Antiphospholipid syndrome - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Antiphospholipid syndrome (APS) is the association of persistently elevated antiphospholipid antibodies with a variety of clinical features characterized by thromboses and pregnancy-related morbidity.
Venous, arterial and microvascular thrombosis can affect any vessel in the body.
Main treatment goals are management of acute thrombosis and prevention of thrombosis recurrence and pregnancy morbidity.
Consideration should also be given to the presence of coexistent autoimmune disease in patients with antiphospholipid syndrome.
Catastrophic disease, which presents as multiple thrombotic events within a short period, often as multiorgan failure, occurs in <1% of patients.

Magnetic resonance direct thrombus imaging of bilateral proximal DVT

From the personal teaching collection of Professor Hunt; used with permission

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History & Exam
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Definition

Epidemiology

Etiology

Pathophysiology

Images

Fibrin immunostain of renal biopsy demonstrating features consistent with thrombotic microangiopathy
Trichrome stain of renal biopsy demonstrating features consistent with thrombotic microangiopathy
Magnetic resonance direct thrombus imaging of bilateral proximal DVT
Livedo reticularis
Livedo reticularis

Citations

Key Articles

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.[Abstract][Full Text]
Barbhaiya M, Zuily S, Naden R, et al. 2023 ACR/EULAR antiphospholipid syndrome classification criteria. Ann Rheum Dis. 2023 Oct;82(10):1258-70.[Abstract][Full Text]
Tektonidou MG, Andreoli L, Limper M, et al. EULAR recommendations for the management of antiphospholipid syndrome in adults. Ann Rheum Dis. 2019 Oct;78(10):1296-304.[Abstract][Full Text]
Sammaritano LR, Bermas BL, Chakravarty EE, et al. 2020 American College of Rheumatology guideline for the management of reproductive health in rheumatic and musculoskeletal diseases. Arthritis Rheumatol. 2020 Apr;72(4):529-56.[Abstract][Full Text]
American College of Obstetricians and Gynecologists. ACOG practice bulletin no. 132: antiphospholipid syndrome. Dec 2012 [internet publication].[Full Text]

Referenced Articles

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