08 July 2022
This month's spotlight on our eLearning platform focuses on pyrexia of unknown origins (PUO). We speak to Dr Jessica Manson from University College London Hospitals, a consultant rheumatologist who has an interest in PUO and haemophagocytic lymphohistiocytosis (HLH).
What’s your clinical role?
I work as a consultant at UCLH and an Associate Professor at UCL. My job is mainly clinical, although I also do quite a lot of research. I provide ward cover and do a mixture of clinics including early inflammatory arthritis, ultrasound and general rheumatology, including HLH.
What’s your approach to reviewing PUO?
Patients with PUO frequently need multi-speciality input, often from infectious diseases, haematology and rheumatology. Sometimes just by us taking a good history from the patient, rheumatologists can identify the cause of the PUO fairly quickly. My approach is to listen to the clinical story and do the tests that are needed. I warn against a full autoimmune screen unless there is a clinically appropriate syndrome. Where there is still diagnostic uncertainty, I almost always do a PET scan.
Any tips for managing these patients?
Taking a comprehensive history and recognising patterns are key, but you must support the clinical impression with the correct investigations. Tissue is the gold standard, so biopsy abnormal tissue when there is any doubt.
When did you first become interested in HLH?
When I was a registrar, I experienced some very unwell patients who were bounced from specialty to specialty and ended up eventually being diagnosed with HLH. Then I went to a BSR conference, where there was a great session on macrophage activation syndrome/ HLH by Dr Rachel Tattersall at Sheffield. I realised that we had overlapping interests and experience, but there were differences in practice, such as access to anakinra for paediatric and adolescent patients but not for adults.
Afterwards I approached Rachel and said I was interested in doing some work on HLH to address the unfairness around access to anakinra and to try to improve outcomes for this group of patients more generally. This led to the development of a cross-specialty group of HLH doctors called HiHASC, which we co-chair.
What’s the future of HLH?
Rheumatologists should be central to managing patients with PUO and with HLH, which is really just the ultimate PUO. I think that managing HLH with a cross-specialty approach and learning from each case will eventually help us reduce the death rate.
Research is needed to really change outcome, but this will take time. We have several drugs available to manage HLH, but none is perfect or without risks. We need to study the patients in large numbers and try to identify patterns that help us decide which drug to use and in which order. We need to recruit patients into biobanks and improve our understanding of the immunology to identify novel therapeutic targets and possible drugs.
Many thanks to Dr Manson for sharing her expertise. Log onto our eLearning platform now where members can access webinars looking at fever in different age groups as well as links to journal articles discussing interesting cases. Hear the ID perspective from Dr Tom Fletcher in this month’s podcast.