Contents
Importantly
- What is the reason for suspecting antiphospholipid syndrome (APLS)?
Suspected ApL diagnosis
- Disease history (thromboembolism, spontaneous abortion)
- Results of medical examinations (see below)
- Antibodies (ANA, anti-cardiolipin and / or beta2 glycoprotein) and / or lupus anticoagulant (coagulation laboratory)
Disease history
- Known Systemic lupus erythematosus (secondary antiphospholipid syndrome)
- Thromboembolism?
- Deep Vein Thrombosis (DVT)
- Pulmonary embolism
- TIA (transient ischemic attack)
- Stroke
- Arterial embolisms
- Known other risk factors for thromboembolism?
- Smoking
- Pill
- Leiden mutation
- Atherosclerosis
- Atrial fibrillation
- Other
- Results of prior pregnancies?
- Spontaneous abortions (period of pregnancy) and deaths
Medical examinations
- Clinical investigation findings
- Signs of SLE or other rheumatic disease?
- Arthritis, skin, blood (low blood cell count), kidneys (edema, urine test), heart, lungs, ANA and other antibodies
- Skin symptoms: Livedo reticularis / «Marbling» in the skin?
- Serum antibody: ANA (possibly subgroups), Cardiolipin antibody, Beta-2 glycoprotein antibody, Lupus anticoagulant (Coagulation laboratory)
- Thrombocytopenia (not often)
Differential diagnoses for CAPS (Kazzaz, NM, 2016) | ||||||
CAPS | TTP HOUSE | DIC | HELP | SRC | HIT | |
Microvascular thrombosis | + | + | + | + | + | - |
Macrovascular thrombosis | + | - | - | + | - | + |
Bleeding | - | - | + | +/- | - | - |
Multiorganic failure | ++ | +/- | +/- | +/- | - | - |
Kidney failure | +/- | ++ | - | +/- | ++ | - |
Mentally reduced | +/- | +/- | - | +/- | - | - |
Lung Disease (ARDS) | ++ / - | - | +/- | +/- | - | - |
Heart disease (Infarction, failure) | +/- | - | - | - | - | - |
Pregnancy | +/- | - | +/- | + | - | - |
Infection | +/- | +/- | +/- | - | - | - |
Malignancy | +/- | - | +/- | - | - | - |
Hemolytic anemia | +/- | ++ | +/- | + | + | - |
Schistocytter | +/- | ++ | +/- | + | + | - |
Thrombocytopenia | +/- | ++ | + | + | +/- | ++ |
Extended PTT | +/- | - | + | - | - | - |
Fibrinogen | Normal | Normal | ↓ | Normal | Normal | Normal |
Liver enzymes | ↑ | Normal | Normal | ↑↑ | Normal | Normal |
APL (> 40 U / ml) | ++ | - | - | - | - | - |
ADAMTS13 | Normal | ↓ | ↓ | Normal | Normal | Normal |
Anti-PF4 | - | - | - | - | - | ++ |
Referral to specialist
- A rheumatologist-referral is applicable if rheumatic symptoms are present or in case of "secondary antiphospholipid syndrome" which is related to SLE or (less often) other rheumatic disease.
- Hematologist referral if a primarily APLS is suspected.
- Maternity clinic/gynaecologist contacted if planned or occurred pregnancy.
Norwegian Directorate of Health (Supervisor for referrals)
Antifosfolipid syndrome BINDVEVESSYKDOMMER.no
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