Systemic Sclerosis Medication

Updated: Apr 16, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Medication

Medication Summary

Attaining a patient-acceptable symptom state in scleroderma can be challenging. [61] 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. [62]

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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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