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      Treatment refractory arthritis and stroke – A case of infective endocarditis caused by Tropheryma whipplei

      case-report

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          Abstract

          Whipple´s disease is a rare multisystem condition affecting < 1/1.000.000 per year. The condition often presents with polyarthritis, diarrhea, and intestinal malabsorption. Endocarditis is seen in a minority of these patients, and is typically culture negative, as the causative agent Tropheryma whipplei does not grow in ordinary culture media. We present the case of a 78-year-old man with a history of seronegative polyarthritis that was refractory to treatment with several biological agents for a duration of 5 years prior to presentation to the emergency department with stroke. Echocardiography revealed aortic valve endocarditis with a 3.6 cm vegetation and multiple smaller vegetations. The patient underwent surgery with aortic valve replacement followed by prolonged antibiotic treatment. 16 S rDNA PCR analysis of the resected valve revealed T. whipplei as the causative agent. Two years after surgery and treatment with antibiotics, the patient’s previously longstanding arthritis had totally disappeared and all rheumatological treatment had been discontinued.

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          Most cited references12

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          Whipple's disease.

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            Systematic review: Whipple's disease (Tropheryma whipplei infection) and its unmasking by tumour necrosis factor inhibitors.

            The classical form of Whipple's disease (WD), clinically characterised by arthropathy, diarrhoea and weight loss, is rare. Recently, other more frequent forms of Tropheryma whipplei infection have been recognised. The clinical spectrum includes an acute, self-limiting disease in children, localised forms affecting cardiac valves or the central nervous system without intestinal symptoms, and asymptomatic carriage of T. whipplei which is found in around 4% of Europeans. Genomic analysis has shown that T. whipplei represents a host-dependent or opportunistic bacterium. It has been reported that the clinical course of T. whipplei infection may be influenced by medical immunosuppression.
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              Tropheryma whipplei Endocarditis

              Tropheryma whipplei endocarditis differs from classic Whipple disease, which primarily affects the gastrointestinal system. We diagnosed 28 cases of T. whipplei endocarditis in Marseille, France, and compared them with cases reported in the literature. Specimens were analyzed mostly by molecular and histologic techniques. Duke criteria were ineffective for diagnosis before heart valve analysis. The disease occurred in men 40–80 years of age, of whom 21 (75%) had arthralgia (75%); 9 (32%) had valvular disease and 11 (39%) had fever. Clinical manifestations were predominantly cardiologic. Treatment with doxycycline and hydroxychloroquine for at least 12 months was successful. The cases we diagnosed differed from those reported from Germany, in which arthralgias were less common and previous valve lesions more common. A strong geographic specificity for this disease is found mainly in eastern-central France, Switzerland, and Germany. T. whipplei endocarditis is an emerging clinical entity observed in middle-aged and older men with arthralgia.
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                Author and article information

                Contributors
                Journal
                IDCases
                IDCases
                IDCases
                Elsevier
                2214-2509
                18 May 2023
                2023
                18 May 2023
                : 32
                : e01800
                Affiliations
                [a ]Department of Cardiothoracic Surgery, Lund University, Skåne University Hospital, Lund, Sweden
                [b ]Department of Infection Medicine, Kristianstad Hospital, Sweden
                [c ]Department of Pathology, Lund University, Skåne University Hospital, Lund, Sweden
                [d ]Department of Cardiology, Lund University, Skåne University Hospital, Lund, Sweden
                [e ]Department of Infection Medicine, Lund University, Skåne University Hospital, Lund, Sweden
                Author notes
                [* ]Correspondence to: Division of Cardiothoracic Surgery, Skåne University Hospital University, SE-221 85 Lund, Sweden. ziyad.ujaimi@ 123456skane.se
                Article
                S2214-2509(23)00124-5 e01800
                10.1016/j.idcr.2023.e01800
                10225878
                3623879e-e904-436d-8b3a-a7aba072d33c
                © 2023 The Authors. Published by Elsevier Ltd.

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 8 May 2023
                : 16 May 2023
                Categories
                Case Report

                whipple’s disease,culture-negative infective endocarditis,seronegative arthritis,immunomodulatory agents

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