The antiphospholipid syndrome is a multisystemic disorder comprising of venous and arterial thrombotic events, recurrent unexplained fetal losses, moderate thrombocytopenia, and a high frequency of neurologic events with laboratory findings of a positive lupus anticoagulant test or anticardiolipin antibody. It may occur in association with other disorders, particularly autoimmune diseases, in which case it is referred to as secondary antiphospholipid syndrome. But when it occurs without obvious underlying disease, it is primary antiphospholipid syndrome. We report a case of primary antiphospholipid syndrome in a 5 year old female child who had a cerebrovascular attack, moderate thrombocytopenia and splenomegaly.
Antibodies, Anticardiolipin ; Antiphospholipid Syndrome* ; Autoimmune Diseases ; Child* ; Child, Preschool ; Female ; Humans ; Lupus Coagulation Inhibitor ; Splenomegaly ; Thrombocytopenia

Antiphospholipid syndrome (APS) is an autoimmune hypercoagulability syndrome. The clinical feature of the antiphospholipid syndrome is characterized by venous or arterial thromboses, recurrent fetal losses and/or thrombocytopenia with evidence of antiphospholipid antibodies, namely lupus anticoagulant, anticardiolipin antibodies. Although APS is most commonly associated with systemic lupus erythematosus or related autoimmune disease (secondary APS), APS also has been identified in patients with vaso-occlusive disease without any other manifestations of connective tissue disease (primary APS). In primary APS, aortic thrombosis has been rarely reported. We report a case of thrombosis of abdominal aorta in primary APS.
Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid ; Antiphospholipid Syndrome* ; Aorta, Abdominal ; Autoimmune Diseases ; Connective Tissue Diseases ; Humans ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic ; Thrombocytopenia ; Thrombophilia ; Thrombosis*

Antiphospholipid syndrome is defined as the presence of lupus anticoagulant antibodies or anticardiolipin antibodies with vascular thrombosis or specific pregnancy complications. Antiphospholipid syndrome can be associated with autoimmune, malignant or infectious diseases. Cutaneous manifestations of antiphospholipid syndrome are variable and can be a first clue to the syndrome or other associated diseases. We report a case of systemic lupus erythematosus and secondary antiphospholipid syndrome presenting as livedo reticularis on both legs of a patient. We recommend that patients with livedo reticularis should be examined for systemic lupus erythematosus or antiphospholipid syndrome.
Antibodies ; Antibodies, Anticardiolipin ; Antiphospholipid Syndrome ; Communicable Diseases ; Humans ; Leg ; Livedo Reticularis* ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic* ; Pregnancy Complications ; Thrombosis

Antiphospholipid syndrome is a multi-system disorder characterized by arterial or venous thromboses and antiphospholipid antibodies, such as lupus anticoagulant or anticardiolipin antibodies. Most common clinical manifestations are recurrent pregnancy losses and deep vein thromboses. Cardiac manifestations in antiphospholipid syndrome include valve abnormalities, occlusive arterial disease, intracardiac emboli, and ventricular dysfunction. Acute myocardial infarction is a rare manifestation of the primary antiphospholipid syndrome. We have experienced a case of myocardial infarction with antiphospholipid syndrome. A 35-year-old man with no cardiovascular risk factors, other than smoking, presented with chest pain. He was diagnosed with an acute myocardial infarction. Our evaluation for coagulapathy revealed elevated lupus anticoagulant antibody. The antiphospholipid syndrome should be considered early in the differential diagnosis as an important cause of unexplained thrombosis in young patients.
Adult ; Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid ; Antiphospholipid Syndrome ; Chest Pain ; Diagnosis, Differential ; Humans ; Lupus Coagulation Inhibitor ; Myocardial Infarction ; Pregnancy ; Risk Factors ; Smoke ; Smoking ; Thrombosis ; Venous Thrombosis ; Ventricular Dysfunction

Antiphospholipid antibody syndrome(APS) has been proposed for those patients with systemic lupus erythematosus(SLE) or with other connective tissue disease who have antiphospholipid antibody and manifestations of venous thrombosis, arterial occlusions, thrombocytopenia, hemolytic anemia, recurrent fetal loss, leg ulcers, and livedo reticularis. A primary antiphospholipid antibody syndrome(PARS), implying those patients who do not have any features of lupus or other connective tissue disease, has been mentioned in many reports but not definitely defined in our country, We recently experienced one case of primary antiphospholipid syndrome with neurologic manifestation. We present this case with a review of the literature.
Anemia, Hemolytic ; Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid* ; Antiphospholipid Syndrome* ; Connective Tissue Diseases ; Humans ; Leg Ulcer ; Livedo Reticularis ; Lupus Coagulation Inhibitor ; Neurologic Manifestations ; Thrombocytopenia ; Venous Thrombosis

