Education, Awareness, Support

Obstetric outcome in patients with primary thrombotic and obstetric APS and its relation to the antiphospholipid antibody profile

This study from Sweden compared their own data with previous publications on pregnancy risk in Hughes Syndrome (APS) patients

Although the numbers were small (38 APS patients), the study confirmed two observations.  Firstly that APS patients suffered more pre-eclampsia,and gave birth to more low birth weight, small infants than controls.  Secondly, "triple positivity" (aCL, LA and anti-Beta 2 GP1) had a similar higher incidence of pre-eclampsia and small infants - even under anti-thrombotic treatment. 

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Transcranial Doppler findings in APS

LUPUS (2019) 28, (4) 483-491

Transcranial Doppler is a method for assessment of cerebral blood flow velocities as well as the detection of micro-embolic signals (`dots’) and right to left circulation shunts. Patients with APS had a higher frequency of shunts than healthy controls.

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Peripheral nervous system involvement in SLE

LUPUS (2019) 28, (4) 465-474

This in-depth study looked at nerve function in SLE.  It has always been my impression that `peripheral’ nerve impairment is relatively uncommon in SLE, but much more prevalent in antiphospholipid syndrome “sticky blood”.

This study seems to support this view.  For example, in a chart of 46 patients `risk factors’, the presence of antiphospholipid syndrome was twice as common in those patients with peripheral nerve involvement (17.3% versus 9.9%).

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Sero-negative antiphospholipid syndrome: Update

LUPUS (2019) 28, (3) 273-274

In 2003, on the 20thanniversary of the antiphospholipid syndrome (APS), we wrote…” as awareness increases, and the number of patients with APS grows, it comes as no surprise that ‘seronegative APS’ provides the focus of day to day clinical discussion- the patient with migraine, stroke, several previous miscarriages, thrombocytopenia, and livedo reticularis, whose antiphospholipid (aPL) tests are doggedly negative”.

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Levels of anti-Mullerian hormone in premenopausal women with the antiphospholipid syndrome and its associations with the risk of clinical complications

LUPUS (2019) 28, (3) 427-431

This rather daunting title carried an important message.

Anti-Mullererian hormone levels give a guide to ovarian function in women.  Low levels are associated with lowered fertility (`ovarian reserve’).

In this study ovarian reserve in women with antiphospholipiid syndrome was not reduced.  Good news.

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Factors associated with development and mortality of pulmonary hypertension in SLE patients

LUPUS (2018) 27, 1769-1772

This large study from Korea (1110 patients) found a prevalence of 4.62% with pulmonary hypertension. As might be expected, there were significant factors – anti-RNP antibody and interstitial lung disease. 

One of the known causes of this high pressure in the pulmonary artery is blood clots. It was interesting to note in this study that there was no increased tendency in those lupus patients with positive anticardiolipin antibodies and positive lupus anticoagulant tests.

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Thrombopoietin mimetics for systemic lupus erythematosus with antiphospholipid antibodies should be discussed separately

LUPUS (2018) 27, 1876-1877

Some cases of ‘low platelets’ (thrombocytopenia) can be very resistant to treatment (e.g. With steroids or intravenous immunoglobulin). 

Amongst the new drugs developed during the past decade, 2 in particular have raised hopes of a new treatment for resistant thrombocytopenia, the so called ‘TPO mimetics’ (thrombopoietin mimetics) (see Lupus 2015; 24: 746-750).

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Cardiovascular reflex tests in SLE.

LUPUS (2018) 27, 1759-176

A review of 13 papers on cardiovascular reflex tests and autonomic nervous system tests in SLE. 

The “autonomic” nervous system – the network of nerves supplying “internal” organs such as the heart, the lung and the gut, can be assessed using a fairly standard battery of tests, such as blood pressure and heart rate changes on lying and standing. 

The conclusion of this important study was that abnormal results in cardiac autonomic tests were common in SLE. 

Unfortunately, there was no information on the subset of lupus patients with antiphospholipid antibodies, highlighted recently by Dr Jill Schofield and others.

