Antiphospholipid syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Feham Tariq, MD [2]

Overview

If left untreated, 90% of patients with antiphospholipid syndrome (APS) progress to develop recurrent thrombotic or thromboembolic events such as pulmonary embolism, Stroke, Transient ischemic attack, Deep vein thrombosis.

Natural History

• If left untreated, 90% of patients with antiphospholipid syndrome (APS) progress to develop recurrent thrombotic or thromboembolic events which include:
  • Pulmonary embolism
  • Stroke
  • Transient ischemic attack
  • Deep vein thrombosis
• Other patients can develop thrombocytopenia, livedo reticularis, skin ulcers, microangiopathic hemolytic anemia (MAHA).
• Obstetrical complications include premature birth, early pregnancy loss and intrauterine growth restriction.

Complications

The complications of APS are described in a systemic order which are as follows:

Thrombotic complications:

Thrombotic events are the hallmark of APS. They are as follows:

Neurological complications:

Following complications are seen on neurological exam:

• Stroke
• Transient ischemic attack

Obstetrical complications: The pregnancy related complications are as follows:

• Maternal thrombosis
• Fetal loss
• Pre- eclampsia
• Placental insufficiency
• Fetal growth restriction
• Iatrogenic preterm birth

Pulmonary complications: Thromboembolic and non-thromboembolic complications include:

• • Diffuse alveolar hemorrhage
  • Pulmonary arterial hypertension
  • Acute respiratory distress syndrome
  • Pulmonary micrthrombosis

Hematological complications:

• • Autoimmune hemolytic anemia
  • Thrombocytopenia
  • Bone marrow necrosis

Cardiac involvement:

• • Valvular thickening and valve nodules also called as Libmann-Sacks endocarditis.
  • Coronary artery disease

Cutaneous complications:

Renal disease:

Adrenal involvement:

• Hemorrhagic infarction of the adrenals

Ocular manifestations:

The ocular manifestations are the following:

• Anterior ischemic optic neuropathy
• Amaurosis fugax
• Retinal venous occlusion

Gastrointestinal complications:

Ischemia of the gastrointestinal tract leads to the following complications:

•  Gastrointestinal bleeding
• Abdominal pain
• Esophageal necrosis with perforation
• Duodenal ulcer

Prognosis

The prognosis of antiphospholipid syndrome is as follows:

• It is associated with increased mortality and morbidity.
• The mean age of death is 59 years.
• Main causes of death in the order of percentage include the following:
  • Thrombosis (31%)
  • Sepsis (27%)
  • Malignancy (14%)
  • Hemorrhage (11%)
  • Systemic lupus erythematosus involvement (8%)
  • Catastrophic APS