Hydroxychloroquine (Plaquenil) is a vital drug in the management of lupus – so much so that many publications recommend lifelong use. Consequently, having to stop taking the drug because of allergic/adverse reaction is a major issue.
In this study from Jerusalem, 13 such patients underwent a gradual desensitisation regime, starting with highly diluted doses of hydroxychloroquine. In 12 of the 13 the patients were subsequently able to continue on standard hydroxychloroquine doses.
In this study 508 patients with APS were studied, 360 with primary APS (PAPS) and 148 with APS associated with SLE (SAPS).
This important study showed a strong association between skin manifestations (notably livedo) and neurological involvement (notably stroke).
This large group of Latin American physicians from centres in South America analysed factors prior to flares of lupus.
A total of 465 cases and controls were studied. Older age at diagnosis and more frequent antimalarial use were protective of flares while azathioprine use, and high disease activity were predictive of flares.
From the very earliest description of the antiphospholipid syndrome it has been recognised that 'non-thrombotic' features as well as thrombotic can occur in Hughes Syndrome.
This study (from Turkey) compared SLE patients with and without antiphospholipid antibodies (aPL). Livedo, neurologic involvement and low platelets were more common in aPL positive SLE patients. Face rash, arthritis and pleurisy were more common in SE/aPL negative patients.
This was the result of a 2014 survey of 2527 member of the national charity LUPUS UK.
Tacrolimus, an immunosuppressive drug has been around a long time, but has not become one of the leading drugs in the lupus world.
This study from China provides a reminder that 'older' drugs may have a significant role.
This study looked at 20 lupus patients with low platelets (often a difficult and stubborn problem). After one month of Tacrolimus treatment, 3 patients achieved a complete response, 14 patients a partial response and 3 did not respond. "After 3 months of Tacrolimus treatment, all patients were significantly improved".
For those wishing to catch up on the important (and ground-breaking) observations linking rheumatology and orthopaedics to the antiphospholipid syndrome, there is no better place to start.
This review by Noureldine and Uthman, from the excellent unit in Beirut, provides a concise overview of the clinical picture – at least as we understand it in 2018.
Clearly avascular necrosis (AVN) is a clearly related problem, but multiple fractures for example are now well recognised.
So, you have Hughes syndrome? Have you ever suffered a bone fracture? A “yes” answer is opening up a whole new avenue of research into fracture risk.
I wish to start by thanking the organizers of this, the 14th International Congress on Antiphospholipid Antibodies, for the honour of giving this opening talk. I will talk briefly about the past and crystal-gaze briefly into the future. But my main focus is on the present – largely through the eyes of a clinician, punctuated from time to time by doctors’ letters – five doctors’ letters, in fact..
Rolando Cimaz and his team have been at the forefront of studies of epilepsy in lupus and antiphospholipid syndrome. In this article, part of the LUPUS ‘Update’ series, they have reviewed he recent published literature.
The main conclusions were that seizures tended to occur early in the course of the disease, reported in between 6% and 12.,5% of lupus patients. As expected there was a strong link with the presence of antiphospholipid antibodies. In particular, anti-B2GP1 antibodies were very strongly associated patients with this antibody being 11 times more likely to have seizures.
This is a very detailed review of the aetiology and treatment of both lupus and antiphospholipid syndrome.
An overall incidence rate of between 3% and 20%, was reported in lupus, especially in the first 5 years of the disease.
While traditional factors of stroke such as smoking diabetes and hypertension contributed to some cases, by far the strongest link was with antiphospholipid antibodies (aPL). The mechanism is considered thrombotic, not haemorrhagic. No surprisingly the best treatment results have been with Warfarin, with an INR of 3.0-4.0.
In the USA, stillbirth happens in about 6.4 per 1000 births.
Recent reports quoted by Ware Branch’s group in Utah (one of the world’s leaders in this field) found that antiphospholipid antibodies were found in 9.6% of stillbirth cases. When other obstetric complications were excluded, about 14% were thought to be secondary to Aps. Within this group, the presence of either a positive lupus anticoagulant, or a ‘triple positive’ (aCL, LA and ant-B2GP1) antibody profile suggested a higher risk.
While the linking of antiphospholipid antibody with stillbirth is another potentially massive advance in obstetrics, the data does require further properly controlled trials “before novel therapies are accepted”.