P031 Whipple's disease: a multidisciplinary conundrum
Authors: Baldeweg Friederike , Nuttall Anna , Arul Dhilanthy , Childerhouse Anna 
Published: April 2021

Background/Aims 

A 45-year-old male patient presented in 2015 with a six-month history of relapsing and remitting polyarthralgia. Hand X-rays appeared normal. Serology showed mildly elevated inflammatory markers. Autoimmune profile including anti-CCP antibody, rheumatoid factor and ANA was negative. His initial diagnosis was palindromic rheumatism. He was under watchful waiting in rheumatology clinic having declined a trial of hydroxychloroquine, when in 2018 he developed severe epigastric pain. Over the subsequent 18 months he was noted to have dramatic weight loss, fatigue and drenching night sweats.

Methods 

Investigations showed microcytic anaemia with elevated inflammatory markers (Hb 98 g/L, CRP 161 mg/L, ESR 68 mm/hr). Serum ACE, bone profile, thyroid function and urate levels were normal. Chest X-ray was unremarkable. HIV and hepatitis screening was negative. Endoscopy with jejunal biopsy was performed, with mild gastritis only on histopathology and normal D2 biopsies. He was found to be H pylori positive, and notably felt his B-symptoms much improved with triple antibiotic and PPI eradication therapy. CT abdomen demonstrated widespread mesenteric lymphadenopathy. Para-aortic lymph node biopsy showed non-necrotising granulomata suggestive of either sarcoidosis or an infective etiology such as tuberculosis (TB). Given the clinical picture, the patient was commenced on high dose oral prednisolone and methotrexate for suspected sarcoidosis.

Results 

The patient made some clinical improvement, particularly with regards to arthralgia, however his B-symptoms returned with any reduction in steroid dose. Serology showed worsening anaemia with iron and folate deficiency, and increasing inflammatory markers. We therefore decided to perform a PET CT and refer to Haematology for consideration of a lymphoproliferative disease. PET CT demonstrated lymphadenopathy without avid uptake. A second lymph node biopsy was performed which showed florid histiocytic infiltration within which there were numerous PAS positive particles consistent with Whipple's disease. This was confirmed as tropheryma whipplei on PCR. Whipple's disease is a rare systemic infectious disease causing arthralgia, diarrhoea, abdominal pain and weight loss. Treatment consists of antibiotic therapy. On further questioning, the patient had grown up on a farm. There is a known association with Whipple's disease in the agricultural community as it is a soil-borne organism.

Conclusion 

Our patient has made an excellent recovery. He remains under the care of our rheumatology team and the London School of Tropical Medicine and Hygiene. Treatment plan is 12 months of combined therapy with doxycycline and hydroxychloroquine. He underwent a lumbar puncture to rule out meningeal Whipple disease. We have also commenced sulfasalazine for persistent arthritis, which we feel could be a reactive phenomenon. Our key learning points from this case were to use a stepwise approach to diagnosis, involve relevant specialty teams and that it in complex cases it is useful to go back to the history.

Disclosure 

F. Baldeweg: None. A. Nuttall: None. D. Arul: None. A. Childerhouse: None.