including Raynaud phenomenon. It has high mortality due to cardiac, pulmonary and renal complications and substantial morbidity from pruritus, pain, digital ulceration, calcinosis, telangiectases and gastrointestinal tract and musculoskeletal involvement. Different subsets of the disease are recognized based on the extent of skin involvement, the presence of hallmark autoantibody reactivities, and more recently on gene expression signatures in the skin. Up to 20% of systemic sclerosis cases have features of overlap with another autoimmune rheumatic disease. Limited and diffuse subsets carry different risks of organ‐based complications. Early and systematic screening to identify the burden of disease and individualize risk is a cornerstone of modern management, which has led to the earlier institution of therapy and significant improvements in outcome over the past two decades.

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D-penicillamine in systemic sclerosis? Yes!

Cited by 16 publications

References 4 publications

Treatment of rapidly progressive systemic sclerosis: current and futures perspectives

Treatment of rapidly progressive systemic sclerosis: current and futures perspectives

Progressive systemic sclerosis with intraoral manifestations: A case report and review

Systemic Sclerosis (Systemic Scleroderma)

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