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Juvenile Systemic Sclerosis Follow-up

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

Further Outpatient Care

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  • 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.
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Further Inpatient Care

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  • 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 (renal crisis, cardiac and/or respiratory failure, surgical emergencies).
  • Other treatments should be accomplished in ambulatory care units or at home whenever possible without sacrificing patient safety.
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Inpatient & Outpatient Medications

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  • As is the case with the treatment of many chronic diseases of childhood, treatment of adolescents and young adults with JSSc is often 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.
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Transfer

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  • The expertise of an experienced pediatric rheumatologist is required.
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Deterrence/Prevention

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

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Prognosis

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  • Although a severely debilitating disease with significant morbidity and mortality, the prognosis of children with JSSc has been reported to be better than in adult patients with systemic sclerosis.
  • The survival of JSSc 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 and failure. A recent study by Martini et al studied 134 patients with JSSc.[6] 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.
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Patient Education

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  • 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.
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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
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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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