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

Kaplan, Frederick S; Al Mukaddam, Mona; Pignolo, Robert J · Bone · 2017

cross_sectional · Level IV

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Abstract

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. 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. 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.

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