PMID- 24289960 OWN - NLM STAT- MEDLINE DCOM- 20141020 LR - 20200205 IS - 1778-7254 (Electronic) IS - 1297-319X (Linking) VI - 81 IP - 3 DP - 2014 May TI - A severe Whipple disease with an immune reconstitution inflammatory syndrome: an additional case of thalidomide efficiency. PG - 260-2 LID - S1297-319X(13)00247-9 [pii] LID - 10.1016/j.jbspin.2013.10.007 [doi] AB - 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. CI - Copyright (c) 2013 Societe francaise de rhumatologie. Published by Elsevier SAS. All rights reserved. FAU - Le Blay, Pierre AU - Le Blay P AD - Service d'immunologie-clinique et therapeutique des maladies osteo-articulaires, CHU Lapeyronie, 295, avenue du Doyen-G.-Giraud, 34000 Montpellier, France. FAU - Rakotonirainy, Henintsoa AU - Rakotonirainy H AD - Service d'immunologie-clinique et therapeutique des maladies osteo-articulaires, CHU Lapeyronie, 295, avenue du Doyen-G.-Giraud, 34000 Montpellier, France. FAU - Lagier, Jean-Christophe AU - Lagier JC AD - Aix Marseille universite, URMITE, UM63, CNRS 7278, IRD 198, Inserm 1095, 13005 Marseille, France. FAU - Raoult, Didier AU - Raoult D AD - Aix Marseille universite, URMITE, UM63, CNRS 7278, IRD 198, Inserm 1095, 13005 Marseille, France. FAU - Puechal, Xavier AU - Puechal X AD - National Referral Center for rare systemic auto-immune diseases, hopital Cochin, AP-HP, universite Paris Descartes, Paris, France. FAU - Pers, Yves-Marie AU - Pers YM AD - Service d'immunologie-clinique et therapeutique des maladies osteo-articulaires, CHU Lapeyronie, 295, avenue du Doyen-G.-Giraud, 34000 Montpellier, France. LA - eng PT - Case Reports PT - Journal Article DEP - 20131128 PL - France TA - Joint Bone Spine JT - Joint bone spine JID - 100938016 RN - 0 (Immunosuppressive Agents) RN - 4Z8R6ORS6L (Thalidomide) SB - IM MH - Adult MH - Humans MH - Immune Reconstitution Inflammatory Syndrome/*drug therapy/immunology MH - Immunosuppressive Agents/*therapeutic use MH - Male MH - Thalidomide/*therapeutic use MH - Whipple Disease/*complications/immunology OTO - NOTNLM OT - Immune reconstitution inflammatory syndrome OT - Motor weakness OT - Proteinuria OT - Thalidomide OT - Whipple disease EDAT- 2013/12/03 06:00 MHDA- 2014/10/21 06:00 CRDT- 2013/12/03 06:00 PHST- 2013/07/29 00:00 [received] PHST- 2013/10/07 00:00 [accepted] PHST- 2013/12/03 06:00 [entrez] PHST- 2013/12/03 06:00 [pubmed] PHST- 2014/10/21 06:00 [medline] AID - S1297-319X(13)00247-9 [pii] AID - 10.1016/j.jbspin.2013.10.007 [doi] PST - ppublish SO - Joint Bone Spine. 2014 May;81(3):260-2. doi: 10.1016/j.jbspin.2013.10.007. Epub 2013 Nov 28.