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AIMS: To review the historical reports on antiphospholipid antibodies (aPL) from the early years of the 20th century; to outline the cardinal features of the antiphospholipid syndrome (APS) from 1983 on, including clinical criteria, etiopathogenesis and current therapy. METHODS: Literature review using PubMed. Articles on the history of aPL and APS were selected. RESULTS: The original aPL were described in patients with syphilis yet in 1906 by Wassermann. A first definition of lupus anticoagulant was proposed in 1963,while the anticardiolipin antibody (aCL) test was depicted twenty years later. The APS, initially reported by Hughes in 1985as the "aCL syndrome", is one of the most prevalent acquired thrombophilia. Venous and arterial thrombosis, associated or not to pregnancy morbidity, comprise the main features. It is a novel disorder firstly associated to systemic lupus erythematosus. A primary form of APS was put forward in 1989, and many APS variants are currently known. Lifelong, full-dose anticoagulation is the mainstream for treatment of thrombotic APS. In obstetric APS, the combination of acetil-salicilic acid and enoxoparin has been a mostly effective therapy. CONCLUSIONS: The sequential characterization of aPL since Wassermann in 1906, and later of the APS in the 1980-thies, is a rather interesting example of how a new entity is sketched step by step. APS is an intriguing novel cause of autoimmune thrombophilia, with a complex pathogenesis and a plethora of clinical and laboratory abnormalities. Treatment is based on life-long anticoagulation.
OBJETIVOS: Revisar os relatos históricos sobre anticorpos antifosfolípides (aAF) dos primeiros anos do século XX; delinear as características cardinais da síndrome antifosfolípide (SAF) a partir de 1983, incluindo critérios clínicos, etiopatogênese e terapia atual. MÉTODOS: Revisão de literatura utilizando o PubMed. Foram selecionados artigos com foco na história dos aAF e da SAF. RESULTADOS: Os aAF foram originalmente descritos em pacientes com sífilis ainda em 1906 por Wassermann. Uma primeira definição do anticoagulante lúpico foi proposta em 1963, enquanto o anticorpo anticardiolipina (aCL) foi descrito 20 anos mais tarde. A SAF, inicialmente reportada por Hughes em 1985 como "síndrome do aCL" é uma das mais prevalentes trombofilias adquiridas. Tromboses arteriais e venosas, associadas ou não à morbidade gestacional, compreendem os achados principais. É uma nova entidade, tendo sido primeiramente associada ao lupus eritematoso sistêmico. Uma forma primária de SAF foi reconhecida em1989, e muitas variantes de SAF são modernamente conhecidas. A terapia-padrão para a SAF trombótica é a anticoagulação plena e ininterrupta. Na SAF obstétrica, a combinação de ácido acetil-salicílico com enoxaparina tem-se mostrado altamente efetiva. CONCLUSÕES: A caracterização sequencial dos aAF desde Wasserman em 1906, e mais tarde da SAF nos anos 1980, é um interessante exemplo de como uma nova entidade é concebida passo a passo. A SAF é uma nova e intrigante causa de trombofilia autoimune, com uma complexa patogênese e uma pletora de manifestações clínicas e laboratoriais. O tratamento é baseado em anticoagulação contínua.
Subject(s)
Pregnancy Complications , Thrombosis , Antiphospholipid SyndromeABSTRACT
A 74-year-old male presented to us with a history of vision loss for 36 hours in the right eye (RE). The RE had a visual acuity of hand movements. The fundus revealed a pale retina, cattle tracking in the retinal vessels, and a cherry-red spot at the macula. The patient was a known case of pyoderma gangrenosum (PG) and had received intravenous methylprednisolone and cyclophosphamide at the onset of visual symptoms. An emergency anterior chamber paracentesis was performed following unsuccessful attempts of ocular massage. The patient improved to 6/9 in the RE 4 months after paracentesis. The patient had an aggressive course of PG, for which he needed a combination of oral steroid, immunomodulator therapy and biologicals. An association between central retinal arterial occlusion and PG has not been reported before, according to the best of authors' knowledge.
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PURPOSE: To report a case of monocular elevation deficiency as the presenting manifestation of systemic lupus erythematosus (SLE). CASE SUMMARY: A 23-year-old, otherwise healthy female presented with a 3-day history of vertical diplopia and headache. She had a left hypotropia, which worsened in adduction and supra-duction and a profound inferior oblique underaction (-3). Magnetic resonance imaging showed an enhancement around the left superior oblique muscle and multiple infarctions in the left midbrain. On repetitive serological tests, anemia, lymphopenia, and anti-phospholipid antibody were positive. A presumptive diagnosis was a myositis of left superior oblique muscle and hyper-coagulation related with anti-phospholipid antibody. Two months after high-dose steroid treatment, the vertical diplopia was resolved. Five months later, the left hypotropia recurred as a more severe form with the inability to elevate the left eye in all directions. In addition, the infarction associated with vasculitis recurred in the left midbrain. As the treatment with high-dose steroid failed to relieve her ocular symptoms, recession of the left inferior rectus was performed 8 months later. One month after the surgery, she developed multiple lesions of erythematous nodosa with tenderness. Skin biopsy of the lesion in the fingers showed the histological findings consistent with lupus. CONCLUSIONS: Eye movement abnormality can be an initial manifestation of SLE, which should be considered as a differential diagnosis especially in young female patients.
