Antiphospholipid syndrome

This code identifies a systemic autoimmune disorder characterized by the presence of antiphospholipid antibodies and a history of blood clots (thrombosis) or pregnancy complications. It is a hypercoagulable state where the immune system mistakenly attacks proteins bound to phospholipids, leading to an increased risk of arterial and venous thrombotic events.

Assign this code when documentation explicitly states a diagnosis of antiphospholipid syndrome, often confirmed by laboratory findings of antiphospholipid antibodies (e.g., lupus anticoagulant, anticardiolipin antibodies, anti-beta-2-glycoprotein I antibodies) in conjunction with clinical criteria such as recurrent thrombosis or obstetric morbidity. This code is appropriate for primary antiphospholipid syndrome or when the syndrome is not secondary to another autoimmune condition like systemic lupus erythematosus.

• Do not use this code if the documentation only indicates the presence of antiphospholipid antibodies without a confirmed diagnosis of the syndrome; refer to the Excludes1 note.
• Distinguish this code from lupus anticoagulant syndrome, which has its own specific code.
• Ensure documentation clearly specifies "antiphospholipid syndrome" to support this diagnosis, rather than just antibody findings.