Previous immunosuppressive therapy is a risk factor for immune reconstitution inflammatory syndrome in Whipple's disease.
BACKGROUND
Whipple's disease is a rare chronic infection caused by Tropheryma whipplei. Although most patients respond to antibiotics, in some of them the start of the treatment is followed by recurrence of inflammation. Since polymerase chain reaction is negative for Tropheryma whipplei, this reinflammation cannot be a relapse of Whipple's disease itself. Very recently, it has been recognised as a complication of Whipple's disease and defined immune reconstitution inflammatory syndrome (IRIS). Our aim is to study the prevalence and the clinical features of IRIS in Italian patients with Whipple's disease.


METHODS
Evidence of IRIS was retrospectively revaluated in the clinical notes of 22 patients with Whipple's disease. Patients with no evidence of IRIS served as controls for the clinical findings.


RESULTS
Recurrence of arthralgia and/or fever allowed a diagnosis of IRIS in 5/22 patients. One patient died. Previous immunosuppressive therapy was found in all 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.


CONCLUSIONS
IRIS is a frequent complication of Whipple's disease and it can be fatal. The risk of IRIS is greatly increased in patients previously treated with immunosuppressive therapy.
Coeliac Centre/First Dept of Internal Medicine, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Italy. f.biagi@smatteo.pv.it
Federico
F
Biagi
Lucia
L
Trotta
Michele
M
Di Stefano
Davide
D
Balduzzi
Alessandra
A
Marchese
Claudia
C
Vattiato
Paola I
PI
Bianchi
Florence
F
Fenollar
Gino R
GR
Corazza
D005260:Female; D006801:Humans; D054019:Immune Reconstitution Inflammatory Syndrome / Q000209:etiology*; D007166:Immunosuppressive Agents / Q000009:adverse effects* / Q000627:therapeutic use; D008297:Male; D016133:Polymerase Chain Reaction; D012189:Retrospective Studies; D012307:Risk Factors; D054851:Tropheryma / Q000302:isolation & purification; D008061:Whipple Disease / Q000150:complications* / Q000188:drug therapy
D007166:Immunosuppressive Agents