title: A severe Whipple disease with an immune reconstitution inflammatory syndrome: an additional case of thalidomide efficiency.
abstract: 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.
authors.affiliation: Service d'immunologie-clinique et thérapeutique des maladies osteo-articulaires, CHU Lapeyronie, 295, avenue du Doyen-G.-Giraud, 34000 Montpellier, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Le Blay
authors.affiliation: Service d'immunologie-clinique et thérapeutique des maladies osteo-articulaires, CHU Lapeyronie, 295, avenue du Doyen-G.-Giraud, 34000 Montpellier, France.
authors.forename: Henintsoa
authors.initials: H
authors.lastname: Rakotonirainy
authors.affiliation: Aix Marseille université, URMITE, UM63, CNRS 7278, IRD 198, Inserm 1095, 13005 Marseille, France.
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Lagier
authors.affiliation: Aix Marseille université, URMITE, UM63, CNRS 7278, IRD 198, Inserm 1095, 13005 Marseille, France.
authors.forename: Didier
authors.initials: D
authors.lastname: Raoult
authors.affiliation: National Referral Center for rare systemic auto-immune diseases, hôpital Cochin, AP-HP, université Paris Descartes, Paris, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Puechal
authors.affiliation: Service d'immunologie-clinique et thérapeutique des maladies osteo-articulaires, CHU Lapeyronie, 295, avenue du Doyen-G.-Giraud, 34000 Montpellier, France.
authors.forename: Yves-Marie
authors.initials: YM
authors.lastname: Pers
mesh_terms: D000328:Adult; D006801:Humans; D054019:Immune Reconstitution Inflammatory Syndrome / Q000188:drug therapy* / Q000276:immunology; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D013792:Thalidomide / Q000627:therapeutic use*; D008061:Whipple Disease / Q000150:complications* / Q000276:immunology
chemical_list: D007166:Immunosuppressive Agents; D013792:Thalidomide
keywords: Immune reconstitution inflammatory syndrome; Motor weakness; Proteinuria; Thalidomide; Whipple disease