Subject(s)
Female , Humans , Young Adult , Anemia , Biopsy , Diagnosis , Diagnosis, Differential , Diplopia , Eye Movements , Fingers , Headache , Infarction , Lupus Erythematosus, Systemic , Lymphopenia , Magnetic Resonance Imaging , Mesencephalon , Myositis , Serologic Tests , Skin , VasculitisABSTRACT
Objective:To observe the change of CD4+CD25+regulatory T cells and Th17 cells in mice with experimental anti-phospholipid antibody syndrome ( EAPS ) .Methods: EAPS model was established by immunizing BALB/c mice with recombinant humanβ2 glycoprotein 1 (rhβ2GP1).The levels of serum anti-β2 glycoprotein 1 (anti-β2GP1),anti-cardiolipin antibody (aCA),IL-17,IL-2,IL-6 and TGF-βwere tested by ELISA.The rate of abortion,activated partial thromboplastin time (APTT) and platelet count were also detected.Flow cytometry was applied to detect the percentages of the CD4+CD25+regulatory T cells and Th17 cells in peripheral blood mononuclear cells.Results:Compared with the control group,the levels of anti-β2 GP1,aCA,IL-17,IL-2 and IL-6 were significantly increased,the rate of abortion was increased,APTT time was prolonged and the levels of TGF-βand platelet count were de-creased in model mice (P0.05),but percentage of Treg cells was lower than that in control group after 12 weeks (P<0.05);the percentage of Th17 cells in model group was higher than that in control group (P<0.05).In addition,the ratio of Treg/Th17 cells was lower in model mice than that in control group.Conclusion: The imbalance of CD4+CD25 Treg/Th17 cells may participate in the pathogenesis of EAPS.
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Objective To investigate clinical features, diagnosis, and treatment of refractory Mycoplasma pneumoniae pneumonia (MPP) combined with thrombosis. Methods One case with refractory MPP associated with thrombosis was retrospectively analyzed with literature review. Results The patient presented with respiratory symptom at the onset, while melosalgia and decreased respiratory sound in left lung were occurred during anti-infection therapy. Thereafter, thrombosis of lower extremity veins and pulmonary embolism were confirmed by a series of examinations. Serum anti-phospholipid antibody was positive. Finally, the patient was treated with a combination of anticoagulation and immunosuppressive therapies. Conclusions The mechanism of refractory MPP combined with thrombosis may be associated with excessive inflammatory response and endothelial cells injury. The thrombosis complication should be suspected in patient of Mycoplasma pneumonia infection with positive anti-phospholipid antibody and low concentration of protein C and immunosuppressive therapy should be implemented promptly.
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Anti-phospholipid antibody syndrome (APLS) is characterized by the presence of anti-phospholipid antibodies, arterial or venous thrombosis, recurrent abortion, and thrombocytopenia. Although heart valve abnormalities are found in most patients with APLS, acute type A dissection associated with APLS is rare. A 44-year-old woman with systemic lupus erythematosus and APLS, who had been treated with corticosteroids, immunosuppressive agents, and warfarin, was admitted with severe back pain. Computed tomography demonstrated aortic dissection extending from the ascending to the abdominal aorta. Emergency ascending aorta replacement was performed. The hypercoagulation associated with APLS made it difficult to achieve optimal postoperative anticoagulant control. Moreover, corticosteroids and immunosuppressive agents may result in postoperative infection. However, this patient was discharged without complications 14 days after the operation.
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Objective To investigate the suppression to 2-glycoprotein-1-specific B cell response by human recombinant 2-glycoprotein-1 domain Ⅰ dimer(rh?2-GP1-DⅠ2) in rh?2-GP1 immunized mice.Methods The effects of treatment using rh?2-GP1-DⅠ2 on titer and ratio of anti-?2-GP1 were analyzed by solid phase ELISA.The number of splenic ?2-GP1-specific antibody-forming cells(AFC) was counted by ELISPOT.Results The levels of anti-?2-GP1 from rh?2-GP1 immunized mice treated with rh?2-GP1-DⅠ2 were significantly decreased compared with those in negative controls(P
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Objective To detect the levels of homocysteine (Hcy) and anti-phospholipid antibodies (APLA) in the hematoplasma of the patients with deep venous thrombosis (DVT), discuss the reason of DVT recurrence and search for the predictors of it. Methods Sixty cases with DVT in our department from January 2001 to April 2003 were collected, which were divided equally into two groups as primary and recurrent, and first degree relative of the 30 DVT recurrent patients were also collected. The author established a control group using 30 cases of out-patient clinic without varicose veins of lower extremity or insufficient venae profundae. Hcy was detected with fluorescence polarization immunoassay (FPIA) and APLA 〔anticardiolipin antibody, ACLA (IgG, IgM); lupus antibody (LA)〕were detected with enzyme linked immunosorbent assay (ELISA). Odds ratios (OR) were also calculated to assess the relative risk of each study group. Results The values of Hcy and ACLA (IgG, IgM) in the primary group and recurrent group were both significantly higher than those of control group and first degree relative group of DVT recurrent patients (P