Management of antiphospholipid syndrome-- pearls

from Garcia D. and Erkan D. Diagnosis and management of the antiphospholipid syndrome. NEJM 378; 2010:2021.  

1.  Ten percent of healthy blood donors are positive for apl  antibodies and one percent are positive for lupus anticoagulant; however after one year, only one percent remain positive, so rechecking titers is key. Its rare for a truly healthy person to  remain positive. Transient apl is common during infections.

2.  Prevalence by underlying condition of persisting moderate to high risk apl antibody profiles:  SLE, 20-30 percent;  women with pregnancy complications, six percent;  patients with venous thrombosis, ten percent;  MI, 11 percent; patients less than fifty with stroke, 17 percent.  

3.  Clinical presentation pearls: among pregnant women, most occur after ten weeks of pregnancy (those in earlier period more likely have genetic anomalies causing miscarriage).  Patients with venous thromboembolism most likely have lower extremity or pulmonary emboli.  

4.  Other clinical features include pulmonary hypertension, livedo reticularis, thrombocytopenia, hemolytic anemia, acute or chronic renal vascular lesions, and moderate or severe cognitive impairment.  

5.  Other lab features to note:  LA test best correlates with clinical events, but may be misleading among patients on warfarin or DOACs.  For ELISA, moderate to high titers ( greater than 40 GPL or 99th percentile of cal or anti B2GP1correlate better with outcomes than lower titers; IgG is more strongly associated with bad clinical events than IgA.  

6. Treating traditional risk factors and avoiding estrogen  is very important.

7.  Anticoagulation for primary prevention or use of aspirin for primary prevention is still controversial.  For patients with persistent apl syndrome and provoked thrombosis, eg. by surgery, or for those with impersistent apl ab's, the benefit of prolonged anticoagulation is "less certain."  

8. Treatment of "warfarin failures" is not known, but options include high intensity warfarin to INO 3-4, addition of aspirin or plaquenil or statin, use of a different anticoagulant such as a  LMWH or a combination.  There is insufficient evidence about DOACs.  

9. Catastrophic apl syndrome includes ARF, ARDS and adrenal hemorrhage.  Diagnosis is definite with involvement of three or more organs and a persistently positive test.  Early treatment with anticoagulants, steroids, IVIG and PLEX is indicated.  

10. Treatment in pregnancy is with low quality evidence, use of low dose aspirin and LMWH,  

if platelets are more than 50 K, no acute therapy

if platelets are more than 20 K, first line is steroids and IVIG not splenectomy

for warm mediated HA steroids are used first

ARF with thrombotic microangiopathy is usually treated with PLEX

Valve disease with high risk use ASA or warfarin for high risk vegetations

APS criteria-- once clinical event (venous or arterial) and/or fetal loss after ten weeks and/or 3 sequential miscarriages before 10 weeks  AND either present LA, ACL ab, or antiB2glycoprotein