Fixed digital contractures revealing light-chain amyloidosis

Joint Bone Spine. 2009 Jul;76(4):404-8. doi: 10.1016/j.jbspin.2008.11.006. Epub 2009 Mar 17.

Abstract

Among the many clinical manifestations of light-chain (AL) amyloidosis, musculoskeletal involvement is rarely reported. We describe the case of a 72-year-old woman who was referred to our rheumatology department for fixed flexion contractures of the fingers that developed concomitantly with a decline in general health. Macroglossia and recent-onset dyspnea were noted. Investigations, which included a tongue biopsy, established the diagnosis of kappa light-chain amyloidosis with soft tissue, bone, and cardiac deposits. Melphalan and dexamethasone therapy was successful in stabilizing the clinical and laboratory abnormalities within 6 months. This case is remarkable in that the musculoskeletal manifestations were at the forefront of the clinical picture and led to the diagnosis.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Amyloidosis / diagnosis*
  • Amyloidosis / drug therapy
  • Amyloidosis / immunology*
  • Contracture / diagnosis
  • Contracture / etiology*
  • Dexamethasone / therapeutic use
  • Female
  • Finger Joint*
  • Humans
  • Immunoglobulin kappa-Chains / metabolism*
  • Melphalan / therapeutic use
  • Treatment Outcome

Substances

  • Immunoglobulin kappa-Chains
  • Dexamethasone
  • Melphalan