Isolated joint involvement in Whipple's disease: a cohort study

Abstract

Background

To analyse the joint manifestations of Whipple's disease and compare clinical isolated joint involvement (CIJI) with systemic joint involvement (SJI).

Methods

A cohort study included patients diagnosed with Whipple's disease and joint involvement at two expert centres. Patients were divided according to their clinical presentation at diagnosis: those with CIJI and those with SJI.

Results

Of the 60 patients, 17 (28 %) exhibited CIJI. Both groups were predominantly middle-aged men (75 %) with a median time to diagnosis of six (2–11) years. Joint involvement was similar, with initial episodic migratory arthritis (91 %) predominantly affecting large joints and lasting 2–7 days. Saliva and stool PCR tests were positive in 59 % and 75 % of CIJI patients, respectively, compared to 91 % and 88 % in the SJI group (P = 0 02 and P = 0 24, respectively). In CIJI patients, duodenal PCR was negative in 65 % of cases and PAS staining consistently negative. Synovial fluid PCR was positive in 100 % of CIJI and 89 % of SJI patients. Treatment with doxycycline and hydroxychloroquine, initiated in 52 (88 %) patients, resulted in resolution of joint symptoms within ten (7–15) days in all but one patient, who had destructive arthritis.

Conclusion

Whipple's disease can present with isolated articular forms. Joint involvement is usually inaugural and stereotypical, with little difference between the CIJI and SJI groups. The diagnosis should be considered in cases with the typical pattern of recurrent intermittent large joint arthritis with elevated acute phase reactants, particularly in middle-aged men. Synovial fluid PCR is a valuable diagnostic tool. Doxycycline and hydroxychloroquine resulted in dramatic improvement.