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Antiphospholipid Syndrome and Pregnancy Clinical Presentation

  • Author: Teresa G Berg, MD, FACOG; Chief Editor: Thomas Chih Cheng Peng, MD  more...
 
Updated: Apr 15, 2015
 

History

The “international consensus statement for the diagnosis of antiphospholipid syndrome,” published in 1999 by Wilson et al, serves as a set of criteria similar to that for the diagnosis of other autoimmune disorders. The criteria were updated in 2006 to reflect new insights into APS. The diagnosis requires that the patient have at least 1 clinical and 1 laboratory criterion.[7, 8]

Clinical and laboratory criteria

Clinical criteria

The clinical criteria for APS include the following:

  • One or more clinical episodes of arterial, venous, or small-vessel thrombosis, occurring within any tissue or organ
  • One or more unexplained deaths of morphologically normal fetuses at or after 10 weeks’ gestation
  • One or more premature births of morphologically normal fetuses at or before 34 weeks’ gestation because of eclampsia or severe preeclampsia or features consistent with placental insufficiency
  • Three or more consecutive, unexplained spontaneous abortions before 10 weeks’ gestation, with maternal anatomic or hormonal abnormalities and paternal and maternal chromosomal causes excluded

Laboratory criteria

Criteria for laboratory testing, which are consistent with current clinical management guidelines from the American Congress of Obstetricians and Gynecologists, include the following[9] :

  • Anticardiolipin antibodies - Anticardiolipin IgG or IgM antibodies present at moderate or high levels (ie, >40 GPL or MPL or >99th percentile) in the blood on 2 or more occasions at least 12 weeks apart
  • Lupus anticoagulant - LAC detected in the blood on 2 or more occasions at least 12 weeks apart, according to the guidelines of the International Society on Thrombosis and Hemostasis[8]
  • Anti-beta2 -glycoprotein I antibodies IgG or IgM - In titers above the 99th percentile for normal as defined by the laboratory performing the test, on 2 or more occasions at least 12 weeks apart

Additional findings in APS

Findings in APS can also include the following:

  • Unexplained transient ischemic attacks or amaurosis fugax[1]
  • Positive result from the Coombs test
  • Hemolytic anemia
  • Chorea
  • Chorea gravidarum
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Physical Examination

The diagnosis of APS is based primarily on clinical history and laboratory data. Patients with secondary APS are more likely to have findings on physical examination, although some physical findings may be associated with primary APS. Thrombosis and stroke are possible residual neurologic findings in APS.

Cutaneous manifestations of APS can include the following:

  • Digital cyanosis
  • Livedo reticularis
  • Digital gangrene
  • Leg ulcers
  • Discoid rash - Ie, a raised, erythematous patch with keratotic scaling and follicular plugging; older lesions may be atrophic
  • Photosensitivity
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Contributor Information and Disclosures
Author

Teresa G Berg, MD, FACOG Associate Professor, Program Director, Director of the Perinatal Diagnostic Center, Department of Obstetrics and Gynecology, University of Nebraska Medical Center

Teresa G Berg, MD, FACOG is a member of the following medical societies: American Institute of Ultrasound in Medicine, Association of Professors of Gynecology and Obstetrics, Central Association of Obstetricians and Gynecologists, Society for Maternal-Fetal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Thomas Chih Cheng Peng, MD Professor (Collateral), Department Obstetrics and Gynecology, Virginia Commonwealth University School of Medicine, VCU Health System

Thomas Chih Cheng Peng, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American Institute of Ultrasound in Medicine, Society for Maternal-Fetal Medicine

Disclosure: Nothing to disclose.

Acknowledgements

Gregory Locksmith, MD Associate Director, Division of Gynecology, Department of Medical Education, Orlando Regional Health System

Disclosure: Nothing to disclose.

Bruce A Meyer, MD, MBA Executive Vice President for Health System Affairs, Executive Director, Faculty Practice Plan, Interim CEO, University Hospitals; Professor, Department of Obstetrics and Gynecology, University of Texas Southwestern Medical School

Bruce A Meyer, MD, MBA is a member of the following medical societies: American College of Obstetricians and Gynecologists, American College of Physician Executives, American Institute of Ultrasound in Medicine, Association of Professors of Gynecology and Obstetrics, Massachusetts Medical Society, Medical Group Management Association, and Society for Maternal-Fetal Medicine

Disclosure: Nothing to disclose.

