항인지질증후군/항인지질항체증후군 Antiphospholipid syndrome (APLS, APS)

피곤하고 왼쪽 가슴 아래 쪽이 아파 왔는데. splenic infarct이었다. 이유는 APLS 였다. 

Lupus anticoagulant test  positive+ splenic infarct . clexane + warfarin bridging 하며 퇴원. 

 

Clinical manifestations 

1) Thrombotic events 

-The hallmark of APLS : venous thromboses are more common than arterial thromboses 

-The risk of both venous and arterial thrombosis and/or thromboembolism is increased in individuals with positive tests for lupus anticoagulant (LA) activity 

-The risk of recurrent thrombosis or thromboembolism may be further enhanced in those with "triple positivity" upon repeated testing 

2) NEurologic involvement 

3)Haematologic abnormalities 

4)Pulmonary involvement 

5)Cardiac involvement 

6)Cutaneous manifestations 

7)Kidney disease 

8)Gastrointestinal involvement 

9)Ocular involvement 

10)Adrenal disease 

11)Osteonecrosis 

 

Lab findings 

1.The three major aPL test 

1)Anticardiolipin antibodies (aCL) immunoglobulin G (IgG) and/or IgM enzyme-linked immunosorbent assay (ELISA)

2)Anti-beta2 glycoprotein (GP) I antibodies IgG and/or IgM ELISA

3)Lupus anticoagulant (LA) test

-moderate-to high titer of IgG or IgM anticardiolipin or anti-beta2GPI antibodies 

-the diagnosis of APLS is made in the presence of one or more of the above aPL in the setting of a vascular thrombosis or a specific type of pregnancy morbidity. 

 

The criteria also requires a repeat APLA test to be positive 12weeks after the initial positive test to exclude clinically unimportant or transient antibodies. 

 

An aPL may be present in some people who do not have APS and are otherwise healthy, who have another autoimmune or rheumatic disease, or who have been exposed to certain drugs or infectious agents.

 

2.Thrombocytopenia 

3.hypocomplementemia 

4.the prolongation of a blood coagulation test (eg. aPTT) 

5.Haemolytic anaemia 

6.a history of a false-positive serologic test for syphilis 

 

Initial management of acute thrombosis 

In individuals with suspected APS,warfarin is typically preferred over a direct oral anticoagulant (DOAC), although a DOAC may reasonably be used in selected individuals. 

 

If the baseline PT and/or aPTT are prolonged, they cannot be used to adequately assess anticoagulation, and it is necessary to use an alternative assay that is insensitive to the aPL (eg, anti-factor Xa activity for heparin, alternative thromboplastin reagent for warfarin ).

 

Secondary thrombosis prevention 

-Long term anticoagulation 

 :suggest anticoagulation with warfarin rather than DOAC 

:For most individuals with APS and an unprovoked thrombotic event, we recommend lifelong anticoagulation. 

-limited role of alternative agents 

      -Immunomodulatory agents 

          :Rituximab, hydroxychloroquine 

      -Statins 

      -Eculizumab 

https://www.ncbi.nlm.nih.gov/books/NBK430980/#:~:text=In%20addition%20to%20clinical%20criteria,clinically%20unimportant%20or%20transient%20antibodies.