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Systemic Sclerosis Medication

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 02, 2016
 

Medication Summary

Treatment regimens have enormous diversity. Currently, no standard therapy is available for skin sclerosis. Raynaud phenomenon often responds to calcium channel blockers, and scleroderma kidney disease often responds to angiotensin-converting enzyme and angiotensin II inhibitors. The treatment depends on the presentation of systemic sclerosis. The efficacy and safety of lidocaine in treating scleroderma has been questions, with an analysis showing a lack of efficacy.[54]

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Immunomodulatory agents

Class Summary

These agents are used to stop disease progression. They act on the host's immune system; they suppress the immune system to prevent fibrosis.

Prednisone (Deltasone, Meticorten, Orasone, Sterapred)

 

Prednisone is an immunosuppressant used for the treatment of autoimmune disorders; it may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Prednisone stabilizes lysosomal membranes and suppresses lymphocytes and antibody production.

Methotrexate (Rheumatrex)

 

Methotrexate is an antimetabolite that inhibits dihydrofolate reductase, thereby hindering DNA synthesis and cell reproduction. A satisfactory response is observed 3-6 weeks after administration. Adjust the dose gradually to achieve a satisfactory response.

Chlorambucil (Leukeran, Leukeran)

 

Chlorambucil alkylates and cross-links strands of DNA, inhibiting DNA replication and RNA transcription.

Cyclosporine (Neoral, Sandimmune)

 

Cyclosporine is helpful in a variety of skin disorders.

Tacrolimus (Prograf)

 

Tacrolimus suppresses humoral (T-lymphocyte) immunity.

Cyclophosphamide (Neosar, Cytoxan)

 

Cyclophosphamide is chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve DNA cross-linking, which may interfere with the growth of healthy and neoplastic cells.

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Antifibrotic agents

Class Summary

These agents are used to decrease fibrosis by interference with collagen metabolism.

Penicillamine (Cuprimine, Cuprimine, Depen)

 

Penicillamine is a metal chelation agent used to treat arsenic poisoning. It forms soluble complexes with metals excreted in urine.

Colchicine

 

Colchicine decreases leukocyte motility and phagocytosis in inflammatory responses.

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Vasoactive agents

Class Summary

These agents are used to modify disease with its vasoactive actions.

Nifedipine (Adalat, Procardia)

 

Nifedipine relaxes coronary smooth muscle and causes coronary vasodilation, which, in turn, improves myocardial oxygen delivery. Sublingual administration generally is safe, despite theoretic concerns.

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Antiplatelet agents

Class Summary

These agents inhibit the cyclo-oxygenase system, decreasing the level of thromboxane A2, which is a potent platelet activator.

Aspirin (Bayer Buffered Aspirin, Bayer Aspirin, Anacin)

 

Aspirin inhibits prostaglandin synthesis, preventing the formation of platelet-aggregating thromboxane A2. It may be used in low doses to inhibit platelet aggregation and improve complications of venous stases and thrombosis.

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Antihypertensive agents

Class Summary

These agents are used to reduce blood pressure.

Reserpine

 

Reserpine depletes norepinephrine and epinephrine. This effect, in turn, depresses sympathetic nerve functions, decreasing the heart rate and lowering the arterial blood pressure.

Methyldopa (Aldomet)

 

Methyldopa stimulates central alpha-adrenergic receptors, resulting in decreased sympathetic outflow. Treatment results in inhibition of vasoconstriction.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Anna Zalewska, MD, PhD Professor of Dermatology and Venereology, Psychodermatology Department, Chair of Clinical Immunology and Microbiology, Medical University of Lodz, Poland

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: XOMA; Biogen/IDEC; Novartis; Janssen Biotech, Abbvie, CSL pharma<br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor and intermittent author; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Mark W Cobb, MD Consulting Staff, WNC Dermatological Associates

Mark W Cobb, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society of Dermatopathology

Disclosure: Nothing to disclose.

Acknowledgements

Bozena Dziankowska-Bartkowiak, MD, PhD Consulting Staff, Department of Dermatology, University Hospital, Medical University of Lodz, Poland

Disclosure: Nothing to disclose.

Anna Sysa-Jedrzejowska, MD, PhD Head, Professor, Department of Dermatology and Venereology, Medical University of Lodz, Poland

Disclosure: Nothing to disclose.

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Face of 65-year old woman with systemic sclerosis and skin thickening of 20 years' duration: Note the pinched nose, taut skin with numerous telangiectasias, and retraction of the lips.
Telangiectasias affecting the face: They are pronounced and numerous, especially in the atrophic phase of the disease. Radical furrowing around the mouth is also characteristic in the later stage of the disease.
Raynaud phenomenon of the hands: Symmetrical acral vasospasm is present, with characteristic pallor, cyanosis, suffusion, and a sense of fullness and tautness.
Puffy appearance of the woman's hand in the edematous phase of early scleroderma.
In systemic sclerosis, ulceration at the tip of the finger is regarded to be secondary to ischemia.
Hand of a woman with scleroderma of several years' duration: The thickened, tight, thin skin over the fingers is the result of self-amputation of the distal phalanx due to ischemia. Moderately severe flexion contractures of the fingers are present.
In systemic sclerosis, skin hyperpigmentation of the lower legs is surrounded by areas of hypopigmentation. The result is a salt-and-pepper appearance.
 
 
 
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