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Characteristics of 1555 childhood-onset lupus in three groups based on distinct time intervals to disease diagnosis: a Brazilian multicentre study.

LUPUS (2018) 27, 1712-1716

As the years go by the number of lupus patients reported from clinics around the world grows and grows. This collaborative study from centres around Brazil, focused on childhood lupus. 

The study picked up a number of trends – for example, in a significant number of children in whom the diagnosis was significantly delayed. This Brazilian stud will provide a useful template for other paediatric lupus reports.

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Bleeding complications and anti-thrombotic treatment in 264 pregnancies in APS.

LUPUS (2018) 27, 1679-168

This collaboration stud from colleagues in France and the USA concluded that current anticoagulant therapy in APS pregnancies (mainly with aspirin plus or minus heparin) is safe.

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SLE mortality rates.

LUPUS (2018) 27, 1577-158

This is a major study of the causes off death of young women in the USA between 2000-2015. 

The good news is that SLE mortality rate has decreased 2.7% every year since 1999. 

The bad new is that mortality rates from SLE remain high compared to those in the general population. A plea is made for more research on similar population-based collections.

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Neurologic manifestations of Antiphospholipid Syndrome

LUPUS (2018) 27, 1404-1414

Our original papers describing the Antiphospholipid Syndrome in 1983 used the title “Thrombosis, abortion, cerebral disease and the lupus anticoagulant”.

In many ways the thrombosis and abortion aspects of Hughes Syndrome have received the lion’s share of recognition.  Yet the ‘neurological disease’ aspect is equally important.

This detailed review by Ricarte and colleagues from Sao Pauli, Brazil, provides a clear and comprehensive review of the topic, from aetiology to treatment. A valuable paper.

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Hydroxychloroquine: An update.

LUPUS (2018) 27, 1402-1404

This short leader follows a detailed review on Plaquenil and the retina.

Plaquenil is a marvellous medicine used worldwide in lupus, Sjogren’s and (increasingly) in Hughes Syndrome. 

Now that the drug is frequently used long term (e.g., over 20 years), a recent review from the ophthalmology world advises more frequent eye examinations. And, for those of us using the drug, a dose of 1/day (200 mg daily) seems more appropriate than higher doses

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Long-term survival of kidney grafts in Lupus Nephritis: a Mexican cohort

LUPUS (2018) 27, 1303-13

This is an important paper, looking at graft survival periods dating back to 1979 (Mexico).

The good news is that Lupus Nephritis graft recipients have satisfactory outcomes, and similar to those of patients with other causes.

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The clinical manifestations and treatment response in South Africans with Lupus Nephritis

LUPUS (2018) 27, 1207-1217

Traditionally, it has been reported that Lupus is rare in sub-Saharan Africa.  But studies from a number of African centres are now being published.

This report, from Durban, included 105 patients (49.1 Indians and 45.3% African blacks).  The report concluded “In conclusion, our multi-ethnic Lupus Nephritis cohort shows a good response to treatment”.

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The ratio of Erythrocyte sedimentation rate for C-reactive protein is useful in distinguishing infection from flare in Systemic Lupus Erythematosus patients presenting with fever

LUPUS (2018) 27, 1123-1129

On what I call a ‘Friday night’ case of a sick Lupus patient admitted to my hospital with fever, one of the first tests I look for is the CRP level.  A high CRP level points strongly towards infection.  An easy lesson I used to teach my students was: High ESR/Low CRP – lupus, high ESR/high CRP – infection.  Of course, there are exceptions, but the rule has proved useful over the years.

This excellent article from Michigan retrospectively looks at SLE patients with a temperature of over 100.3 F confirmed the idea of an ESR/CRP ratio as a diagnostic aid, and, by analogy, the importance of CRP – this “mysterious” protein.

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Incremental direct medical costs of Systemic Lupus Erythematosus patients in the years preceding diagnosis: A general population based-study

LUPUS (2018) 27, 1247-1248

This interesting study from Vancouver studied 3632 Lupus patients (and 18060 controls) estimating health care costs over a 5-year period.  Not unexpectedly, even years before diagnosis, SLE patients incurred significantly elevated medical costs compared with general population.

