All normal activities on hold.  The worldwide corona virus has reached the U.K.  Normal clinical activity totally shattered.  Our Prime Minister in intenstive care.  Self-isolation.

Our normal lupus clinic has, for now, converted to telephone consultations.  A major problem is the lack of available screening tests.  For example, a week ago, one of my lupus patients rang for advice – she had developed a fever.  As it turned out, it was a lupus flare which had been grumbling for some weeks.  But you can see the difficulty.

Perhaps this is a good time to talk about the topic of fever in connective tissue diseases, including lupus and Hughes Syndrome.

Patient of the Month

“A Friday night case”

Mrs J.J., aged 27, was diagnosed with probable lupus. She had suffered from frequent headaches and rashes as a teenager.

She became pregnant but miscarried after 3 months.  A few days after the miscarriage, she became unwell with fever, chest discomfort and a widespread rash.  She also developed pain and swelling in the calf.

Hospital tests showed a leg clot – a DVT, and she was started on heparin.  However, her temperature continued to be raised.

Blood tests showed a raised ESR (65), normal CRP and (later), positive anti-DNA, anti-Ro and aPL (antiphospholipid/aPL) tests.

What next?

Tests for infection (including gynae infection, UTI and chest infections) proved negative.

Lupus?  Certainly – positive anti-DNA is one of the most specific tests (for lupus) in the whole of medicine.

Hughes Syndrome?  One in 5 lupus patients are positive for aPL (the miscarriage and DVT are almost certainly significant here).

Infection?  Always the worry – including in lupus patients.  Yet an ‘infection’ screen was negative.  Critically, however, her CRP (C-Reactive protein) was normal. 

What is this patient teaching us?

In the late 1970s I published a paper with my colleague, Professor Mark Pepys, who ran the lab next to mine.  Mark had become an international expert on blood proteins, important in inflammation.  One – a mysterious protein known as CRP, would be found to be raised during infection and in inflammation.  However, in our lupus patients the CRP remained low in inflammation, but in infection became raised.

Measurement of CRP has become a standard test in lupus – especially in a ‘Friday night’ case of lupus with fever.  The CRP/ESR ratio has been the ‘go to’ pair of blood tests in this situation.

In our ‘patient of the month’, the diagnosis was lupus, with features of the antiphospholipid/Hughes Syndrome.  Started on a short course of steroids and plaquenil, happily the patient responded immediately. 

Professor Graham R V Hughes MD FRCP

London Lupus Centre

London Bridge Hospital