A cumulative analogue joint involvement scale (CAJIS) for fibrodysplasia ossificans progressiva (FOP)

Frederick S. Kaplan, Mona Al Mukaddam, Robert J. Pignolo

Research output: Contribution to journalArticlepeer-review

15 Scopus citations

Abstract

Background Fibrodysplasia ossificans progressiva (FOP) is a catastrophic genetic disorder of progressive heterotopic ossification (HO). Assessment of functional mobility in FOP will be essential to support clinical trials of investigational agents. Results Of necessity, we developed a simple, rapidly-administered, cumulative analogue joint involvement scale (CAJIS) for FOP based on assessments in 144 individuals worldwide with classic FOP. Conclusions CAJIS scores correlated with patient age, activities of daily living, and ambulatory function with excellent inter-rater variability. We show here that the CAJIS score provides an accurate and reproducible snapshot of total body and regional mobility burden in FOP.

Original languageEnglish (US)
Pages (from-to)123-128
Number of pages6
JournalBone
Volume101
DOIs
StatePublished - Aug 1 2017

Keywords

  • ACVR1
  • Activities of daily living
  • Ambulation
  • Bone morphogenetic protein signaling
  • Fibrodysplasia ossificans progressiva
  • Heterotopic ossification

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Physiology
  • Histology

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