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Heart rate variability in patients with Systemic Lupus Erythematosus: a systemic review and methodological considerations

LUPUS (2018) 27, 1225-1239

This is a detailed review from Poland on a topic that is becoming increasingly studied.  Heart rate and hart rate variability are controlled by the autonomic nervous system (the “automatic” organs such as the lungs, the heart, the blood vessels, the skin temperature and the gut).

It is interesting to know that a number of studies are appearing linking autonomic dysfunction (such as POTS: Paroxysmal orthostatic tachycardia syndrome) with certain autoimmune disorders – Sjogren’s Syndrome, Hughes Syndrome and Lupus.  Measurement of heart rate variability could prove a useful clinical tool.

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Selena Gomez, Lupus and the impact of celebrity health disclosure on public awareness

LUPUS (2018) 27 1045-1046

This interesting letter to Lupus highlighted the influence of the media, and in this case, the public disclosure of the lupus diagnosis by Selina Gomez on public awareness.

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Characteristics of Systemic Lupus Erythematosus in a sample of the Egyptian population: a retrospective cohort of 1109 patients from a single centre

LUPUS (2018) 27, 1030-1038

This study of over a thousand lupus patients came from a respected lupus group in Cairo, studied and treated between 1980 and 2016.

The results were in agreement with other studies conducted in the middle East.  However, one striking difference was that major organ involvement was exceptionally low.

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Trends in use of Hydroxychloroquine during pregnancy in Systemic Lupus Erythematosus patients from 2001 to 2015

LUPUS (2018) 27, 1012-1017

Hydroxychloroquine has become an important drug in lupus, especially in the long-term management of mild to moderate cases.  However, its use in pregnancy is limited, both by the patients’ desire to avoid all medication, but also, significantly, by the opinion of many doctors that hydroxychloroquine is contraindicated – stopped at pregnancy onset – with the almost expected flare three months into the pregnancy. 

This study from Boston of 5,300 lupus patients found that the percentage of patients continuing hydroxychloroquine during pregnancy rose from 12.4% (in 2001) to 37.7% in 2015.  Still a low figure.

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Hydroxychloroquine desensitisation, an effective method to overcome hypersensitivity – a multicentre experience

LUPUS (2018) 27, 703-707

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.

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The cutaneous manifestations are significantly related to cerebrovascular in a Serbian cohort of patients with Hughes Syndrome.

LUPUS (2018) 27 858-863

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).

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Predictive factors of flares in Systemic Lupus Erythematosus patients: data from a multi-ethnic Latin American cohort

LUPUS (2018) 27, 536-544

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.

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Antiphospholipid antibodies and non-thrombotic manifestations of Systemic Lupus Erythematosus.

LUPUS (2018) 27, 665-699

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.

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Individuals living with Lupus: findings from the LUPUS UK Members Survey 2014

LUPUS (2018) 27, 681-687

This was the result of a 2014 survey of 2527 member of the national charity LUPUS UK.


  • Mean time to diagnosis 6-4 years
  • 47% initially had a different diagnosis prior to lupus
  • Fatigue (91%) and joint pain 77% were commonest symptoms
  • 73% were unable to carry out their usual activities
  • 32% were seeking support beyond medication
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Efficacy and safety of Tacrolimus in Systemic Lupus Erythematosus patients with refractory Thrombocytopenia: A retrospective study

LUPUS (2018) 27, 60-65

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".

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Antiphospholipid (Hughes) Syndrome: Insights for Orthopaedics

LUPUS (2018) 27, 3-5

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.

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Hughes Syndrome/APS. 30 years on. What have we learnt?

Lupus (2014) 23, 400-406

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..

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Lupus, Antiphospholipid Syndrome and Epilepsy: An Update

Lupus (2017) 26, 3-5

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.

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Stroke in SLE & APS: risk factors, clinical manifestations, neuroimaging, and treatment.

LUPUS (2017) 26, 529-536

This is a very detailed review of the etiology 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.

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Stillbirth, the impact of APS

LUPUS (2017) 26, 237-239

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”.

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