Clinical and laboratory predictors and prevalence of immune reconstitution inflammatory syndrome in patients with Whipple's disease

Abstract

AimsWhipple's disease (WD) is a rare and potentially fatal infectious disease caused by Tropheryma whipplei. It is characterized by a long prodromal phase that mimics a rheumatological disease, often leading to treatment with immunosuppressants. Immune reconstitution inflammatory syndrome (IRIS) is currently the most important complication of WD, requiring prompt recognition and treatment as it can be fatal. However, data on the epidemiology of IRIS are scarce. We aimed to identify clinical and laboratory predictors of IRIS at time of WD diagnosis and to evaluate whether prevalence of IRIS has changed over time.Methods45 patients with WD (10 F, 52±11 years) were followed‐up between January‐2000 and December‐2021. Clinical and laboratory data at diagnosis of WD were retrospectively collected and compared among patients who developed IRIS and those who did not.ResultsErythrocyte sedimentation rate (ESR, 33±12 vs. 67±26 mm/h, p<0.01), platelet count (PLT, 234,000±80,000 vs. 363,000±119,000/μL, p<0.01), and body mass index (22±2 vs. 20±3, p=0.04) differed significantly between patients who subsequently developed IRIS and those who did not. ROC analysis identified ESR≤46mm/h (AUC 0.88, 95%CI 0.72‐1.00) and PLTs≤327,000/μL (AUC 0.85, 95%CI 0.70‐1.00) as the best cut‐off values to discriminate WD patients at highest risk of developing IRIS. Prevalence of IRIS remained stable (22.2%) over time.ConclusionsLow ESR and PLT count at diagnosis help identify WD patients at high risk of developing IRIS. Instead, a greater inflammatory response suggests a lower risk of IRIS. Prevalence of IRIS did not change over two decades.This article is protected by copyright. All rights reserved.