Abstract

Whipple disease (WD) is a rare infectious systemic disease. Rheumatologists are at the frontline of WD diagnosis due to the early rheumatological manifestations. An early diagnosis is crucial, as usual anti-rheumatic drugs, especially TNF inhibitors, may worsen the disease course. We conducted a retrospective multicentre national study from January 2010 to April 2020 to better characterize the rheumatological features of WD. Classic WD (CWD) was defined by positive periodic acid-Schiff (PAS) staining of a small-bowel biopsy sample, and non-CWD (NCWD) was defined by negative PAS staining of a small-bowel biopsy sample but at least one positive Tropheryma whipplei (TW) polymerase chain reaction (PCR) for a digestive or extradigestive specimen. Sixty-eight patients were enrolled, including 11 CWD patients. Twenty patients (30%) received TNF inhibitors during the WD course, with inefficacy or symptom worsening. More digestive symptoms and systemic biological features were observed in CWD patients than in NCWD patients, but both patient groups had similar outcomes, especially concerning the response to antibiotics and relapse rate. Stool and saliva TW PCR sensitivity were both 100% for CWD and 75% for NCWD and 89% and 60% for small-bowel biopsy sample PCR, respectively. WD encountered in rheumatology units has many presentations, which might result from different pathophysiologies that are dependent on host immunity. Given the heterogeneous presentations and the presence of chronic carriage, multiple TW PCR tests on samples from specific rheumatological sites when possible should be performed, but samples from nonspecific digestive and extradigestive sites also have great value.

Highlights

  • Whipple disease (WD) is a rare infectious systemic disease

  • immune reconstitution inflammatory syndrome (IRIS) and relapse rates did not differ among groups. In this multicentre rheumatological observational cohort study, we identified 68 patients with WD, all but one with rheumatological involvement, of whom 60 had arthritis, 63 had elevated CRP levels, 20 had ineffective biological therapy, and all had a response to antibiotics

  • We propose a new concept of Arthropathic Whipple disease (AWD) and Probably Arthropathic Whipple Disease (PAWD) for patients with compatible symptoms but negative or unrealized periodic acid-Schiff (PAS) staining of SB biopsy samples but with several positive polymerase chain reaction (PCR) for digestive or nonspecific extraintestinal specimens, with a spectacular response to antibiotics and CRP normalization

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Summary

Introduction

Whipple disease (WD) is a rare infectious systemic disease. Rheumatologists are at the frontline of WD diagnosis due to the early rheumatological manifestations. Given the heterogeneous presentations and the presence of chronic carriage, multiple TW PCR tests on samples from specific rheumatological sites when possible should be performed, but samples from nonspecific digestive and extradigestive sites have great value. The improvement of polymerase chain reaction (PCR) techniques has enabled the description of localized forms without any digestive involvement based on PCR analysis of specific nondigestive specimens. Some of these forms of WD have been known for years, such as localized culture-negative endocarditis or localized neurological forms. A better understanding of the rheumatological presentation of the disease is necessary to make earlier diagnoses since immunosuppressive (IS) treatments, such as TNF inhibitors (TNFis), may worsen the disease course and may increase the frequency of immune reconstitution inflammatory syndrome (IRIS)[6,12]

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