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Antiphospholipid Syndrome (APS) Patient Management

Diagnosis in autoimmune diseases (ADs) is rarely singular. Testing may uncover one disease, but these patients are likely to have additional or related autoimmune conditions. For example, who have an underlying disease, such as Systemic Lupus Erythematous (SLE), may be diagnosed with APS with confirmed positive antiphospholipid antibodies (APL).1,2

When evaluating the patient’s prognosis and developing his or her treatment plan, in addition to conventional risk factors for cardiovascular events, an aPL (antiphospholipid) profile is helpful.3 Several studies reported that patients with multiple positivity for aPL antibodies tests have a higher risk for thrombotic events.3,4 While patients with APS may still develop morbidity and mortality despite treatment, the evaluation of a patient’s risk factors from aPL testing can help inform your clinical decisions to prevent the complications.5

Several well-established clinical guidelines can help facilitate the creation of a management plan that can then be personalized to meet that patient’s needs:

PROGNOSIS

Recurrent thrombosis, which can lead to thrombotic events, is the hallmark of APS. Functional prognosis in patients diagnosed with APS is considered poor, with considerable morbidity and mortality despite current selected therapies. To determine a patient’s long-term prognosis, you will need to evaluate his or her risk for repeated thrombotic events.6 Not all aPL carriers display a similar thrombotic hazard. Several risk factors can help you determine the likelihood of your patient experiencing these potentially fatal events:7

  • aPL Profile - The thrombotic risk increases with the number of positive aPL tests, and patients with three positive tests among lupus anticoagulant, anti-cardiolipin and anti-β2 glycoprotein display the highest vascular hazard. Patients carrying both anti-cardiolipin and anti-β2 glycoprotein I isotypes are at higher risk of developing clinical events.
  • Cardiovascular Risk Factors - Particularly, hypertension has emerged as an independent predictor for a first thrombotic event in aPL carriers.
  • Site of Thrombosis- The risk of recurrence was higher for arterial than venous events
blood cells

Special attention should be paid to patients with APS who are pregnant or are planning to become pregnant.

Some of these patients will present with only one symptom, such as repeated pregnancy loss or cytopenia (thrombocytopenia or hemolytic), while others will experience the entirety of the syndrome. Unfortunately, treatments may fail to prevent complications associated with APS in 20-30% of pregnancies.8

Underlying Diseases Associated with APS

Ongoing headaches, memory loss, chest pain, anemia, and fatigue should not be dismissed as primarily linked to APS, as these can also be associated with a myriad of diseases within the autoimmune landscape, particularly SLE, with a smaller cohort being linked to SS, and RA.9 Percentages of patients with APS who have concurrent, underlying autoimmune conditions range from:9

2.2 percent of patients

Sjogren's Syndrome:
2.2% of patients

1.8 percent of patients

RA:
1.8% of patients

36 percent of patients

SLE:
up to 36% of patients

Precise Management - An Approach Unique to Each Patient

The clinical presentations of APS manifestations may be highly diverse; as with many other conditions, so it is important to personalize the patient’s management. Observation of the patient’s clinical events and an assessment of his or her particular prothrombotic risk factors can help you determine which targeted therapies are most appropriate.6 It is also important to identify and personalize management strategies that can address those risk factors.

Patient Management

Continued Testing and Monitoring

Patients with autoimmune diseases are inherently more likely to develop additional or related autoimmune conditions, so it’s important that a long-term management plan be tailored to the signs and symptoms presented at the time. A thorough follow-up is needed to identify an undiagnosed associated disorder in an individual who has not yet displayed signs or symptoms. This is particularly important with overlap syndrome, which may cause manifestations due to multiple syndromes or diseases.

Due to the wide clinical and serological spectrum of the syndrome, there remains no standard practice for treating aPL-positive patients, and each patient must be treated and managed on an extremely individualized basis.7 You may need to pursue further diagnostic testing as you continue to monitor overlapping symptoms and clinical events so that you can further refine your treatment plan.

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References
  1. Ünlü O, Zuily S, Erkan D. The clinical significance of antiphospholipid antibodies in systemic lupus erythematosus. Eur J of Rheumatol. 2016;3(2):75-84.
  2. Rojas-Villarraga A, Amaya-Amaya J, Rodriguez-Rodriguez A, et al, Introducing Polyautoimmunity: Secondary Autoimmune Diseases No Longer Exist. Autoimmune Diseases. 2012;2012:9254319.
  3. Sciascia Savino, Sanna Giovanni, Murru Veronica, et al. GAPSS: the Global Anti-Phospholipid Syndrome Score. Rheumatology. Oxford University Press; 2013 Aug;52(8):1397-1403.
  4. Otomo Kotaro, Atsumi Tatsuya, Amengual Olga, et al. Efficacy of the antiphospholipid score for the diagnosis of antiphospholipid syndrome and its predictive value for thrombotic events. Arthritis & Rheumatism. John Wiley & Sons, Inc.; 2012 Feb;64(2):504-512.
  5. Cervera R, Serrano R, Pons-Estel G J, Ceberio-Hualde L, et al. Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients. Annals of the Rheumatic Diseases. BMJ; 2015 Jun 24;74(6):1011-1018.
  6. Del Papa N, Vaso N. Management of Antiphospholipid Syndrome. Ther Adv Musculoskel Dis. 2010;2(4):221-227.
  7. Chighizola CB, Ubiali T, Meroni PL. Treatment of Thrombotic Antiphospholipid Syndrome: The Rationale of Current Management - An Insight into Future Approaches. J of Immunol Res. 2015;2015:951424.
  8. Schreiber K, Hunt BJ. Pregnancy and Antiphospholipid Syndrome. Semin Thromb Hemost. 2016;42(7):780-788.
  9. Cervera R , Piette JC , Font J et al. Antiphospholipid syndrome: Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients . Arthritis Rheum 2002; 46: 1019-1027.