1.Antiphospholipid Syndrome and Vascular Ischemic (Occlusive) Diseases: An Overview.
Yonsei Medical Journal 2007;48(6):901-926
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
2.Raise the realization of childhood rheumatic disease.
Chinese Journal of Pediatrics 2003;41(6):405-407
3.Lupus anticoagulant-hypoprothrombinemia in healthy adult.
Choon Hae CHUNG ; Chi Young PARK
The Korean Journal of Internal Medicine 2008;23(3):149-151
The presence of lupus anticoagulant is associated with an elevated risk of venous and arterial thrombosis, and recurrent miscarriages as well. For some cases, this disease can present with bleeding as a consequence of lupus anticoagulant hypoprothrombinemia (LAHPS). LAHPS is a rare disease and it is reported to be most frequent in young females with/without systemic lupus erythematosus or in healthy children who are suffering with a viral infection. In such cases, steroid therapy is usually effective in normalizing the biological abnormalities and controlling the bleeding problems. A 34-year-old previously healthy man was admitted to our department because of his prolonged coagulation times; these abnormalities were discovered before performing orthopedic surgery. The prothrombin time (PT) was 15.2 sec, and the activated partial thromboplastin time (APTT) was 37.7 sec. A 1:1 dilution of patient plasma with normal plasma nearly corrected the PT, but this failed to correct the APTT. Evaluation of the clotting factors revealed decreased levels of factors II, V, VIII, IX and XI. The presence of LA was demonstrated by the dRVVT test, and the patient was diagnosed with LAHPS. He was successfully treated with corticosteroid before performing the orthopedic surgery.
Adrenal Cortex Hormones/therapeutic use
;
Adult
;
Humans
;
Hypoprothrombinemias/*diagnosis/drug therapy/immunology/physiopathology
;
Lupus Coagulation Inhibitor/*immunology
;
Lupus Erythematosus, Systemic/*diagnosis/immunology/physiopathology
;
Male
;
Partial Thromboplastin Time
;
Preoperative Care
;
Prothrombin Time
4.Clinical significance of antiphospholipid antibody in pediatric patients and review of literature.
Hui-Jie XIAO ; Ji-Yun YANG ; Tian-Ji GAO ; Jian-Ping HUANG ; Yong YAO ; Yan ZHANG
Chinese Journal of Pediatrics 2004;42(8):571-573
OBJECTIVEAntiphospholipid antibody (APL) is a particularly important laboratory diagnostic criterion for antiphospholipid syndrome (APS). The significances of positive APL in childhood are seldom reported nor fully understood. The purpose of this study was to analyze 13 cases with positive APL seen in our hospital and to study the relationship between the positive rates of APL and various clinical diseases especially systemic lupus erythematosus (SLE) in order to improve the clinical diagnoses and treatment level of APS in children.
METHODSThe clinical data collected from 2000 to 2002 of 13 hospitalized children with positive APL were retrospectively evaluated. Enzyme linked immunosorbent assay (ELISA) and indirect immunofluorescence technique were used respectively to detect APL and antineutrophil cytoplasmic autoantibodies (ANCA) of sera from those children. Other various indexes were also detected according to different characteristics of different diseases.
RESULTSEight cases had SLE; 2 had acute post-streptococcal infections. The other 3 cases did not show any evidences of primary diseases; they probably had primary APS. SLE was the most common primary diseases to cause development of APL and the cases with SLE showed more severe cutaneous vasculitis than SLE patients who were negative for APL. There was no significant relationship between the positive rates of APL and that of ANCA. Eight APL positive cases complicated with thrombocytopenia and bleeding were treated with high dosage of immunoglobulin [400 mg/(kg.d), for 3 - 5 d] intravenously; the clinical conditions of these cases were ameliorated soon. While the 5 cases who had thrombotic vasculitis and thromboembolism were treated with anticoagulant and antithrombotic therapy with low molecular weight heparin [50 - 100 U/(kg.d)], which led to good clinical effects.
CONCLUSIONSThe clinical manifestations of children positive for APL were somehow different from those of adults. Positive APL itself may be nonspecific, it can occur from different causes of diseases. APL detection may be useful to suggest anticoagulant and/or antithrombosis therapy. Treatments for APS should be variable according to different causes and severity of diseases, in the cases of thrombocytopenia and bleeding, high dose intravenous immunoglobulin should be given as soon as possible, while in the cases of thrombotic vasculitis and thromboembolism, anticoagulant and antithrombotic therapy should be given soon.
Adult ; Antibodies, Antineutrophil Cytoplasmic ; blood ; Antibodies, Antiphospholipid ; blood ; immunology ; Anticoagulants ; therapeutic use ; Antiphospholipid Syndrome ; blood ; complications ; diagnosis ; therapy ; Child ; Fibrinolytic Agents ; therapeutic use ; Hemorrhage ; etiology ; therapy ; Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Lupus Erythematosus, Systemic ; immunology ; Streptococcal Infections ; immunology ; Thrombocytopenia ; etiology ; therapy ; Thromboembolism ; drug therapy ; etiology ; Thrombosis ; drug therapy ; etiology ; Vasculitis ; drug therapy ; etiology
5.Case reports of transient loss of vision and systemic lupus erythematosus.
Annals of the Academy of Medicine, Singapore 2007;36(2):146-149
INTRODUCTIONNeuropsychiatric manifestations can occur in up to two-thirds of patients with systemic lupus erythematosus (SLE). The presentations as well as the underlying immunopathogenic mechanisms can be heterogeneous and therefore have an enormous impact on therapeutic options.
CLINICAL PICTUREWe describe 2 patients who presented similarly with acute onset binocular reversible visual loss. The first patient had anti-phospholipid syndrome and optic neuritis, while the second patient suffered from posterior reversible leukoencephalopathy syndrome.
TREATMENTPatient one was treated with anti-coagulation and immunosuppression while the second patient required the withdrawal of immunosuppression and supportive therapy.
OUTCOMEBoth patients responded favourably and had complete visual recovery.
CONCLUSIONSDifferent management strategies have to be employed for similar presentations having different aetiologies, underscoring the need for constant clinical vigilance.
Adult ; Antiphospholipid Syndrome ; complications ; etiology ; Brain Diseases ; etiology ; immunology ; Epilepsy, Tonic-Clonic ; etiology ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; microbiology ; physiopathology ; therapy ; Lupus Vasculitis, Central Nervous System ; diagnosis ; Magnetic Resonance Imaging ; Optic Neuritis ; etiology ; Salmonella Infections ; complications ; Salmonella enteritidis ; Time Factors ; Vision Disorders ; etiology ; immunology