ISR Autumn Meeting 2017

Case Presentation Award

Cathy Donaghy

TBA (17A161)

Is It Still Still’s?

Author(s)

Dr C. Donaghy, Dr A. Cairns

Department(s)/Institutions

Belfast HSCT

Introduction

Autoinflammatory disease has a varied and often complex presentation, with a wide range of differential diagnoses to be considered.

Aims/Background

We present a case of a young male who presented systemically very unwell, and responded well to immunosuppressive treatment.

Method

A 26 year old homosexual male was admitted with high grade fever, rigors, sore throat, joint pains and transient pink rash. He had a dental abscess with tooth extraction one week prior to admission. He was commenced on intravenous (IV) antibiotics for a possible streptococcal throat infection. He developed severe chest pain and hypoxia. Imaging revealed pericardial and pleural effusions. Extensive infective screening, including HIV testing on two occasions, was negative. Autoimmune screening was negative. Ferritin was markedly elevated at >4400, and a diagnosis of Adult Onset Still’s Disease (AOSD) was made. He had an immediate response to IV steroids, with improvement in clinical, laboratory and radiological parameters. He was commenced on IV tocilizumab, but had a rapid deterioration, with return of pyrexia, runs of ventricular tachycardia, and rise of ferritin to >60000. He was commenced on anakinra, and had significant clinical improvement, which was sustained. He continued on anakinra with low dose methotrexate for two years. Following a throat infection with associated lymphadenopathy a HIV test was performed, which returned positive. Methotrexate and anakinra were stopped at the time of positive HIV testing. He was commenced on anti-retroviral therapy.

Results

He has remained off immunosuppressive treatment for over two years and has had no recurrence of symptoms suggestive of AOSD. The possibility of HIV seroconversion illness at the time of his initial presentation has since been considered. HIV testing was carried out on two occasions during the acute illness, although there was an interval of only 15 days between testing.

Conclusions

His original diagnosis remains uncertain. His clinical features and laboratory parameters met the diagnostic criteria for AOSD, and he had a dramatic response to treatment with steroids and anakinra. A learning point has been to ensure repeat HIV testing is carried out at an interval of no less than four weeks in the case of a possible seroconversion illness. The patient remains well on anti-retroviral therapy and has since been discharged from further follow up with rheumatology.