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Antiphospholipid Syndrome and Pregnancy Differential Diagnoses

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

Diagnostic Considerations

Careful evaluation is necessary following a spontaneous abortion occurring prior to 10 weeks’ gestation before it can be considered unexplained.

With regard to thrombocytopenia, other possible causes of the disorder, such as HIV infection, and various conditions, such as drug -induced thrombocytopenia, thrombotic thrombocytopenia, gestational thrombocytopenia, and preeclampsia, should be considered. Other autoimmune diseases should also be ruled out in patients with thrombocytopenia.

The following are findings that can be secondary to autoimmune disorders other than APS and that require an appropriate evaluation:

  • Arthritis - Patients may have nonerosive arthritis involving 2 or more peripheral joints
  • Serositis - This may be (1) pleuritis or pleural effusion or (2) pericarditis or pericardial effusion
  • Renal disorder - Proteinuria of 0.5 g/day or the presence of cellular casts
  • Neurologic disorder - Criteria include seizures in the absence of other causes or psychosis in the absence of other causes.
  • Hematologic disorder - Features, without an otherwise explainable cause, include (1) hemolytic anemia with reticulocytosis, (2) leukopenia of less than 4000 cells/µL on at least 2 occasions, (3) lymphopenia of less than 1500 cells/µL, or (4) thrombocytopenia of less than 100,000 cells/µL
  • Immunologic disorder

Systemic lupus erythematosus

Many manifestations of SLE can be seen in women with APS. A complete evaluation to exclude SLE as a primary disorder is appropriate if symptoms are present.

The clinical manifestations of SLE include the following:

  • Skin lesions - 84-71%
  • Arthritis - 63-95%
  • Nephritis - 46-77%
  • Raynaud phenomenon - 10-58%
  • Neuropsychiatric features - 0-59%
  • Lymphadenopathy - 0-58%
  • Pleurisy - 37-56%
  • Mucous membrane ulceration - 7-54%
  • Pericarditis - 29-45%
  • Splenomegaly - 9-18%
  • Aseptic necrosis - 0-10%

Clinical evidence of glomerulonephritis is found in more than 50% of cases. However, if biopsies are performed on all patients, the incidence of some nephritis may be as high as 90%. SLE is associated with encephalopathy and seizures, to a lesser degree with ischemic stroke, and, rarely, with subarachnoid hemorrhage.

 
 
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 loss Heparin 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 stroke Heparin 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 thrombosis No 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
LGBSS High-dose IVIG at 400-1500 mg/kg/day for several days IVIG at 400-1500 mg/kg/d for several days
aPL Antibodies Without APS
LAC or medium to high level of aCL IgG No treatment No treatment
Low levels of aCL IgG, only aCL IgM, or only aCL IgA without LA, aPL, or aCL No treatment No treatment
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