systemic lupus erythematous ( sle ) is an autoimmune disease presenting many kinds of autoantibodies in serum . in case of elevated levels of antiphospholipid antibodies ,
the patient presents recurrent episodes of arterial and venous thrombosis , thrombocytopenia and spontaneous fetal losses .
the vascular thrombosis may occur at any small and large vessels resulting in various clinical manifestations . here , we reported a case of secondary antiphospholipid syndrome in a lupus patient who was presented with budd - chiari syndrome , due to the thrombosis of the intrahepatic portion of the inferior vena cava ( ivc ) and persistent elevation of anticardiolipin antibody .
a 32-year old korean female was admitted with a complaint of edema and pain on the left leg for 2 months . in january 1993 , she was diagnosed as sle at another hospital and manifested gross hematuria , proteinuria , azotemia , anemia , thrombocytopenia , hypocomplementemia , positive fana , positive anti - ds dna antibody and positive anti - ena antibody . in may 1993 , she was transferred to kangnam st .
we checked the titer of anticardiolipin antibody ( aca ) , which was 100 gpl iu / ml and followed up the titer of aca regularly at 2 months intervals and the titer of aca igg was persistently elevated .
her parity was 0 - 0 - 0 - 0 and she showed thrombocytopenia ( 35,000/mm ) and prolonged partial thromboplastin time ( 59.2 sec , control 27.0 sec ) .
her clinical features were antiphospholipid syndrome in sle and she was treated with baby aspirin 100 mg / day and steroid ( prednisolone 1mg / kg ) at the outpatient clinic . from april 1994 , she suffered from edema and pain on the left leg and was admitted to our hospital in june 1994 . on admission , she had polyarthralgia but did not complain of fever , malar rash , photosensitivity , oral ulcer , raynaud s phenomenon , xerostomia , xerophalmia and allopecia . on physical examination ,
pulse rate 76 beats per minute , respiration rate 20 per minute and body temperature 36.5c .
there were no pathologic lesions in her eyes , ears , nasal or oral mucosa .
hemoglobin was 10.6 g / dl , hematocrit 32% , white blood cell 8.110/mm ( neutrophil 82% .
renal function showed blood urea nitorgen 5.3 mg / dl , creatinine 0.9 mg / dl and 24hr urine protein 6.18 g / day .
urinanalysis showed 0 to 2 white cells and 10 to 20 red cells per high power fields .
iu / l , alkaline phosphatase 229 iu / l , total bilirubin 0.7 mg / dl , total protein 4.1 g / dl and albumin 2.1 g / dl .
the prothrombin time was 11.8 sec ( control 12.1 sec ) and activated partial thromboplastin time 59.2 sec ( control 26.6 sec ) .
on immunologic studies , fana was positive ( homogenous pattern , titer 1 : 1280 ) , anti - ds dna antibody 5 iu / ml , c3 21 mg / dl and c4 15 mg / dl .
rheumatoid factor was negative and anca was positive ( gs - ana , titer 1 : 80 ) .
the immunoglobulin g , a , m levels revelaed 928 , 274 , 106 mg / dl , respectively .
the serum viral hepatitis markers revealed that hbs antigen was negative , hbs antibody positive and hcv antibody negative .
abdominal ultrasonography showed a moderate amount of ascites , moderate splenomegaly and marked coarse increased liver echogenecity . at computed tomography of the abdomen
doppler ultrasonography of the left leg showed no thrombosis in the superficial femoral vein and popliteal vein .
inferior and superior venocavograms showed obstructions at the intrahepatic portion of ivc and at both subclavian veins and abnormal collateral vessels were found around the obstructions ( fig . 2 , 3 ) .
we injected heparin 5,000 units and urokinase 500,000 units intravenously at the bolus during venocavogram and further thrombolytic therapy ( heparin 5,000 units / day and urokinase 500,000 units / day continuously all day long ) was done for additional two days .
we followed the venocavogram to evaluate the extent of thrombosis , compared with pre - thrombolytic therapy , but we could not find any interval change .
we decided to give the patient warfarin 5 mg / day , prednisolone 1 mg / kg and baby aspirin 100 mg / day and to follow her up at the outpatient clinic .
the antiphospholipid syndrome ( aps ) is characterized by the production of autoantibodies directed against negatively charged phospholipid .
clinically , it is associated with multiple thrombotic events , thrombocytopenia and recurrent fetal loss .
the syndrome can be primary without other systemic disease or secondary , i.e. part of systemic lupus erythematosus .
antiphospholipid antibodies ( apls ) are also found in certain malignancies , or as a side effect to certain drugs .
