Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

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)

Next

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.

Previous
Next

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.

Previous
Next

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.

Previous
Next

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.

Previous
Next

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%).

Previous
Next

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.

Previous
Next

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.

Previous
 
 
Contributor Information and Disclosures
Author

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.

Coauthor(s)

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.

References
  1. Johnson SR, Swiston JR, Granton JT. Prognostic factors for survival in scleroderma associated pulmonary arterial hypertension. J Rheumatol. 2008 Aug. 35(8):1584-90. [Medline].

  2. Martini G, Foeldvari I, Russo R, Cuttica R, Eberhard A, Ravelli A, et al. Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database. Arthritis Rheum. 2006 Dec. 54(12):3971-8. [Medline].

  3. [Guideline] Kowal-Bielecka O, Landewé R, Avouac J, Chwiesko S, Miniati I, Czirjak L, et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis. 2009 May. 68(5):620-8. [Medline].

  4. Martini G, Vittadello F, Kasapçopur O, Magni Manzoni S, Corona F, Duarte-Salazar C, et al. Factors affecting survival in juvenile systemic sclerosis. Rheumatology (Oxford). 2009 Feb. 48(2):119-22. [Medline].

  5. ss.

  6. Black CM, Denton CP. Therapy of Systemic Sclerosis. Van de Putte LBA, Furst DE, Williams HJ, van Riel PLCM, eds. Therapy of Systemic Rheumatic Disorders. 1998. 495-545.

  7. Bottoni CR, Reinker KA, Gardner RD, Person DA. Scleroderma in childhood: a 35-year history of cases and review of the literature. J Pediatr Orthop. 2000 Jul-Aug. 20(4):442-9. [Medline].

  8. Foeldvari I. New developments in juvenile systemic and localized scleroderma. Rheum Dis Clin North Am. 2013 Nov. 39(4):905-20. [Medline].

  9. Foeldvari I, Tyndall A, Zulian F, et al. Juvenile and young adult-onset systemic sclerosis share the same organ involvement in adulthood: data from the EUSTAR database. Rheumatology (Oxford). 2012 Oct. 51(10):1832-7. [Medline].

  10. Foti R, Leonardi R, Rondinone R, Di Gangi M, Leonetti C, Canova M, et al. Scleroderma-like disorders. Autoimmun Rev. 2008 Feb. 7(4):331-9. [Medline].

  11. Gerhold K, Becker MO. Nailfold capillaroscopy in juvenile rheumatic diseases: known measures, patterns and indications. Clin Exp Rheumatol. 2014 Jun 6. [Medline].

  12. Hoeper MM. Pulmonary hypertension in collagen vascular disease. Eur Respir J. 2002 Mar. 19(3):571-6. [Medline].

  13. Kaal SE, van Den Hoogen FH, de Jong EM, Viëtor HE. Systemic sclerosis: new insights in autoimmunity. Proc Soc Exp Biol Med. 1999 Oct. 222(1):1-8. [Medline].

  14. LeRoy EC. Pathogenesis of Systemic Sclerosis (scleroderma). Koopman WJ, ed. Arthritis and Allied Conditions. 1997. 1481-90.

  15. Levy BD. Eicosanoids in scleroderma: lung disease hangs in the balance. Arthritis Rheum. 2005 Dec. 52(12):3693-7. [Medline].

  16. Nagaya N. Drug therapy of primary pulmonary hypertension. Am J Cardiovasc Drugs. 2004. 4(2):75-85. [Medline].

  17. Poormoghim H, Lucas M, Fertig N, Medsger TA Jr. Systemic sclerosis sine scleroderma: demographic, clinical, and serologic features and survival in forty-eight patients. Arthritis Rheum. 2000 Feb. 43(2):444-51. [Medline].

  18. Rabinovich CE. Challenges in the diagnosis and treatment of juvenile systemic sclerosis. Nat Rev Rheumatol. 2011 Oct 11. 7(11):676-80. [Medline].

  19. Reiff A, Weinberg KI, Triche T, et al. T lymphocyte abnormalities in juvenile systemic sclerosis patients. Clin Immunol. 2013 Oct. 149(1):146-55. [Medline].

  20. Rosenkranz ME, Agle LM, Efthimiou P, Lehman TJ. Systemic and localized scleroderma in children: current and future treatment options. Paediatr Drugs. 2006. 8(2):85-97. [Medline].

  21. Russo RA, Katsicas MM. Clinical characteristics of children with Juvenile Systemic Sclerosis: follow-up of 23 patients in a single tertiary center. Pediatr Rheumatol Online J. 2007 May 1. 5:6. [Medline]. [Full Text].

  22. Shimizu M, Hashida Y, Ueno K, et al. Successful treatment with bosentan for pulmonary hypertension and reduced peripheral circulation in juvenile systemic sclerosis. Pediatr Cardiol. 2011 Oct. 32(7):1040-2. [Medline].

  23. Silver RM. Variant forms of scleroderma. Koopman WJ, ed. Arthritis and Allied Conditions. 1997. 1465-80.

  24. Steen V. Advancements in diagnosis of pulmonary arterial hypertension in scleroderma. Arthritis Rheum. 2005 Dec. 52(12):3698-700. [Medline].

  25. Vancheeswaran R, Black CM, David J, Hasson N, Harper J, Atherton D, et al. Childhood-onset scleroderma: is it different from adult-onset disease. Arthritis Rheum. 1996 Jun. 39(6):1041-9. [Medline].

  26. Zulian F. Systemic sclerosis and localized scleroderma in childhood. Rheum Dis Clin North Am. 2008 Feb. 34(1):239-55; ix. [Medline].

  27. Zulian F, Martini G. Childhood systemic sclerosis. Curr Opin Rheumatol. 2007 Nov. 19(6):592-7. [Medline].

  28. [Guideline] Zulian F, Woo P, Athreya BH, Laxer RM, Medsger TA Jr, Lehman TJ, et al. The Pediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for juvenile systemic sclerosis. Arthritis Rheum. 2007 Mar 15. 57(2):203-12. [Medline].

 
Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.