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Chronic polyarthritis as the first manifestation of juvenile systemic lupus erythematosus patients

E G N Cavalcante1, N E Aikawa, R G Lozano

  • 1Pediatric Rheumatology Unit, Children's Hospital, São Paulo, Brazil.

Lupus
|June 4, 2011
PubMed

Insights

Chronic polyarthritis is a rare manifestation in juvenile systemic lupus erythematosus (JSLE). This study highlights the "rhupus" overlap, a distinct JSLE subgroup with poor response to NSAIDs alone.

Area of Science:

  • Pediatric Rheumatology
  • Immunology
  • Systemic Autoimmune Diseases

Background:

  • Juvenile Systemic Lupus Erythematosus (JSLE) is a complex autoimmune condition affecting children.
  • Chronic polyarthritis is an uncommon but significant manifestation in JSLE patients.
  • Understanding JSLE's diverse clinical presentations is crucial for effective management.

Purpose of the Study:

  • To determine the prevalence of chronic polyarthritis in juvenile systemic lupus erythematosus (JSLE).
  • To characterize the clinical features, treatment strategies, and outcomes of JSLE patients with chronic polyarthritis.
  • To investigate the 'rhupus' overlap syndrome in the context of JSLE.

Main Methods:

  • Retrospective analysis of 5419 patients from a Pediatric Rheumatology Unit (1983-2010).
  • Identified 271 patients with JSLE (ACR criteria) and evaluated for chronic polyarthritis.
  • Defined 'rhupus' as overlap between juvenile idiopathic arthritis (ILAR criteria) and JSLE; assessed clinical, laboratory, and radiographic data.

Main Results:

  • Chronic polyarthritis occurred in 2.6% (7/271) of JSLE patients, presenting initially in all cases.
  • Three patients exhibited 'rhupus' with chronic polyarthritis, rheumatoid factor, autoantibodies, and radiographic abnormalities.
  • Treatment involved NSAIDs, prednisone, antimalarials; three 'rhupus' patients required immunosuppressants due to non-responsiveness.

Conclusions:

  • Chronic polyarthritis is a rare but distinct manifestation in pediatric lupus.
  • 'Rhupus' in JSLE may represent a unique clinical subgroup with a poorer prognosis and limited response to NSAIDs.
  • Further research into potential genetic factors for this JSLE subtype is warranted.
Abstract

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