Differential diagnostic value of rheumatic symptoms in patients with Whipple’s disease

Abstract

Most patients with Whipple’s disease have rheumatic symptoms. The aim of our prospective, questionnaire-based, non-interventional clinical study was to assess whether these symptoms are useful in guiding the differential diagnosis to the rheumatic disorders. Forty patients with Whipple’s disease, followed by 20 patients for validation and 30 patients with rheumatoid-, 21 with psoriatic-, 15 with palindromic- and 25 with axial spondyloarthritis were recruited for the present investigation. Patients with Whipple’s disease and patients with rheumatic disorders were asked to record rheumatic symptoms on pseudonymized questionnaires. The data obtained were subjected to multiple logistic regression analysis. Episodic pain with rapid onset, springing from joint to joint was most common in patients with palindromic arthritis and second most common and somewhat less conspicuous in Whipple’s disease. Continuous pain in the same joints predominated in patients with rheumatoid-, psoriatic-, and axial spondyloarthritis. Multiple logistic equations resulted in a predicted probability for the diagnosis of Whipple’s disease of 43.4 ± 0.19% (M ± SD) versus a significantly lower probability of 23.8 ± 0.19% (M ± SD) in the aggregate of patients with rheumatic disorders. Mean area under the curve (AUC) ± SD was 0.781 ± 0.044, 95% CI 0.695–0.867, asymptotic significance p < 0.001. The logistic equations predicted probability for the diagnosis of Whipple’s disease in the initial series of 40 patients of 43.4 ± 0.19% was not significantly different in the subsequent 20 patients of 38.2 ± 0.28% (M ± SD) (p = 0.376). The data may be useful in a predictive algorithm for diagnosing Whipple’s disease. The project is registered as clinical study DRK S0001566.

Whipple’s disease unmasked by TNF inhibitor therapy for treatment of seronegative rheumatoid arthritis

“…Patients with multisystem problems and patients who worsen on immunosuppressive therapy should be evaluated for Whipple’s disease. Whipple’s disease should be excluded in middle aged patients particularly men with protracted seronegative rheumatoid arthritis unresponsive to therapy with episodic arthritis sparing the toes and fingers 15. Patients have a dismal outlook if Whipple’s disease is undiagnosed and we recommend screening for Whipple’s disease on a case-by-case basis before instituting TNFI and/or other immunosuppressive therapies.…”

Morbus Whipple

“…Die Arthritis betrifft große Gelenke häufiger als kleine Gelenke und verläuft üblicherweise nicht deformierend. Das unregelmäßig wiederkehrende Beschwerdebild kann zur Fehldiagnose eines palindromen Rheumatismus führen, in Fällen mit begleitendem Fieber wird gelegentlich ein adulter Morbus Still vermutet [ 2 , 3 ].…”

“…Zwischen der Erstmanifestation der Erkrankung und der Diagnose vergehen in vielen Fällen Jahre bis Jahrzehnte, und so präsentieren sich die Patienten im rheumatologischen Kontext häufig mit chronischer Oligo- oder Polyarthritis mit hartnäckig-therapierefraktärem Krankheitsverlauf [ 3 , 6 ]. Steroidtherapien mildern die Arthritis oft für kurze Zeit und vermitteln den Eindruck einer Behandelbarkeit der Erkrankung durch Immunsuppressiva.…”

Maladie de Whipple : généralités et manifestations articulaires

“…Les principaux diagnostics différentiels devant les manifestations articulaires sont la polyarthrite rhumatoïde, le rhumatisme psoriasique et les spondylarthropathies [19,20].…”