Full Papers
The high prevalence of Whipple's disease in patients with inflammatory rheumatic diseases
S. Maimaris1, G. Germanò2, A. Torre3, C. Bianco4, P.I. Bianchi5, C. Montecucco6, A. Schiepatti7, M. Frassi8, G. Fontana9, F. Crisafulli10, L. Dardani11, L. Attard12, P. Cambieri13, G. Arpa14, C. Salvarani15, A. Cavazza16, A. Di Sabatino17, F. Biagi18
- Department of Internal Medicine and Therapeutics, University of Pavia; and Istituti Clinici Scientifici Maugeri IRCCS, Gastroenterology Unit of Pavia Institute, Italy.
- Rheumatology Unit, Dipartimento Medicine Specialistiche, Azienda USL- IRCCS, Arcispedale Santa Maria Nuova, Reggio Emilia, Italy.
- Department of Internal Medicine and Therapeutics, University of Pavia, Italy.
- Department of Internal Medicine and Therapeutics, University of Pavia, Italy.
- First Department of Internal Medicine, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
- Department of Internal Medicine and Therapeutics, University of Pavia; and Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
- Department of Internal Medicine and Therapeutics, University of Pavia; and Istituti Clinici Scientifici Maugeri IRCCS, Gastroenterology Unit of Pavia Institute, Italy.
- Department of Clinical and Experimental Sciences, University of Brescia; and Rheumatology and Clinical Immunology, ASST Spedali Civili, Brescia, Italy.
- Department of Clinical and Experimental Sciences, University of Brescia; and Rheumatology and Clinical Immunology, ASST Spedali Civili, Brescia, Italy.
- Department of Clinical and Experimental Sciences, University of Brescia; and Rheumatology and Clinical Immunology, ASST Spedali Civili, Brescia, Italy.
- Rheumatology Unit, Dipartimento Medicine Specialistiche, Azienda USL- IRCCS, Arcispedale Santa Maria Nuova, Reggio Emilia, Italy.
- Infectious Diseases Unit, Department for Integrated Infectious Risk Management, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Italy.
- Department of Microbiology & Virology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
- Anatomic Pathology Unit, Istituti Clinici Scientifici Maugeri IRCCS, Pavia, Italy.
- Rheumatology Unit, Dipartimento Medicine Specialistiche, Azienda USL- IRCCS, Arcispedale Santa Maria Nuova, Reggio Emilia; and Dipartimento Chirurgico, Medico, Odontoiatrico e di Scienze Morfologiche con Interesse Trapiantologico, Oncologico e di Medicina Rigenerativa - University of Modena and Reggio Emilia, Modena, Italy.
- Pathology Unit, Azienda USL/IRCCS di Reggio Emilia, Italy.
- Department of Internal Medicine and Therapeutics, University of Pavia; and First Department of Internal Medicine, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
- Department of Internal Medicine and Therapeutics, University of Pavia; and Istituti Clinici Scientifici Maugeri IRCCS, Gastroenterology Unit of Pavia Institute, Italy. federico.biagi@unipv.it
CER18654
2026 Vol.44, N°1
PI 0111, PF 0115
Full Papers
PMID: 41196269 [PubMed]
Received: 24/02/2025
Accepted : 02/07/2025
In Press: 03/11/2025
Published: 21/01/2026
Abstract
OBJECTIVES:
Whipple’s disease (WD) is a rare systemic chronic infection with a large diagnostic delay that favours immunomediated complications. Rheumatological symptoms mimicking rheumatological conditions (RC) usually appear first. However, prevalence of WD among patients with RC is still unknown, therefore, we aimed to study the prevalence of WD in a rheumatological setting and identify clinical/laboratory parameters that detect RC patients at high risk of WD.
METHODS:
Data of 23,094 patients attending a rheumatological outpatient clinic between 6/2019 and 8/2023 were retrospectively analysed. Clinical features of WD patients in this cohort were compared with a separate retrospective cohort of 55 WD patients for validation.
RESULTS:
Due to unsatisfactory response to disease-modifying anti-rheumatic drugs (DMARDs) and/or development of gastrointestinal/systemic symptoms, 38 patients were referred for duodenal biopsy and WD was diagnosed in 6/38. Thus, prevalence of WD was 6/23,094 (0.03%, 95% CI 0.01–0.06%). Considering patients with a clinical suspicion of WD, prevalence rose to 6/38 (15.78%, 95% CI 6.02–31.25%), and over 20% in males (5/21, 23.81%, 95% CI 8.22–47.2%). Interestingly, at diagnosis, erythrocyte sedimentation rate and C-reactive protein were elevated in all patients with WD. This finding was confirmed in the separate cohort of 55 patients with WD.
CONCLUSIONS:
WD is rare but in a rheumatological setting its prevalence is much higher than expected. Physicians should be aware of this condition and investigate its presence to reduce diagnostic delay, unnecessary DMARDs and risk of complications. Correct interpretation of clinical picture, including erythrocyte sedimentation rate and C-reactive protein, is the key to reach this diagnosis.
