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Juvenile Systemic Sclerosis Medication

  • Author: Donald A Person, MD, FAAP, FACR; Chief Editor: Harumi Jyonouchi, MD  more...
Updated: Sep 12, 2014

Calcium Channel Blocking Agents

Class Summary

These agents are helpful in treating patients who develop tissue ischemia of digital tip ulcers. Dihydropyridine calcium channel blockers (nifedipine, nicardipine) have more pronounced peripheral vasodilatory effect.

Nifedipine (Adalat, Procardia)


Effective in vasospastic conditions.

Relaxes coronary smooth muscle and produces coronary vasodilation, which, in turn, improves myocardial oxygen delivery.

Nicardipine (Cardene)


For IV use when PO route is not possible.

Individualized slow IV infusion at a concentration of 0.1 mg/mL with constant infusion; blood pressure falls within min (50% decrease in 45 min)


ACE Inhibitors

Class Summary

Previously, hypertensive renal crisis was the most dreaded complication of systemic sclerosis. However, with the development of the ACE inhibitors (eg, captopril, enalapril), the prognosis of such patients has improved remarkably.

Captopril (Capoten)


Prevents conversion of angiotensin I to angiotensin II (a potent vasoconstrictor), resulting in lower aldosterone secretion.

Enalapril (Vasotec)


Competitive inhibitor of ACE. Reduces angiotensin II levels, decreasing aldosterone secretion.


Angiotensin Ii Receptor Antagonist

Class Summary

Consider these agents if unable to use ACE inhibitors for hypertension, renal insufficiency, and renal crisis.

Losartan (Cozaar)


Nonpeptide angiotensin II–receptor antagonist that blocks the vasoconstrictor and aldosterone-secreting effects of angiotensin II. May induce a more complete inhibition of the renin-angiotensin system than ACE inhibitors. Does not affect the response to bradykinin and is less likely to be associated with cough and angioedema. For patients unable to tolerate ACE inhibitors. Less effective in patients with scleroderma than with primary Raynaud phenomenon. May modify some serum markers of vascular damage and possibly modulate some of the underlying tissue damage in scleroderma.


Nonsteroidal Anti-inflammatory Agents

Class Summary

These agents are used to treat the arthritis of systemic sclerosis. They have analgesic, antiinflammatory, and antipyretic activities. Their mechanism of action is not known, but may inhibit cyclooxygenase activity and prostaglandin synthesis. Other mechanisms may also occur (eg, inhibition of leukotriene synthesis, lysosomal enzyme release, lipoxygenase activity, neutrophil aggregation, various cell-membrane functions).

Naproxen (Aleve, Naprosyn, Anaprox)


Anti-inflammatory of the arylacetic acid group of derivatives with good benefit-risk ratio. PO-administrated drugs with a half-life of 12 h.

Ibuprofen (Motrin, Ibuprin)


Anti-inflammatory of the propionic acid group with good benefit-risk ratio. PO-administrated drugs with a half-life of 2-3 h, respectively.


Immunosuppressive Agents

Class Summary

Interstitial lung disease associated with systemic sclerosis is a major therapeutic challenge. Treatment with high-dose corticosteroids, methotrexate, and cyclophosphamide has shown variable response among different patients. Slower-acting antirheumatic drug or disease-modifying antirheumatic drugs (eg, penicillamine) have been used for their anti-inflammatory and anticollagen effects.

Cyclophosphamide (Cytoxan, Neosar)


Chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA.

Penicillamine (Cuprimine, Depen)


The fact that penicillamine interferes with collagen cross-linking in vitro is the oft-quoted basis for its use in systemic sclerosis.

Retrospective studies using historic controls suggested minimal beneficial effect.

Methotrexate (Rheumatrex, MTX, Trexall)


Antimetabolite used for immunomodulatory therapy.

Prednisone (Deltasone, Orasone)


Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production.


Immune Globulin

Class Summary

This agent is purified preparation of gamma globulin. It is derived from large pools of human plasma and comprises 4 subclasses of antibodies, approximating the distribution of human serum. It is used for immune modulation.

Immune globulin intravenous (Carimune NF, Sandoglobulin, Gamunex, Privigen)


Neutralize circulating myelin antibodies through anti-idiotypic antibodies; down-regulates proinflammatory cytokines, including INF-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells and augments suppressor T cells; blocks complement cascade; promotes remyelination; may increase CSF IgG (10%).


