Long-term outcome of eosinophilic fasciitis: A cross-sectional evaluation of 35 patients.

BACKGROUND: Eosinophilic fasciitis (EF) is a connective tissue disease with an unknown long-term course.

OBJECTIVE: To evaluate presence and determinants of residual disease damage in patients with EF after long-term follow-up.

METHODS: Patients with biopsy-proven EF were included for this cross-sectional study. Outcome measures included the Physician's Global Assessment of Disease Activity, Physician's Global Assessment of Damage (PhysGA-D), skin pliability scores, passive range of motion, and health-related quality of Life (HRQoL) questionnaires.

RESULTS: In total, 35 patients (24 of whom were female [68.6%]) with a median age of 60 years participated. All patients had detectable residual damage. Impairment of HRQoL, assessed by the Dermatology Quality of Life Index and the 36-Item Short-Form Survey, correlated to the extent of residual damage. The PhysGA-D score at participation correlated to signs of severe disease at presentation, such as increased C-reactive protein level (Spearman's rho [rs ] = 0.486, P = .006), involvement of the neck (rs  = 0.528, P = .001) and trunk (rs  = 0.483, P = .003), prolonged time to disease remission (rs  = 0.575, P = .003), and presence of concomitant morphea (rs  = 0.349, P = .040). Lastly, maximum methotrexate dose correlated negatively to PhysGA-D score at study participation (rs  = -0.393, P = .022).

LIMITATIONS: Sample size.

CONCLUSION: All patients with EF had detectable residual damage. Impairment of HRQoL correlated to the extent of residual damage. Advanced age and signs of severe disease at presentation were associated with the severity of residual damage.