the prevalence of apls in sle is 20% to 50% and approximately one - third of sle patients may have clinical manifestations of aps . the exact mechanism by which apls cause aps is still unkown .
since phospholipid is the essential constituent of cell membrane and coagulation pathway , most efforts to elucidate the mechanism have been based on vascular endothelial cell and platelet function .
antiphospholipid antibodies reduce the release of protacyclin at the vascular endothelial cell and enhance the production of thromboxane at the platelet and interfere with many cascades of the coagulation pathway - factor x - converting complex , prothrombinase complex , antithrombin iii , thrombomodulin and protein c and s etc .
protein c and a co - factor , protein s , act together to degrade activated factors v and viii , which leads to inhibit clotting .
antiphospholipid antibodies require a serum co - factor in order to bind to phospholipid , known as 2-glycoprotein 1 ( 2-gp1 ) .
2-glycorpotein 1 inhibits the activation of factor xii and the formation of prothrombinase complex , here , apls disturb the inhibitory effect of 2-gp1 resulting in the hypercoagulable state .
these thromboses may occur at any organs , causing thrombophlebitis and gangrene of the limbs , budd - chiari syndrome , addison s disease , pulmonary embolism , livedo reticularis , retinal vessel thrombosis , stroke , myocardial infarction , renal artery thrombosis , hepatic infarction , chorea , psychosis and avascular necrosis of bones .
budd - chiari syndrome is characterized by ascites , hepatomegaly , abdominal pain , varicose veins on the lower legs and collateral vessels at the abdominal wall because of the obstruction of the hepatic vein or the inferior vena cava .
the syndrome was described , at first , by budd and chiari in 1845 and 1899 , respectively , and has been mainly reported in east asia , south africa and india .
the pathogenesis of vascular obstruction is still unclear but some cases of membranous obstruction of the inferior vena cava ( movc ) have been reported in korea , and movc has been known to have a relationship with oral contraceptive agents or type b viral hepatitis . recently .
budd - chiari syndrome , associated with lupus anticoagulant , has been reported in many countries , but budd - chiari syndrome , as a manifestation of secondary antiphospholipid syndrome in sle , is rare and has not yet been reported in korea .
the patient who has secondary antiphospholipid syndrome could die unexpectedly , from either cardiopulmonary arrest or circulatory failure , depending on which organ is predominanlty involved by the extensive occlusions . in this instance
, the differential diagnosis is often difficult between lupus vasculitis , thrmobotic thrombocytopenic purpura or disseminated intravascular coagulation . to eleminate apls , energetic immunosuppression , e.g. , with pulse cyclophosphamide , plasmapheresis or gammaglobulin infusion
is sometimes effective , but there is usually a rapid rebound to pre - treatment levels shortly after discontinuation of the therapy .
anti - hypertensives , cholesterol - lowering agents and treatment of active nephritis are needed to remove additional risk factors .
it is important to prevent occlusive vascular complications in patients who have not experienced thrombosis .
for the prophylaxis of venous thermbosis , low - dose subcutaneous heparin ( 10,000 to 15,000 units / day ) is useful and patients who show resistance to the above doses require higher doses of subcutaneous heparin(25,000 units / day ) or intravenous heparin(40,000 units / day ) . in the case of occlusive vascular complications ,
patients require anticoagulation on a long - term basis , and probably for life , because of the tendency of recurrence .
warfarin is used up to 20 mg / day and inr should be kept a a level of 3 to 4 rather than 2 to 2.5 . in the case of using heparin ,
anticoagulation should be controlled by the recalcification test ( howell test ) not by aptt because of the interference of the apls with the latter . to prevent fetal loss , low dose aspirin ( 30 to 80 mg / day )
if the patient is receiving warfarin , because of previous thrombotic manifestation , this must be changed to subcutaneous heparin , at least in the first trimester , since warfarin is teratogenic .
 antiphospholipid syndrome is characterized by recurrent episodes of arterial and venous thrombosis , spontaneous fetal losses , thrombocytopenia and persistently elevated levels of antiphospholipid antibodies . 
 we experienced a case of budd - chiari syndrome in a 32-year old female lupus patient who was presented with left leg edema , ascites and esophageal varix . 
 the clinical and laboratory findings were compatible with the cirteria for systemic lupus erythematosus ( sle ) and she was found to have anticardiolipin antibody , thrombocytopenia and prolonged partial thromboplastin time . initially , she was treated with intravenous heparin and urokinase and she was followed up with warfarin , baby aspirin and steroids . 
INTRODUCTION
CASE REPORT
DISCUSSION