Prostacyclin Analogues

Class Summary

Prostacyclins, specifically epoprostenol, are indicated for the long-term treatment of pulmonary hypertension associated with scleroderma disease. They may also reduce pain and the occurrence of digital ulcerations. Additionally, prostacyclins may improve lesion scores and ischemic lesion scores. Other prostacyclin analogues being investigated for use in systemic sclerosis include an orally administered prostacyclin (beraprost), iloprost (Ventavis), and subcutaneously administered Remodulin (treprostinil). Iloprost and treprostinil are currently US Food and Drug Administration (FDA)-approved for pulmonary artery hypertension. Iloprost is available as an aerosolized inhaled agent, but an intravenous form is currently under investigation.

Epoprostenol (Flolan)


Analogue of PGI2 has potent vasodilatory properties, immediate onset of action, and half-life of approximately 5 min. Potent pulmonary and systemic vasodilator. In addition to vasodilator properties, contributes to inhibition of platelet aggregation and plays role in inhibition of smooth muscle proliferation. Requires permanent, central venous catheter together with portable infusion pump for IV administration. Indicated for long-term IV treatment of primary pulmonary hypertension and pulmonary hypertension associated with the scleroderma spectrum of disease in NYHA Class III and Class IV patients in whom conventional therapy does not produce an adequate response.


Gastric Acid Secretion Inhibitor

Class Summary

This agent is indicated for prevention or treatment of gastroesophageal reflux disease.

Omeprazole (Prilosec)


Decreases gastric acid secretion by inhibiting the parietal cell H+/K+ -ATPase pump. Used for up to 4-8 weeks to treat and relieve symptoms of active duodenal ulcers. May use for up to 8 wk to treat all grades of erosive esophagitis.

Contributor Information and Disclosures

Donald A Person, MD, FAAP, FACR Medical Director (Emeritus), Pacific Island Healthcare Project; Expert Consultant in Pediatrics, Pediatric Rheumatology, Telemedicine, and Scientific Review, Tripler Army Medical Center; Professor of Pediatrics, F Edward Herbert School of Medicine, Uniformed Services University of the Health Sciences; Clinical Professor of Pediatrics and Public Health (Retired), University of Hawaii, John A Burns School of Medicine

Donald A Person, MD, FAAP, FACR is a member of the following medical societies: Society for Experimental Biology and Medicine, American Academy of Pediatrics, American College of Rheumatology, American Medical Association, American Pediatric Society, American Society for Microbiology, American Society of Tropical Medicine and Hygiene, Association of Military Surgeons of the US, Clinical Immunology Society, Federation of American Societies for Experimental Biology, Pediatric Infectious Diseases Society, Society for Pediatric Research

Disclosure: Nothing to disclose.


Cecilia P Mikita, MD, MPH Associate Program Director, Allergy-Immunology Fellowship, Associate Professor of Pediatrics and Medicine, Uniformed Services University of the Health Sciences; Staff Allergist/Immunologist, Walter Reed National Military Medical Center

Cecilia P Mikita, MD, MPH is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology

Disclosure: Nothing to disclose.

Luke M Webb, MD Staff Physician, Department of Allergy and Immunology, Evans Army Community Hospital

Luke M Webb, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology

Disclosure: Nothing to disclose.

David J Schwartz, MD Staff Physician, Department of Allergy and Immunology, Eisenhower Army Medical Center

David J Schwartz, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Allergy, Asthma and Immunology

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David J Valacer, MD 

David J Valacer, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association for the Advancement of Science, American Thoracic Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD Faculty, Division of Allergy/Immunology and Infectious Diseases, Department of Pediatrics, Saint Peter's University Hospital

Harumi Jyonouchi, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Medical Association, Clinical Immunology Society, New York Academy of Sciences, Society for Experimental Biology and Medicine, Society for Pediatric Research, Society for Mucosal Immunology

Disclosure: Nothing to disclose.

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An 8-year-old girl with overlap syndrome with evolution to progressive systemic sclerosis (PSS).
Photo of hands revealing sclerodactyly. This demonstrates the progression of disease over 7 years.
Chest radiograph revealing diffuse, coarse interstitial marking with bilateral lower lobe bronchiectasis.
Axial CT scan of the chest of a 15-year-old female adolescent with progressive systemic sclerosis (PSS).
Esophagram revealing dysmotility.
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