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Juvenile Systemic Sclerosis Treatment & Management

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

Medical Care

Pharmacologic management of juvenile systemic sclerosis (JSSc) has been abysmal. The treatment of children with chronic rheumatic disease is multifaceted and requires attention to general health measures including; nutrition, rest, maximizing school attendance, and exercise. Treating a child with systemic sclerosis requires a team approach, ideally including a nurse educator, physical therapist, occupational therapist, nutritionist, and social worker. No treatment or combination of medical or surgical treatments has proven unequivocally efficacious in JSSc. However, therapeutic strategies have been developed that are directed toward the individual patient and the organ systems involved in that patient.

Given the lack of consensus that exists regarding any pharmaceutical management of JSSc, the European League Against Rheumatism (EULAR) task force, which included pediatric rheumatologists and representatives of patients with JSSc, was convened and in 2007, attempted to establish some treatment recommendations. The group established a final set of 14 recommendations. Among experts in the field, a consensus of greater than 85% was reached on 9 of these 14 recommendations.[4]

  • Meta-analysis on dihydropyridine-type antagonist and prostanoids indicated that nifedipine and intravenous iloprost reduce both the severity and frequency of Raynaud phenomenon attacks in patients with JSSc. Dihydropyridine-type antagonist (nifedipine) should be considered first-line therapy for juvenile systemic sclerosis–associated Raynaud phenomenon. Intravenous (IV) prostanoids (iloprost) should be used to treat severe Raynaud phenomenon.
  • Two randomized clinical trials have demonstrated that intravenous prostanoid (particularly iloprost) are effective in healing digital ulcers.
  • Despite some conflicting data and its known toxicity, cyclophosphamide should be considered for the treatment of JSSc–related interstitial lung disease. As with the use of cyclophosphamide in other conditions (childhood SLE) in order to prevent hemorrhagic cystitis, adequate hydration and frequent voiding must be emphasized.
  • Glucocorticoids (most commonly prednisone), have few indications in treating juvenile systemic sclerosis. The use of glucocorticoids in treating juvenile systemic sclerosis–associated myositis, arthritis, and tenosynovitis, may be indicated. However, several studies have demonstrated a higher incidence of renal crisis in patients with juvenile systemic sclerosis treated with glucocorticoids emphasizes; thus, careful monitoring of blood pressure and renal function is needed in these patients.
  • ACE inhibitors and angiotensin receptor blockers (ARBs) are considered to be the most effective and safest treatment option for long term management of hypertension, renal insufficiency, and renal crisis in patients with juvenile systemic sclerosis.
  • Methotrexate has been shown to improve a clinical monitoring scale known as the skin score in early diffuse systemic sclerosis in adults. Although studies in children are not currently available, expert opinion suggests that methotrexate would be the treatment of choice for skin manifestations of juvenile systemic sclerosis, particularly in the earlier phases of the disease.
  • Recommendations for treatment of GI manifestations of juvenile systemic sclerosis include proton pump inhibitors (PPIs), including omeprazole for preventing or treating gastroesophageal reflux symptoms. Prokinetic agents for treating symptoms related to motility disturbances. Finally, rotating antibiotics to include doxycycline and ciprofloxacin to decreased bacterial overgrowth which can lead to malabsorption.
  • Interstitial lung disease associated with juvenile systemic sclerosis is a major therapeutic challenge. Treatment recommendations made by the EULAR group also included recommendations for treatment of pulmonary artery hypertension associated with juvenile systemic sclerosis. Randomized clinical trials have demonstrated improved exercise tolerance in patients with pulmonary artery hypertension with the use of several medications, including bosentan, sitaxsentan (clinical trials stopped worldwide because of liver injury), and sildenafil. Despite the emerging evidence that these medications may benefit these patients, many experts in the field have called for further pediatric trials before making general recommendations regarding these medications in pulmonary artery hypertension secondary to juvenile systemic sclerosis.
  • On December 10, 2010, Pfizer announced that sitaxsentan (also known as sitaxsentan; brand name of Thelin) is being withdrawn in regions where it is approved (the European Union, Canada, and Australia). Additionally, clinical trials for sitaxsentan are being discontinued worldwide. This decision was based on a review of safety information from clinical trials and post marketing reports of life-threatening idiosyncratic risk for liver injury.
  • Additional treatment considerations are as follows:
    • Vascular therapy may take on several forms and is not necessarily pharmacologic. Early on, Raynaud phenomenon may respond to avoidance of tobacco, cold exposure, and vasoconstricting medications.
    • Biofeedback has been helpful in some patients with the development of tissue ischemia of digital tip ulcers. Local management of digital ulcers is indicated.
    • The arthritis of systemic sclerosis may respond to nonsteroidal anti-inflammatory drugs (NSAIDs) but to a lesser extent than the arthritis associated with other connective tissue diseases.

Surgical Care

Because involvement in patients with juvenile systemic sclerosis widely varies, surgical management must be individualized.

  • Surgery to release contractures is occasionally indicated, and a few patients benefit from the surgical release of entrapped nerves.[5]
  • Emergency life-saving surgery in patients with juvenile systemic sclerosis who have a ruptured viscus may be required.
  • Amputation should be considered only in extreme cases and if no other therapeutic options have proven effective.
  • Sympathectomy as a treatment of the peripheral vascular disease is no longer used.


The treatment of severe, chronic and debilitating pediatric diseases such as JSSc requires a team approach.

  • The pediatric rheumatologist team leader must be a specialist experienced in the care of patients with JSSc.
  • The team should also include a pediatric gastroenterologist, pediatric nephrologist, pediatric cardiologist, and pediatric pulmonologist.
  • The team should also include a nurse educator, occupational therapist, physical therapist, nutritionist, and social worker.
  • Telemedicine may play a role in long-distance consultation and treatment of patients with JSSc who reside far from full-service institutions.
  • Recent experimental therapies that necessitate other consultations, such as stem cell, renal, cardiac, and lung transplantation, are beyond the scope of this discussion. The author does not recommend organ transplantation in any child with JSSc.
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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