Expert explores updated antiphospholipid syndrome guidelines via patient cases
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The emergence of multiple documents outlining treatment recommendations for antiphospholipid syndrome between 2018 and 2020 has saved lives, according to a presenter at the 2020 Congress of Clinical Rheumatology-West.
Maarten Limper, MD, PhD, from the University of Utrecht, in the Netherlands, suggested that a talk about APS based on actual evidence “would have been an impossible task” as recently as 2018. The “sad conclusion” was that, prior to that, recommendations were largely based on consensus and expert opinion. “Things have changed, luckily, in the meantime,” he said.
With that, rather than offering a point-by-point overview of the documents that have emerged since 2018, he framed the talk by discussing three case patients demonstrating the three types of APS. He described one potential thrombotic, one potential obstetric and one potential catastrophic APS patient. The presentation included questions about how and when to make a diagnosis and what the treatment protocol should entail.
The potential thrombotic APS patient was a 54-year-old man with a BMI of 38 who was no longer a smoker but who had smoked for many years. He presented with sudden dyspnea, an O2 saturation of 93% and high heart and respiratory rates. Workup showed no signs of inflammation and no anemia but slightly decreased kidney function.
Limper posed the question of whether such a patient with a first-time, unprovoked thrombotic event should be tested for APS antibodies. He noted that the 2019 European Society of Cardiology guidelines call for testing in patients younger than 50 years with a family history of APS. “This guy was on the cusp,” Limper said. “The right answer is no and yes; both of them are correct.”
Limper ultimately tested the patient, who was diagnosed with thrombotic APS.
Regarding treatment, Limper provided multiple options, including high doses of aspirin; vitamin K antagonist to an international normalized ratio (INR) of 2-3 for 6 months; the same vitamin K regimen for life; or a direct-acting oral anticoagulant (DOAC).
After one year of treatment with vitamin K antagonist to an INR of 2-3, the patient was in stable condition, according to Limper. “DOACs in APS should probably be avoided,” he said.
The final point Limper offered is that if a patient such as this one recurs, clinicians should act immediately because “it will be worse,” he said.
The suspected obstetric APS patient was a 28-year-old woman who did not drink or smoke and played sports regularly. In 2019, she miscarried once at 6 weeks and then, not long after, miscarried again at 8 weeks.
“Would you test for APS antibodies?” Limper asked CCR-West attendees. An affirmative answer would be because these events may, in fact, signify obstetric APS. However, a negative answer is because “miscarriages are not uncommon.”
The 2018 American College of Obstetricians and Gynecologists (ACOG) guidelines stipulate that no workup is needed until after the second consecutive early pregnancy loss, according to Limper. “What they tried to tell us here is that you might consider a test after these two early pregnancy losses,” he said. “The general concept these days is to not wait for a third miscarriage.”
While Limper described this case as a “difficult choice,” he suggested that clinicians recently have been erring on the side of more testing.
The patient was ultimately tested and diagnosed with triple-positive APS. She was treated with low-dose aspirin, according to recommendations. Her condition stabilized but she experienced a stillbirth at 23 weeks. “Unfortunately, this happens to those triple positive patients,” Limper said. “The placenta showed evidence of thrombotic disease.”
The suspected catastrophic APS patient was a 43-year-old man with thoracic pain and multi-organ failure. The question Limper raised was whether a so-called “tissue diagnosis” was necessary before initiating treatment for catastrophic disease, because treatment paradigms for this condition are “not so simple.”
The answer, according to Limper, is to treat at first clinical suspicion. “Luckily, cAPS is rare, because it is bad,” he said. “It is life-threatening. Do not wait until there is clear evidence, or for the third organ to fail.”
Survival outcomes for cAPS are optimized by treatment with “triple therapy” using heparin, corticosteroids and intravenous immunoglobulin (IVIG). “Treat first, then think,” Limper said.
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Limper M. Antiphospholipid Syndrome: State of the Art on Clinical Practice Guidelines. Presented at: Congress of Clinical Rheumatology-West annual symposium; October 8-11, 2020 (virtual meeting).
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