Humans ; Antibodies, Antiphospholipid ; Antiphospholipid Syndrome/drug therapy*

OBJECTIVE: Catastrophic antiphospholipid syndrome (CAPS), also known as Asherson's syndrome, is a special subtype of antiphospholipid syndrome (APS) characterized by multiple intravascular thrombosis involving multiple organs systems or tissues simultaneously or continuously, high titer antiphospholipid antibodies and high mortality rate. This article's aims was to analyze the clinical manifestation, laboratory examination and treatment therapy of CAPS for the purpose of improving the understanding, diagnosis and treatment of the disease in clinical practice. METHODS: Retrospective analysis and descriptive statistics were applied to the clinical manifestations and laboratory findings of 14 CAPS cases from APS Shanghai Database (APS-SH) with catastrophic antiphospholipid. RESULTS: Of the 14 CAPS patients, 12 cases satisfied the 2003 CAPS Classification Criteria accepted in the 10th International Congress on Antiphospholipid Antibody, and were diagnosed as definite APS and 2 cases were diagnosed as probable CAPS. Three cases were categorized as primary APS and 11 as APS secondary to systemic lupus erythematosus (SLE). Infection was mostly commonly seen before the onset of CAPS, followed by SLE activity and surgery. Among the involved organs, systems and tissues, brain and lung were most commonly affected sites of arterial thrombosis while peripheral vein was most commonly affected in venous thrombosis events among the clinical events. Triple positivity of anticardiolipin antibody (aCL), anti-β2 glyeoprotein I antibody (aβ2GPI), lupus anticoagulant (LA) were detected in 54.55% of the patients. Thrombocytopenia and decreased hemoglobin were frequently seen in the CAPS patients, and the majority proved to be hemolytic anemia. Of all the cases, 6 ended with death. The triple therapy strategy (anticoagulants, glucocorticoid, intravenous immunoglobulin and/or plasma exchange) could help to improve prognosis, cyclophosphamide and rituximab might benefit the patients with other comorbidities such as SLE and micro-angiopathic hemolytic anemia (MHA). CONCLUSION CAPS patients suffer from life-threatening acute multiple small vessel thrombosis with high titer of antiphospholipid antibody, potentially leading to multiple organ failure and a poor prognosis, thus early diagnosis and sufficient treatment are critical to prevent the progression of disease and improve the prognosis.
Antibodies, Antiphospholipid ; Antiphospholipid Syndrome/therapy* ; Catastrophic Illness ; Humans ; Lupus Coagulation Inhibitor ; Retrospective Studies ; Thrombosis/etiology*

Antibodies, Antiphospholipid ; immunology ; therapeutic use ; Antiphospholipid Syndrome ; diagnosis ; immunology ; therapy ; Child ; Humans

The neurological manifestations of antiphospholipid syndrome (APS) are diverse. Transverse myelitis (TM) is an uncommon, but well-known neurological complication of systemic lupus erythematosus (SLE). On the other hand, the reported cases associated with primary APS are extremely rare. To our knowledge, this is the first report of TM in a patient with primary APS in Korea. A 32-year-old male patient was admitted with the sudden onset of numbness, a tingling sensation, and weakness in both lower extremities. He had a 19 months history of external iliac and femoral arterial thromboses prior to admission. The laboratory results indicated the presence of anticardiolipin antibodies of the IgG class and lupus anticoagulant. No other autoantibodies were detected and there were no apparent clinical manifestations of SLE or multiple sclerosis. A T2-weighted magnetic resonance (MR) image showed swelling and increased intensity of the cervical and thoracic spinal cord between C6 and T7 with slight enhancement by contrast medium. After steroid pulse therapy, the patient's symptoms were gradually relieved and the abnormal findings on MR imaging disappeared.
Adult ; Antiphospholipid Syndrome/*complications ; Human ; Male ; Myelitis, Transverse/*etiology/therapy

Antiphospholipid syndrome (APS) is primarily considered to be an autoimmune pathological condition that is also referred to as "Hughes syndrome". It is characterized by arterial and/or venous thrombosis and pregnancy pathologies in the presence of anticardiolipin antibodies and/or lupus anticoagulant. APS can occur either as a primary disease or secondary to a connective tissue disorder, most frequently systemic lupus erythematosus (SLE). Damage to the nervous system is one of the most prominent clinical constellations of sequelae in APS and includes (i) arterial/ venous thrombotic events, (ii) psychiatric features and (iii) other non- thrombotic neurological syndromes. In this overview we compare the most important vascular ischemic (occlusive) disturbances (VIOD) with neuro-psychiatric symptomatics, together with complete, updated classifications and hypotheses for the etio-pathogenesis of APS with underlying clinical and laboratory criteria for optimal diagnosis and disease management.
Antibodies, Antiphospholipid/immunology ; Antiphospholipid Syndrome/diagnosis/*immunology/therapy ; Arterial Occlusive Diseases/diagnosis/*immunology/therapy ; Cerebrovascular Disorders/diagnosis/immunology/therapy ; Humans ; Lupus Erythematosus, Systemic/diagnosis/immunology/therapy

