Further Outpatient Care

Subsequent outpatient care includes the following:

Carefully monitor medication doses and the INR if applicable.
Closely observe the patient for clinical events.
Ensure the care of any underlying connective-tissue disease.

Inpatient & Outpatient Medications

The suggested medications include heparin, warfarin, aspirin, and, in selected cases, hydroxychloroquine, intravenous immunoglobulin, and corticosteroids.

Corticosteroids are rarely used for the treatment of recurrent fetal loss because of the increased risk of maternal morbidity. Generally, the use of corticosteroids is reserved for specific nonthrombotic manifestations, such as associated thrombocytopenia, autoimmune hemolytic anemia, or the treatment of an underlying connective-tissue disease.

Prescribe antihypertensive drugs when necessary.

Administer antihyperlipidemic agents including statins when appropriate.

Transfer

When treating seriously ill patients with catastrophic APS, transfer the patient to a setting where plasma exchange can be performed or where intravenous immunoglobulin or cyclophosphamide can be administered if needed.

Deterrence/Prevention

Instruct the patient to avoid smoking.

Inform the patient to avoid oral contraceptives or estrogen replacement therapy.

Ensure that the patient avoids any prolonged immobilization.

Complications

Permanent functional disability or death can occur at a relatively young age. Complications may include the following:

Stroke
Myocardial infarction
Pulmonary hypertension
Renal failure ^[41]

Prognosis

With appropriate medication and lifestyle modifications, most individuals with primary antiphospholipid syndrome (APS) lead normal healthy lives. However, subsets of patients continue to have thrombotic events despite aggressive therapies. In these patients and in patients with catastrophic APS, the disease course can be devastating, often leading to significant morbidity or early death.

In large European cohort studies, 10-year survival is approximately 90-94%.^[42]

A retrospective study suggested that hypertension or medium-to-high titers of IgG anticardiolipin antibody are risk factors for a first thrombotic event in asymptomatic patients with antiphospholipid (aPL) antibodies.^[43]Primary prophylaxis against thrombosis appears to offer significant protection in such cases. Annual incidence of first thrombosis is approximately 0-5% in patients with a positive aPL test without previous thrombosis.^[16]

Patients with secondary APS carry a prognosis similar to that of patients with primary APS; in the former, however, morbidity and mortality may also be influenced by these patients' underlying autoimmune or rheumatic condition. In patients with SLE and APS, aPL antibodies have been associated with neuropsychiatric disease and have been recognized as a major predictor of irreversible organ damage.

Women with aPL antibodies who experience recurrent miscarriages may have favorable prognoses in subsequent pregnancies if treated with aspirin and heparin.

Patient Education

Patient education may include the following:

Stress the importance of early recognition of a possible clinical event.
Instruct patients taking warfarin to avoid sports with excessive contact.
Instruct patients taking warfarin to avoid excessive consumption of foods that contain vitamin K. ^[44]
Limit activity in patients with acute DVT.
Instruct the patient to avoid prolonged immobilization.
Discuss the importance of planned pregnancies so that long-term warfarin can be switched to aspirin and heparin before pregnancy is attempted.
Stress the importance of minimizing modifiable risk factors in primary prevention of aPL-positive patients with no history of thrombosis.

For patient education information, see Blood Clot in the Legs.

Questions

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Media Gallery

Antiphospholipid syndrome. Livedo reticularis.
Antiphospholipid syndrome. Arterial thrombosis resulting in ischemia and necrosis of the foot.

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Author

Suneel Movva, MD Fellow in Rheumatology, Division of Rheumatology, Allergy and Immunology, Winthrop University Hospital

Disclosure: Nothing to disclose.

Coauthor(s)

Elise Belilos, MD Section Head of Rheumatology, Division of Rheumatology, Allergy and Immunology, Winthrop University Hospital; Assistant Professor of Clinical Medicine, Department of Internal Medicine, Stony Brook University School of Medicine

Elise Belilos, MD is a member of the following medical societies: American College of Rheumatology, Arthritis Foundation

Disclosure: Nothing to disclose.

Steven Carsons, MD Chief, Division of Rheumatology, Allergy and Immunology, Winthrop University Hospital; Professor of Medicine, Stony Brook University School of Medicine

Steven Carsons, MD is a member of the following medical societies: American College of Rheumatology, New York Academy of Sciences, Society for Experimental Biology and Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Lawrence H Brent, MD Associate Professor of Medicine, Sidney Kimmel Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Stock ownership for: Johnson & Johnson.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Carlos J Lozada, MD Director of Rheumatology Fellowship Training Program, Professor of Clinical Medicine, Department of Medicine, Division of Rheumatology and Immunology, University of Miami, Leonard M Miller School of Medicine

Carlos J Lozada, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology

Disclosure: Received honoraria from Pfizer for consulting; Received grant/research funds from AbbVie for other; Received honoraria from Heel for consulting.

Acknowledgements

The authors gratefully acknowledge the contributions of Amiel Tokayer, MD.