Following the sunniest May on record, the good weather came to an end. Back to clouds and some rain. Still on ‘lockdown’…
Many people have asked me (in fact, most patients) about the difference between Hughes Syndrome and lupus. I know I have touched on this quite a lot in past blogs, but I thought I would use this month’s June blog to spell out the similarities and differences.
Patient of the Month
Hughes Syndrome or Lupus?
Mrs J.T. first came to see me as a new patient ten years ago at the age of 28. She had had a ten year history of fatigue, aches and pains and rashes (mainly on the chest and hands). More recently she had suffered from hair loss, chest discomfort and swelling in her groin and armpits.
Her past history included 2 pregnancy miscarriages, migraine headaches and a suspected deep vein thrombosis (DVT).
The family history included a diagnosis of lupus in a cousin, and a lung clot in her mother.
Investigations revealed a low platelet count (90,000) and a trace of protein in the urine, but with normal kidney functions tests.
The immunology tests showed positive anti-DNA anibodies, positive anti-Ro and positive tests for aPL (all 3 tests positive).
What is this patient reaching us?
Fairly easy: rashes, swollen glands, hair loss, aches and pains, also possible percarditis. A family history of lupus and, most of all – a positive anti-DNA antibody test (the strongest evidence for lupus). And, to a lesser extent, a positive anti-Ro test – seen in l in 5 lupus patients and associated with a tendency to skin rashes.
Diagnosis : Lupus
Treatment : Plaquenil 1/day and, to start, a short course of steroids (the Plaquenil can take1 to 3 months to ‘kick in’.
But what about Hughes Syndrome?
The 2 miscarriages, the migraine history, the suspected DVT, strongly supported by the positive aPL tests (all 3) suggests the antiphopholipid syndrome. Aspirin treatment?
In fact the patient had both.
Remember: 1 in 5 lupus patients also have positive aPL and features of Hughes Syndrome. This is such an important point. Yes, lupus and Hughes Syndrome are separate anto-immune diseases but with significant overlap.
Those of us who treat lupus patients recognise that some of the features seen in a number of patients – the recurrent miscarriages, the low platelet count, the migraine and the suspected thrombosis, are more likely linked to a positive aPL. Important in terms of treatment.
Finally, just reminder
Isolated Hughes Syndrome rarely goes on to lupus.
Lupus patients do have a risk (1 in 5) of Hughes Syndrome.
Professor Graham R V Hughes MD FRCP
London Lupus Centre
London Bridge Hospital