eMedicine Specialties > Pediatrics: General Medicine > Allergy & Immunology

Juvenile Systemic Sclerosis: Follow-up

Author: Luke M Webb, MD, Fellow, Department of Allergy and Immunology, Walter Reed Army Medical Center
Coauthor(s): David J Schwartz, MD, Fellow, Department of Allergy and Immunology, Walter Reed Army Medical Center; Cecilia P Mikita, MD, MPH, Associate Program Director, Allergy-Immunology Fellowship, Assistant Professor of Pediatrics and Medicine, Uniformed Services University of the Health Sciences; Hospital Intern Director, Staff Allergist/Immunologist, Walter Reed Army Medical Center
Contributor Information and Disclosures

Updated: Jul 20, 2009

Follow-up

Further Inpatient Care

  • Patients with juvenile systemic sclerosis (JSSc) are admitted to the hospital on a limited basis, and then only to treat critical care emergencies, often in an ICU (eg, renal crisis, impending respiratory failure, surgical emergencies).
  • Other treatments should be accomplished in ambulatory care units or at home whenever possible without sacrificing patient safety.

Further Outpatient Care

  • Outpatient care needs to be individualized.
  • Stress independent activities of daily living, good nutrition, and a healthy and positive attitude.
  • Monitor medication and admit patients to the hospital only for definitive medical or surgical treatment.
  • The team (see Medical Care) is integral to the outpatient care of a child with systemic sclerosis.
  • The importance of continued schooling and independent living cannot be overemphasized.

Inpatient & Outpatient Medications

  • As with the treatment of many chronic diseases, treatment of adolescents and young adults with juvenile systemic sclerosis may be complicated by poor compliance. Individual responsibility is encouraged and emphasized by the team, particularly the nurse educator and social worker.
  • Further, appropriate support should be provided for family members and caregivers.  School attendance, independence, and compliance with medication and exercise programs need to be encouraged and reinforced.

Transfer

  • The expertise of an experienced pediatric rheumatologist is required.

Deterrence/Prevention

  • Idiopathic disease is not preventable.
  • Conditions that resemble juvenile systemic sclerosis secondary to toxins or metabolic perturbations may be quite amenable to preventive principles.

Complications

Prognosis

  • Although a severely debilitating disease with significant morbidity and mortality, the prognosis of children with systemic sclerosis appears to be better than in adult patients with systemic sclerosis.
  • The survival of juvenile systemic sclerosis at 5 years, 10 years, 15 years, and 20 years after diagnosis is 89%, 80-87.4%, 74-87.4%, and 69-82.5%, respectively. These survival rates are significantly higher than those reported in patients with adult-onset systemic sclerosis.
  • The most common causes of death are secondary to complications of cardiac, renal, or pulmonary involvement. A recent study by Martini et al studied 134 patients with juvenile systemic sclerosis.5 All patients who died had a diffuse form of the disease, with rapid progression and early signs of internal organ involvement, suggesting two courses: a more rapid course and a slow course with lower mortality.
  • As discussed above, regular and frequent monitoring of cardiac, renal, and pulmonary function are critical while caring for this patient population. However, further research is still required to better define disease activity so that a standardized approach to treatment of this disease may be established.

Patient Education

  • As with all chronic disease, systemic sclerosis requires continual reinforcement of education concerning adequate nutrition, independent mobility, and control of potential adverse effects of medication.

Miscellaneous

Medicolegal Pitfalls

  • Issues of missed diagnosis (commonly another rheumatic disease) and obtaining informed consent for sometimes heroic therapies (leading to wrongful death litigation) constitute the major legal pitfalls. For this reason, experienced specialists are essential in the diagnosis and treatment of patients with systemic sclerosis.
  • These patients require a treatment team with a pediatric rheumatologist as team leader and case manager.
 
Acknowledgments

Completion of this review was greatly aided by the expertise and groundwork laid by the previous author of this article, Donald Person, MD. The authors are deeply indebted to his work on prior versions of this topic.



More on Juvenile Systemic Sclerosis

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Multimedia: Juvenile Systemic Sclerosis
References
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References

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

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

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

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Further Reading

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Keywords

 juvenile systemic sclerosis, JSSc, scleroderma, Scl, progressive systemic sclerosis, PSS, progressive pulmonary fibrosis, cutaneous sclerosis, linear scleroderma, en coup de sabre, morphea, CREST syndrome, calcinosis, Raynaud phenomenon, Raynaud's phenomenon, Raynaud's, esophageal hypomotility, sclerodactyly, telangiectasia, dermatosclerosis, sclerosis corii, sclerosis cutanea, connective tissue disease, connective tissue disease, pericarditis, dilated cardiomyopathy, polyarthralgia, polyarthritis, systemic lupus erythematosus, SLE, gastroesophageal reflux, vitiligo, heart failure, treatment, diagnosis

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Contributor Information and Disclosures

Author

Luke M Webb, MD, Fellow, Department of Allergy and Immunology, Walter Reed Army Medical Center
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, and American College of Physicians
Disclosure: Nothing to disclose.

Coauthor(s)

David J Schwartz, MD, Fellow, Department of Allergy and Immunology, Walter Reed Army Medical Center
David J Schwartz, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American College of Allergy, Asthma and Immunology, and American Medical Association
Disclosure: Nothing to disclose.

Cecilia P Mikita, MD, MPH, Associate Program Director, Allergy-Immunology Fellowship, Assistant Professor of Pediatrics and Medicine, Uniformed Services University of the Health Sciences; Hospital Intern Director, Staff Allergist/Immunologist, Walter Reed Army Medical Center
Cecilia P Mikita, MD, MPH is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American College of Allergy, Asthma and Immunology, and Clinical Immunology Society
Disclosure: Nothing to disclose.

Medical Editor

Ann O'Neill Shigeoka, MD †, Former Clinical Associate Professor, Department of Pediatrics, Division of Immunology-Rheumatology, University of Utah School of Medicine
Ann O'Neill Shigeoka, MD † is a member of the following medical societies: American Federation for Medical Research, Clinical Immunology Society, Pediatric Infectious Diseases Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

David J Valacer, MD, Consulting Staff, Hoffman La Roche Pharmaceuticals
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, and New York Academy of Sciences
Disclosure: Nothing to disclose.

CME Editor

David Pallares, MD, Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville
David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology
Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD, Associate Professor, Division of Pulmonary Allergy/Immunology and Infectious Diseases, Department of Pediatrics, UMDNJ-New Jersey Medical School
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 Mucosal Immunology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

 
 
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