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Longterm outcome of amyloidosis associated with juvenile idiopathic arthritis

Kai Immonen1, Anneli Savolainen, Hannu Kautiainen

  • 1Department of Medicine, North Karelia Central Hospital, Joensuu, Finland. kai.immonen@fimnet.fi

The Journal of Rheumatology
|April 3, 2008
PubMed

Insights

Juvenile idiopathic arthritis (JIA)-associated amyloidosis has a poor prognosis, with significant mortality. However, aggressive treatment can lead to regression of renal disease and enable patients to lead active lives.

Area of Science:

  • Rheumatology
  • Nephrology
  • Pediatrics

Background:

  • Juvenile idiopathic arthritis (JIA) is a chronic inflammatory condition in children.
  • Amyloidosis, a complication of chronic inflammation, can affect multiple organs, including the kidneys.
  • Understanding the long-term outcomes of JIA-associated amyloidosis is crucial for patient management.

Purpose of the Study:

  • To investigate the clinical outcomes and survival rates of patients diagnosed with amyloidosis secondary to JIA.
  • To identify factors influencing mortality and morbidity in this patient cohort.
  • To assess the impact of treatment strategies on the progression of renal disease and overall survival.

Main Methods:

  • A retrospective review of patient records and biopsy files from a tertiary pediatric rheumatology center in Finland (1976-2003).
  • Inclusion criteria: patients under 19 years with biopsy-proven amyloidosis.
  • Data collection included medical history, treatment, survival, cause of death, and renal function assessment.

Main Results:

  • Twenty-four patients with JIA-associated amyloidosis were identified.
  • At diagnosis, 67% had proteinuria, but none had renal insufficiency.
  • The 10-year survival rate was 75%; 42% of patients died, primarily due to JIA-related complications.
  • Aggressive treatment with disease-modifying antirheumatic drugs and/or cytostatics was associated with significantly lower mortality compared to prednisolone monotherapy (p=0.001).
  • Renal function improved or stabilized in many survivors, with proteinuria resolving in some cases.
  • Survivors achieved good educational and personal outcomes, including successful pregnancies.

Conclusions:

  • JIA-associated amyloidosis carries a poor prognosis with substantial mortality.
  • Vigorous treatment regimens, including DMARDs and cytostatics, are associated with improved survival and potential regression of renal disease.
  • Despite the severity, successful management can enable patients to lead active and fulfilling lives.
Abstract

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