Clinical benefits of diagnosing incipient AA amyloidosis in pediatric rheumatic diseases as estimated from a retrospective study

Abstract

Objective. The diagnosis of AA amyloidosis could not be made in eight patients with pediatric rheumatic diseases as later verlfied employing the more sensitive combination of Congo red and additional immunocytochemistry (CRIC). The objective of this paper is to estimate the benefit of CRIC by reevaluating the historical charts with respect to the question as to which of the diagnostic and therapeutic measures would have been altered if the correct diagnosis had been known at the time of the primary biopsy

Methods. All subsequent biopsies of eight children with historically missed AA amyloidosis in their primavy biopsies were retrieved, together with the historical data including the Congo red stains of the biopsies. The biopsies were reexamined blindly for the presence of amyloid and the results were compared with the historical data concerning diagnostic and therapeutic measures

Results. Using CRIC, AA amyloidosis could be identified an average of approximately three years earlier as cornpared to the historical data. This gain in time would certainly have altered the diagnostic as well as the therapeutic options, i.e. 10 out of 21 biopsies would have been spared and the earlier diagnosis would have initiated more significant antiinflammatory therapy

Conclusion. Very early detection of amyloid reduces the diagnostic burden and unveils an option for a consequent antiinflammatory therapy very early in the course of AA amyloidosis.

Key Words:

• Am-vloidosis
• immunohistochemistry
• diagnostic errors
• juvenile rhettmatoid arthritis
• familial Mediterranean fever
• health expenditures