Education, Awareness, Support

March 2010

by Professor Graham R V Hughes MD FRCP

Rain, rain, snow, cold!  So different from last year.  This cold grey winter has gone on for nearly four months.  Today, the last day of March, we were wrapped up in overcoats, umbrellas and scarves.  I really feel sorry for the tourists, scurrying from shop to shop.  What a difference a year makes!

Earlier on this month I took part in a renal/kidney conference at Hammersmith Hospital.  This was, for me, an excellent one day meeting on kidney lupus, organised by the Hammersmith Hospital nephrologists, Liz Lightstone and Megan Griffiths.  My job was to review the effects of the antiphospholipid syndrome on the kidney (not simply kidney thrombosis, but also kidney artery and vein occlusion).

For me, it was a journey back in time.  Surprisingly, perhaps, I had not been back to Hammersmith for 25 years, since I had left for St Thomas’ Hospital in 1985.  It is an example of how time flies – especially when one is busy.  “The Hammersmith” has changed little – a slightly dingy collection of buildings snuggled up to the high walls of Wormwood Scrubs prison.

My memories of the Hammersmith are still vivid – for it was here that we described the antiphospholipid syndrome.  The ward rounds, the animated discussions of our cases, the presentation of our findings to the ‘grand rounds’ – and, in 1982, the major presentation of the syndrome to the ‘Heberden Round’ – the annual meeting of the British Society of Rheumatology.

In particular, the vivid memories of so many of my research fellows who played such an active part in the description of the syndrome – Dr Helen Englert (Australia), Dr Genevieve Derne (Belgium), Dr Charles Mackworth-Young (London), Dr Bernie Colaco (London), Dr Mee-Ling Boey (Singapore) and later, Dr Aziz Gharavi (Iran), Dr Nigel Harris (Jamaica) and Dr Ron Asherson (Cape Town).

And the memory of the first ‘world’ conference on APS (35 registrants) held at Hammersmith.  And the day on 26th November 1983, when our Lancet research paper on labs methods for detecting antiphospholipid antibodies was published – when we celebrated by going to an Italian café for lunch in nearby Shepherd’s Bush!

Patient of the Month

Mrs IB (61) was referred to me by an old friend Pat England, a semi-retired orthopaedic surgeon.  Her history was one of ‘classical’ Hughes Syndrome, with lifelong migraine, one DVT, balance problems and deteriorating memory.  Interestingly, her daughter also had a history of severe migraine, having had to spend two to three days each month in the school ‘darkness’ sick room. 

Mrs IB’s past history was otherwise ‘uneventful’ apart from two years previously having three fractured metatarsal bones in the foot.

She was tested for antiphospholipid antibodies and found positive.  She was started on low-dose aspirin (with the possibility of a heparin trial if aspirin was less than successful).


What is this patient teaching us?

Look at the last part of the history: three spontaneous fractures of the toe bones (metatarsals).

Some years ago, at St Thomas’ hospital, we noticed a ‘run’ of Hughes Syndrome patients with seemingly spontaneous fractures of the bones in the feet.

This condition – widely known as ‘march fracture’ following its description in some army recruits after long training marches, was always regarded as a simple stress fracture.

Following our observations in St Thomas’, we rapidly collected a series of a dozen or more patients with Hughes Syndrome who had had similar ‘march fractures’.  (Dr Sangle, one of our able research fellows was largely instrumental in following up on these patients).  It seemed more than a coincidence to us, and suggested that the bones, just like other organs, might suffer from impaired circulation in Hughes Syndrome.

Since our publication on this association, a number of similar observations have appeared including ‘spontaneous’ fractures of ribs, vertebrae and other bones.

Whilst this complication of Hughes Syndrome is rare, it does widen the spectrum of the disease to include orthopaedics.

Until now, the impact of the syndrome on the speciality of orthopaedics was largely limited to the (important) matter of pre-operative blood screening for ‘sticky blood’ prior to knee or hip surgery.

Now it seems the ripples are spreading more widely.

 

Professor Graham R V Hughes MD FRCP

Professor Graham R V Hughes MD FRCP

Head of The London Lupus Centre
London Bridge Hospital