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Musculoskeletal manifestations in cystic fibrosis

Estelle Botton1, Alain Saraux, Hermine Laselve

  • 1Service de rhumatologie, Hôpital de la cavale blanche, CHU Brest, 29609 Brest cedex, France.

Joint Bone Spine
|October 18, 2003
PubMed

Insights

Adults with cystic fibrosis (CF) frequently experience bone and joint issues, including low bone density and arthritis. Early recognition and management of these musculoskeletal complications are crucial for improving patient outcomes.

Area of Science:

  • Rheumatology
  • Pulmonology
  • Endocrinology

Background:

  • Bone and joint problems are common in pediatric cystic fibrosis (CF) but understudied in adults.
  • Adult CF patients exhibit low bone mineral density despite high calcium intake, with variable vitamin D levels.
  • Short stature, low BMI, central hypogonadism, fractures, and kyphosis are prevalent in adults with CF.

Purpose of the Study:

  • To review the spectrum of bone and joint manifestations in adult cystic fibrosis patients.
  • To highlight the prevalence and characteristics of CF-associated arthropathies and other musculoskeletal conditions.
  • To discuss relevant autoantibodies and immune markers in adult CF patients.

Main Methods:

  • Literature review of studies focusing on bone and joint manifestations in adult cystic fibrosis.
  • Analysis of reported cases and prevalence data for CF arthropathy and related conditions.
  • Examination of serological findings, including autoantibodies and immune complexes.

Main Results:

  • CF arthropathy affects 2-8.5% of patients, presenting with arthritis and potential skin eruptions; NSAIDs are effective.
  • Hypertrophic osteoarthropathy occurs in 2-7% of patients, associated with respiratory failure.
  • Elevated rheumatoid factor titers, circulating immune complexes, and specific antineutrophil cytoplasmic antibodies (BPI/AZ) are noted in some adult CF patients.

Conclusions:

  • Adults with CF face significant bone and joint complications, including low bone density, fractures, and various forms of arthritis.
  • Musculoskeletal manifestations in adult CF require increased clinical attention for timely diagnosis and management.
  • Further research into the pathogenesis and treatment of these conditions in adult CF is warranted.

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