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You are here: Home / Articles / APS: What Rheumatologists Should Know about Hughes Syndrome

APS: What Rheumatologists Should Know about Hughes Syndrome

February 17, 2016 • By Graham R.V. Hughes, MD, FRCP

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One of the questions on which there is debate is the prevalence of psychiatric manifestations.

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Explore This Issue
February 2016

It has been my view that acute psychosis has been more a feature of lupus than APS, but in this I may be wrong. One of my patients, a woman with APS and OCD (obsessive compulsive disorder), improved with anticoagulation treatment. Interestingly, her teenage son, also a patient with OCD (and aPL positive), found the neuropsychiatric manifestations were far less prominent when aspirin treatment was started.

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Not the Brain

Bone & joints—Have you suffered any fractures? A question not perhaps part of the routine history taking in APS patients. And yet, spontaneous bone fracture is becoming well recognized following the report of 27 spontaneous metatarsal fractures by Dr. Shirish Sangle in APS patients.30

Anecdotal reports suggest that (ischemic) bone fractures may be an important manifestation of the disease: My colleague, Professor Munther Khamashta, has a Hughes syndrome patient with normal DEXA and parathyroid studies who has had 57 spontaneous fractures. Clearly, this is an important area for clinical research.

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Likewise, avascular necrosis (AVN) hip pain is not uncommon in our APS patients. MRI has shown early AVN in some cases with no other risk factors, such as steroids. My clinical impression is that the hip pain often improves when heparin or warfarin is started.

Heart—A major concern is the presence of angina and other cardiac symptoms in APS. Despite a few early reports of myocardial ischemia and coronary thrombosis, it’s only in recent years that publications revealing a high frequency of these complications have increased in frequency.

Two examples are the research of Greco et al showing a relatively high prevalence of positive aPL tests in patients with cardiac ischemia, and the striking observation that women smokers on the pill who were aPL positive had a relative risk of 22 times for the development of myocardial infarction.31,32

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The cardiac links are increased by the reporting of an association with cardiac syndrome X, angina with normal coronary angiographs.33 It’s a fair bet that aPL testing will become a standard in the cardiology clinic, especially in symptomatic women under 40.

Like migraine, stroke is one of the recognized complications of APS/Hughes syndrome. Figures as high as 1 in 5 young strokes (under 45) having positive aPL tests have been reported.

GI tract & liver—Since we made our observations on focal stenotic lesions in various arteries, including celiac and mesenteric arteries, we have focused more on symptoms of abdominal angina in patients with aPL/APS.34 This is a difficult condition to quantify, but we have seen a number of cases in whom clopidogrel or heparin has resulted in improvement (sometimes marked) in post-prandial pain.

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Filed Under: Conditions, Systemic Inflammatory Syndromes Tagged With: Antiphospholipid Antibody Syndrome (APS), brain, Clinical, Diagnosis, Hughes Syndrome, joint, patient care, pregnancy, rheumatologist, stroke, symptom, thrombosisIssue: February 2016

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