SAT0453 JOINT MANIFESTATIONS OF WHIPPLE’S DISEASE: CLINICAL AND RADIOLOGICAL PRESENTATION OF 19 PATIENTS
Authors: Marie Desmurs, Hélène Petit, Jacques-Eric Gottenberg
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Whipple's disease (WD) is a rare chronic systemic bacterial infection caused by <i>Tropheryma whipplei</i>. It affects middle-aged men with diarrhea, weight loss, fever and joint manifestations. It usually develops as oligoarthritis or polyarthritis of large joints. WD may mimic chronic inflammatory rheumatisms. Some studies and case reports show that immunosuppressive treatment can worsen WD<sup>1</sup>. <h3>Objectives:</h3> Clinical and radiological (radiograph, ultrasound (US), MRI) presentation of joint manifestations of WD. <h3>Methods:</h3> This retrospective monocentric observational study was led in Strasbourg University Hospital, Reference Center for Rare Diseases and included patients with WD diagnosed between 2006 and 2017. Patients were included if they had joint manifestations, positive <i>T.whipplei</i> polymerase chain reaction in stools or/and saliva, positive <i>T.whipplei</i> polymerase chain reaction on duodenal biopsy and/or positive periodic acid-Schiff performed on duodenal biopsy and good evolution with antibiotics. <h3>Results:</h3> Nineteen patients including fifteen men were included. The median age at diagnosis was 57. The median time from first symptoms to diagnosis was 60 months. Before the diagnosis of WD, 13 patients had immunosuppressive therapy. Clinical presentation was: 11 polyarthritis, 3 oligoarthritis, 1 monoarthritis and 4 inflammatory polyarthralgia including 3 with axial pain. Wrists (15/19), ankles (11/19) and knees (10/19) were the most affected. The metacarpophalangeal joints were affected in 8 patients. Eight patients had diarrhoea and weight loss. C-reactive protein median was 45 mg/l. Feet and hands radiographies were performed for seventeen patients. Five patients had carpitis and two patients had tarsitis. Two patients had unilateral sacroiliitis. Hand and wrist US were performed for twelve patients. Ten patients had joint damage. We found 7 radiocarpal and intercarpal synovitis, including 5 with a positive power Doppler (PD) signal, in 5 patients. US showed one distal radio-ulnar synovitis with no PD signal. Four patients had metacarpophalangeal synovitis including 3 with a positive PD signal. Proximal inter-phalangeal synovitis with no PD signal was found for one patient. There was no involvement of distal interphalangeal joint. Four patients had unilateral tenosynovitis including 2 with a positive PD signal. Feet US were done for ten patients. US showed joint damage for six patients. Three patients had metatarsophalangeal synovitis including one with a positive PD signal. Ankle synovitis with no PD signal was found for two patients. US showed one tarsal synovitis with a positive PD signal. Two patients had tenosynovitis including one with a positive PD signal. US showed joint abnormalities in 6 patients with normal X-rays. Hands joints MRI were performed for three patients. Carpitis was found for one patient. One patient had radio-ulnar synovitis with tenosynovitis. One patient had radio-ulnar synovitis with metacarpophalangeal synovitis. Six patients had one or two erosions on feet or hands: 2 on radiographs, 3 on US and 3 on MRI (including one shown by US). <h3>Conclusion:</h3> Clinical and radiological (radiograph and US) joints manifestation in WD affect large joints. However, small joints can also be affected. Axial involvement is rare. WD can affect both joints and tendons. Erosions are rare and never exceed 2 per patient in our series. US can show joint damage whereas radiograph is normal. US should be systematic for patient with WD in order to improve knowledge about this disease and to distinguish it from autoimmune arthritis. <h3>References</h3> [1] - Meunier M, Puechal X, Hoppé E, Soubrier M, Dieudé P, Berthelot JM, et al. Rheumatic and musculoskeletal features of Whipple disease: a report of 29 cases. J Rheumatol. déc 2013;40(12):2061-6. <h3>Disclosure of Interests:</h3> Marie Desmurs: None declared, Hélène Petit: None declared, Jacques-Eric Gottenberg Grant/research support from: Bristol-Myers Squibb, Grant/research support from: Bristol-Myers Squibb, Consultant for: Bristol-Myers Squibb, Lilly, Pfizer, Sanofi-Genzyme, UCB Pharma, Consultant for: Bristol-Myers Squibb, Eli Lilly, UCB, Sanofi-Genzyme, Pfizer