Juvenile Systemic Sclerosis Differential Diagnoses

Updated: Nov 14, 2022
  • Author: Donald A Person, MD, FAAP, FACR; Chief Editor: Harumi Jyonouchi, MD  more...
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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/JIA and evolved to SLE, then to systemic vasculitis, then to aplastic anemia, and then to death from lymphoblastic lymphoma.

Differential Diagnoses