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Juvenile Systemic Sclerosis Differential Diagnoses

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

Diagnostic Considerations

Many of the pediatric systemic rheumatic diseases, including: dermatomyositis, systemic lupus erythematosus (SLE), vasculitis syndromes, and juvenile rheumatoid arthritis/juvenile idiopathic arthritis (JRA/JIA), have numerous clinical features in common. These clinical similarities, in addition to the common laboratory findings that may be found among these diseases, can lead to diagnostic difficulties. Occasionally, even experienced rheumatologists may have some difficulty in making a definitive diagnosis.

Overlap syndromes are well described and mentioned in this topic because systemic sclerosis, dermatomyositis, SLE, systemic vasculitis, and systemic JRA (Still disease) have been previously noted in various combinations within pediatric patients or in the evolution of any single rheumatic disease of childhood.

Establishing a specific diagnosis is important for prognosis and treatment. In some patients, a specific diagnosis may take months to years to establish. With recent attempts at better defining diagnostic criteria for juvenile systemic sclerosis (JSSc), the time between onset of symptoms and diagnosis will hopefully decrease. Some of the most challenging pediatric rheumatologic diseases to differentiate include those that overlap between scleroderma and dermatomyositis. Systemic vasculitis may mimic almost any of the other systemic rheumatic diseases.

A significant number of children with an underlying systemic rheumatic diathesis evolve over time fulfilling diagnostic criteria for different rheumatic diseases at any point in time. The current author has cared for children with definite acute rheumatic fever (ARF) that evolved to pauciarticular JRA, then to polyarticular JRA, and finally to SLE. Others began as localized myositis that evolved to polymyositis, then to dermatomyositis, to scleroderma, then to JSSc. Still others started as JRA and evolved to SLE, then to systemic vasculitis, then to aplastic anemia, and then to death from lymphoblastic lymphoma.

Differential Diagnoses

 
 
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.

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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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