gms | German Medical Science

Background: Autoinflammatory diseases (AID) cause inflammation in multiple organ systems. To quantify organ damage caused by inflammation, the autoinflammatory disease damage index (ADDI) has been developed [1]. In this pilot study, the performance of the ADDI score has been tested in a cohort of patients with AID.

Methods: In this retrospective single centre pilot study, the ADDI score was calculated automatically in moderately to severely affected AID patients at baseline and at last follow-up using ARDIS (arthritis and rheumatology documentation and information system). Patient characteristics, ADDI scores, physician assessment of disease severity and data on disease duration and treatment were recorded.

Results: A total of 22 patients with the diagnosis of CAPS (10), TRAPS (2) HIDS (2) and FMF (8) were included. Organ damage was detected by ADDI score in 12/22 patients (55%). In 5 CAPS patients ADDI score values reflected clinical improvement with IL-1 inhibition. In spite of severe disease manifestation, scores remained low in patients with other AID and in CAPS patients with hearing loss.

Conclusion: Changes in items like hearing loss, amyloidosis, fatigue and percentiles for weight and length are not reflected sufficiently in the ADDI score. Also expression of damage from diseases like FMF, HIDS and TRAPS is not recorded adequately. This pilot study is limited by the small number of patients. Also mostly children have been examined. Presumably in adults with more pronounced organ damage ADDI scores may be more meaningful.

Because the ADDI score only indicates rough changes, more sensitive instruments like percentile curves and audiograms have to be recorded in addition to monitor for slight changes in organ damage of AID patients.