Antiphospholipid syndrome (APS) is characterized by a combination of arterial/venous thrombosis or obstetric morbidity and antiphospholipid antibodies such as anticardiolipin antibody or lupus anticoagulant. Digital gangrene is an uncommon manifestation of primary APS. A 61 year-old woman was admitted because of acrocyanosis and progressive digital gangrene in both hands for 1 month. Serologically, lupus anticoagulant was positive. Angiography showed obstruction of digital arteries with collaterals, but there was no evidence of atheromatous plaque or vasculitis. Cardiac echogram did not reveal intracardiac thrombosis or valvular vegetations. She had no evidence of other thrombotic disorders, connective tissue disease, infections or malignancy. Treatment of intravenous heparin and prostaglandin E1 led to stabilization of the symptom, and the necrotic digits were surgically removed. Pathological examination of the amputed digital stump showed arterial and arteriolar thrombotic occlusion, proliferation of capillaries, and ischemic necrosis. After oral anticoagulation therapy, she has been followed without recurrence.We describe the first case of primary APS presenting as digital gangrene in Korea and review the literatures.
Alprostadil ; Angiography ; Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid ; Antiphospholipid Syndrome* ; Arteries ; Capillaries ; Connective Tissue Diseases ; Female ; Gangrene* ; Hand ; Heparin ; Humans ; Korea ; Lupus Coagulation Inhibitor ; Middle Aged ; Necrosis ; Thrombosis ; Vasculitis

OBJECTIVE: To investigate the association of antiphosphoilpid antibodies with thrombosis and to evaluate the clinical usefulness of anticardiolipin isotypes measurement in patients with systemic lupus erythematosus (SLE). METHODS: We examined the prevalence of IgG, IgM and IgA anticardiolipin antibod (aCL) isotypes measured by ELISA assay and lupus anticoagulant (LA) measured by kaolin clotting time in 158 patients. RESULTS: The prevalence of aCL and their IgM and IgA isotypes were 43.7%, 24. 7%, 17. 7% and 17. 1% respectively. The prevalence of LA was 7.8%. The presence of IgG aCL (81,81%, odds ratio(OR)=17.55, p (0.001) and LA (45.45%, OR=16.67, p ( 0. 001) were significantly associated with thrombosis. A regression analysis showed that IgG aCL (OR=13.62, p=0.002) and LA (OR=10.59, p=0. 002) were also associated with thrombosis. CONCLUSION: Our results suggest that the detection and serial monitoring of IgG aCL is a useful tool for diagnosis of thrombotic complication due to antiphosholipid antibodies in SLE.
Antibodies ; Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid* ; Diagnosis ; Enzyme-Linked Immunosorbent Assay ; Humans ; Immunoglobulin A ; Immunoglobulin G ; Immunoglobulin M ; Kaolin ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic* ; Prevalence ; Thrombosis*

Libman-Sacks endocarditis is characterized by noninfectious vegetations on the cardiac valves of patients with systemic lupus erythematosus (SLE). Valvular abnormalities are found in about one third of patients with primary anitiphospholipid syndrome. SLE patients, with antiphospholipid antibodies, have a higher prevalence of valvular involvement than those without these antibodies. A 29 year old man was referred for evaluation of severe orthopnea. He presented with the clinical features of multi-organ failure (heart, lung and kidney). His serological and immunological findings were typical of a SLE flare up. His level of anticardiolipin antibody (IgG) was highly increased and his lupus anticoagulant was positive. A blood culture revealed no growth of bacterial organism. Transthoracic and transesophageal echocardiography showed non-mobile, verrucous vegetations on the anterior leaflet of the mitral valve, with moderate mitral regurgitation.
Adult ; Antibodies ; Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid ; Echocardiography, Transesophageal ; Endocarditis* ; Heart Valves ; Humans ; Lung ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic ; Mitral Valve ; Mitral Valve Insufficiency* ; Prevalence

BACKGROUND: Many infections are associated with antiphospholipid antibodies (aPLs). The purpose of this study was to investigate the prevalence, persistence, clinical significance, and characteristics of aPLs in hepatitis B virus (HBV)-infected patients. METHODS: This study included 143 patients with HBV infection and 32 healthy individuals as controls. The presence of anticardiolipin antibodies (aCL Ab), anti-beta2-glycoprotein I antibodies (beta2GPI Ab), and lupus anticoagulant (LA) was assessed. RESULTS: The total prevalence of aPLs in HBV-infected patients was 12.6% (18 of 143). Of these 18 patients, 15 had low to medium titers of aCL Ab (10 with IgM, 4 with IgG, and 1 with both isotypes). beta2GPI Ab and LA were detected in 3 (2.1%) and 2 (1.4%) patients with HBV infection, respectively. In follow-up specimens from 14 patients with elevated levels of aCL Ab or beta2GPI Ab, 10 (71.4%) showed the persistent presence of aPLs. No clinical manifestations related to aPLs were identified. CONCLUSION: In HBV-infected patients, the most frequently detected antiphospholipid antibodies were IgM aCL Ab, which have a weak association with the clinical manifestations of APS. Unlike the transient presence reported for other infection-associated aPLs, most aPLs were persistently detected over a 12-week period in patients with HBV infection.
Antibodies ; Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid ; Follow-Up Studies ; Hepatitis B virus ; Hepatitis B, Chronic ; Hepatitis, Chronic ; Humans ; Immunoglobulin G ; Immunoglobulin M ; Lupus Coagulation Inhibitor ; Prevalence

Thrombosis is a well known manifestation in patients with systemic lupus erythematosus, along with lupus anticoagulant, anticardiolipin antibody and anti beta2-glycoprotein I. We describe here a 44-year-old female with an abdominal aorta thrombosis of SLE and the patient had no antiphospholipid antibodies. She had this unusual site of thrombosis and this was associated with protein C and S deficiency. She had no other cause of thrombosis. After anticoagulant treatment, her thrombosis of the abdominal aorta resolved.
Adult ; Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid ; Aorta ; Aorta, Abdominal ; beta 2-Glycoprotein I ; Female ; Humans ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic ; Protein C ; Thrombosis