Bogdan Nowicki, MD, PhD Head of Infectious Disease Laboratory, Associate Professor, Departments of Microbiology and Immunology, Obstetrics and Gynecology, University of Texas Medical Branch at Galveston

Disclosure: Nothing to disclose.

Stella Nowicki, DDS Head of Infectious Diseases Laboratory, Associate Professor, Departments of Obstetrics and Gynecology, Microbiology and Immunology, University of Texas Medical Branch at Galveston

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

References
  1. Fischer-Betz R, Specker C, Brinks R, Schneider M. Pregnancy outcome in patients with antiphospholipid syndrome after cerebral ischaemic events: an observational study. Lupus. 2012 Oct. 21(11):1183-9. [Medline].

  2. Alijotas-Reig J, Ferrer-Oliveras R, Ruffatti A, et al. The European Registry on Obstetric Antiphospholipid Syndrome (EUROAPS): A survey of 247 consecutive cases. Autoimmun Rev. 2015 May. 14(5):387-395. [Medline].

  3. Bouvier S, Cochery-Nouvellon E, Lavigne-Lissalde G, et al. Comparative incidence of pregnancy outcomes in treated obstetric antiphospholipid syndrome: the NOH-APS observational study. Blood. 2014 Jan 16. 123(3):404-13. [Medline].

  4. Asherson RA, Khamashta MA, Ordi-Ros J, et al. The "primary" antiphospholipid syndrome: major clinical and serological features. Medicine (Baltimore). 1989 Nov. 68(6):366-74. [Medline].

  5. Kochenour NK, Branch DW, Rote NS, Scott JR. A new postpartum syndrome associated with antiphospholipid antibodies. Obstet Gynecol. 1987 Mar. 69(3 Pt 2):460-8. [Medline].

  6. Cowchock FS, Reece EA, Balaban D, Branch DW, Plouffe L. Repeated fetal losses associated with antiphospholipid antibodies: a collaborative randomized trial comparing prednisone with low-dose heparin treatment. Am J Obstet Gynecol. 1992 May. 166(5):1318-23. [Medline].

  7. Wilson WA, Gharavi AE, Koike T, et al. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum. 1999 Jul. 42(7):1309-11. [Medline].

  8. [Guideline] Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006 Feb. 4(2):295-306. [Medline].

  9. [Guideline] The American College of Obstetricians and Gynecologists. Antiphospholipid Syndrome. ACOG Practice Bulletin. January/2011. 118:1-8. [Full Text].

  10. Sciascia S, Murru V, Sanna G, Roccatello D, Khamashta MA, Bertolaccini ML. Clinical accuracy for diagnosis of antiphospholipid syndrome in systemic lupus erythematosus: evaluation of 23 possible combinations of antiphospholipid antibody specificities. J Thromb Haemost. 2012 Dec. 10(12):2512-8. [Medline].

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Table. Proposed Management for Women With aPL Antibodies
Feature Management
Pregnant Nonpregnant
APS with prior fetal death or recurrent pregnancy lossHeparin in prophylactic doses (15,000-20,000 U of unfractionated heparin or equivalent per day) administered subcutaneously in divided doses with low-dose aspirin daily



Calcium and vitamin D supplementation



Optimal management uncertain; options include no treatment or daily treatment with low-dose aspirin
APS with prior thrombosis or strokeHeparin to achieve full anticoagulation (does not cross the placenta)Warfarin administered daily in doses to maintain international normalized ratio of =3
APS without prior pregnancy loss or thrombosisNo treatment or daily treatment with low-dose aspirin or daily treatment with prophylactic doses of heparin plus low-dose aspirin; optimal management uncertain No treatment or daily treatment with low-dose aspirin; optimal management uncertain
LGBSSHigh-dose IVIG at 400-1500 mg/kg/day for several daysIVIG at 400-1500 mg/kg/d for several days
aPL Antibodies Without APS
LAC or medium to high level of aCL IgGNo treatmentNo treatment
Low levels of aCL IgG, only aCL IgM, or only aCL IgA without LA, aPL, or aCLNo treatmentNo treatment
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