Antiphospholipid Syndrome ; diagnosis ; etiology ; therapy ; Child ; Humans ; Lupus Erythematosus, Systemic ; complications ; Male

Abortion, Habitual ; complications ; Antibodies, Antiphospholipid ; blood ; Antiphospholipid Syndrome ; complications ; pathology ; therapy ; Erythema ; complications ; Heart Valve Diseases ; complications ; Humans ; Kidney Diseases ; complications ; Skin Diseases ; complications ; Thrombosis ; complications

Antiphospholipid syndrome is a thrombotic disorder characterized by arterial or venous thrombosis with the presence of antiphospholipid antibodies (aPA). We reported a 38- year-old man suffering deep vein thrombosis associated with antiphospholipid antibody syndrome. He underwent an interventional procedure of intravascular thrombolytic therapy and stent insertion due to deep vein thrombosis in the lower extremities. On the next day of the procedure, he complained of low back pain, motor weakness in lower extremities, sensory loss and voiding difficulty. Lumbar MRI revealed epidural hematoma between T12 and L2 spine, resulting in cauda equina syndrome. Twenty days later, pulmonary thromboembolism was newly diagnosed. In laboratory test, aPA was detected. Therefore, he was finally diagnosed as antiphospholipid syndrome. We reported this unusual case with the review of literatures.
Antibodies, Antiphospholipid ; Antiphospholipid Syndrome* ; Hematoma ; Low Back Pain ; Lower Extremity ; Magnetic Resonance Imaging ; Polyradiculopathy ; Pulmonary Embolism ; Spine ; Stents ; Thrombolytic Therapy ; Venous Thrombosis*

This case report we presented aims to report a-31-year-old man with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) who developed myocardial infarction (MI) and also aims to discuss the possible mechanisms. The results showed that traditional risk factors alone do not cause coronary heart disease with SLE, and SLE-related factors influence the atherogenic process. We found that although SLE patients with acute MI benefit from percutaneous coronary intervention (PCI) therapy, it is very important to choose the reasonable antithrombotic strategies in patients with SLE and APS undergoing PCI who require oral anticoagulant therapy.
Adult ; Angioplasty, Balloon, Coronary ; Antiphospholipid Syndrome ; complications ; Humans ; Lupus Erythematosus, Systemic ; complications ; Male ; Myocardial Infarction ; etiology ; therapy

BACKGROUNDS: Antiphospholipid antibodies which are frquently found in systemic lupus erythematosus and primary antiphospholipid syndrome are associated with recurrent abortions and thromboembolism. In this study the authors investigated whether antiphospholipid antibodies are found in Graves disease, a representative organ-specific autoimmune disease and what is the clinical implication of the antiphospholipid antibodies if they appear in Graves disease. METHODS: Anticardiolipin antibody and lupus anticoagulant activity were measured in 57 untreated hyperthyroid Graves patients. 42 euthyroid patients with thyroid nodules served as controls. RESULTS: Eight of the 57 patients with Graves disease had anticardiolipin antibody which was significantly more frequent than in control group. Six of the eight patients who had anticardiolipin antibody had IgM type antibody and two had IgG type antibody. All their antibody activity declined with several months of antithyroid drug therapy and finally disappeared when the patients became euthyroid. Presence of anticardiolipin antibody had no relationship with clinical events such as spontaneous abrtion and thromboembolism. CONCLUSION: Anticardiolipin antibody is frequently found in patients with Graves disease. They seem to appear as an epiphenomenon of autoimmunity and they seem not to have any clinical implications.
Abortion, Habitual ; Antibodies, Anticardiolipin* ; Antibodies, Antiphospholipid ; Antiphospholipid Syndrome ; Autoimmune Diseases ; Autoimmunity ; Drug Therapy ; Female ; Graves Disease* ; Humans ; Immunoglobulin G ; Immunoglobulin M ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic ; Pregnancy ; Thromboembolism ; Thyroid Nodule