La sindrome infiammatoria da immunoricostituzione nel morbo di Whipple

Abstract

Objectives: Whipple’s disease (WD) is a rare chronic multisystemic infection caused by Tropheryma whipplei (TW), a Gram-positive bacterium. Usually WD responds to prolonged antibiotic treatment, but in some patients there is the recurrence of an inflammation after starting the therapy. This inflammation does not respond to antimicrobials but responds to steroids. Since polymerase chain reaction is negative for TW, this reinflammation cannot be a relapse of WD itself. Recently, it has been recognised as a complication of WD and defined immune reconstitution inflammatory syndrome (IRIS). To study the prevalence and the clinical features of IRIS in Italian patients with WD. Methods: In our clinic we have seen twenty-two patients affected by WD between Jan 2000 and Sept 2011. Evidence of IRIS was retrospectively revaluated in the clinical notes. Patients with no evidence of IRIS served as controls. Results: We have diagnosed IRIS in 5/22 patients because of recurrence of arthralgia and/or fever. One patient died. Previous immunosuppressive therapy was found in 5/5 patients with IRIS but only in 7/17 controls (Fisher test, p=0.039). Age at diagnosis and diagnostic delay were higher in patients with IRIS compared to controls. However, statistical significance was not reached. Probably because of the relatively small sample, clinical features did not differ between patients and controls. Conclusions: IRIS is a rather frequent complication of WD that must be promptly recognised because its course can be fatal. The risk of IRIS is greatly increased in patients previously treated with immunosuppressive therapy.