We report the case of a 38-year-old man who presented with severe
diarrhea,
weight loss of 10 kg, ankles
paresthesia and severe motor weakness in the left fibular nerve territory after introduction of
azathioprine and
corticosteroid for
proteinuria. Coloscopy and gastroscopy revealed a typical aspect of
Whipple disease (WD), associated with both positive PAS staining and specific immunohistochemistry. T. whipplei PCR results were positive in blood, faeces, saliva and duodenal biopsy specimens. Diagnosis of WD with systemic manifestations was retained and
doxycycline plus
hydroxychloroquine therapy were started. This treatment improved
joint pain, and skin and intestinal symptoms. One month later, our patient presented with
fever and an important inflammatory syndrome (CRP 150 mg/dL and 16.8 10(9)/L leukocytes), while no
infection was found despite a thorough review. We concluded it was an
immune reconstitution inflammatory syndrome (IRIS). Manifestations persisted despite increasing
corticosteroids and
thalidomide (200 mg/day) was introduced with good efficacy on these symptoms. WD may be revealed by non-specific symptoms such as
weight loss or
arthralgia, but also by many other misleading signs. Our observation illustrates the highly polymorphic clinical presentation of WD, and the diagnostic difficulties that may arise. This is also a new report of
thalidomide effectiveness in IRIS in WD.