Evaluation of core decompression outcome in systemic lupus erythematosus with hip osteonecrosis: a retrospective cohort study

Abstract Background Osteonecrosis is a major cause of morbidity for patients with systemic lupus erythematosus (SLE). Although core decompression is an approved and trusted technique to prevent further joint deterioration, this surgical method seems to be less beneficial for SLE patients. We aimed to evaluate the outcomes of core decompression in SLE patients with primary stages of femoral head osteonecrosis. Methods In this study, 23 patients (39 affected hip joints) with osteonecrosis of the femoral head with stage II of the disease, based on the Ficat-Arlet classification system, underwent core decompression. Also, patients demographic characteristics, clinical data, medication history, comorbidities, immunological findings, hip plain radiographs, history of total hip arthroplasty after core decompression, and patients satisfaction with joint function according to the Oxford hip score questionnaire were obtained. Results In the study, 53.8% of affected joints showed signs of radiographic deterioration in follow-up imaging. Sixty-one and a half percent (61.5%) of patients had unsatisfactory joint performance. A third (33.3%) of affected hip joints underwent total hip arthroplasty up to 5 years from core decompression. SLE patients with a history of receiving bisphosphonate were 83.2% less dissatisfied with their joint function than patients without a history of bisphospho-nate use (P < 0.02). Of the 23 studied cases, the mean cumulative dose of prednisolone before and after core decompression surgery was 46.41 mg and 14.74 mg respectively. Besides, one case (2.6%) that had a high anti-phospholipid antibodies level during follow-up did not have any radiographic deterioration, and 9 cases (23.1%) had some degrees of radiographic deterioration. Conclusions The patients group that used bis-phosphonate, had a higher level of satisfaction with joint function after core decompression. Patients with high-level anti-phospholipid antibodies are related to a poor prognosis after core decompression.

Approach to the primary adrenal insufficiency caused by anti-phospholipid syndrome / 中华内分泌代谢杂志

The causes of primary adrenal insufficiency(PAI) are varying, however, anti-phospholipid syndrome (APS) is a relatively rare one. PAI lacks unique clinical manifestations, so the confirmation of PAI was easily to be neglected by physicians. We report a case with abdominal pain as the first complaint, followed by multiple infections, thrombotic events, and aggravating fatigue. Through a series of the laboratory examination, medical imaging, and pathology examination, this patient was diagnosed as PAI caused by APS. Combining this report with literature review, we aim to raise awareness of the disease and avoid misdiagnosis and missed diagnosis.

Manifestaciones relacionadas con alteraciones en la coagulación en lupus eritematoso sistémico de inicio juvenil. Reporte de casos y revisión de la literatura / Manifestations related to alterations in coagulation in systemic lupus erythematosus of juvenile onset. Case report and literature review

RESUMEN Las alteraciones hematológicas son comunes en los pacientes con lupus eritematoso sistémico (LES). Pueden expresarse relacionadas con el compromiso de las líneas celulares y con la presencia de alteraciones de la coagulación. El compromiso en la coagulación se asocia con manifestaciones trombóticas. Se han descrito factores de riesgo asociados a trombosis, como la presencia de niveles elevados de homocisteína, déficit adquirido de la proteína S, proteína C y antitrombina. Sin embargo, la diátesis hemorrágica también se ha descrito con menor frecuencia y relacionada con el déficit de factores de la coagulación, secundaria a la presencia de inhibidores. Presentamos 3 pacientes con LES juvenil con manifestaciones hematológicas poco usuales y revisión de la literatura relacionada. Se concluye que las manifestaciones hematológicas en LES juvenil no solo se relacionan con alteraciones en las líneas celulares. Trombosis vasculares y trastornos hemorrágicos deben sospecharse. El diagnóstico precoz y el tratamiento temprano disminuyen la morbimortalidad relacionada con este tipo de manifestaciones.

ABSTRACT Haematological alterations are common in patients with systemic lupus erythematosus (SLE). These haematological manifestations may be expressed related to the involvement of cells affected and coagulation changes. The compromise in coagulation is associated with thrombotic manifestations. Risk factors associated with thrombosis have been described, such as the presence of elevated levels of homocysteine, acquired deficit of protein S, protein C, and antithrombin. However, the haemorrhagic diathesis has also been described at a lower frequency and related to the acquired deficiency of coagulation factors caused by the development of autoantibodies directed against coagulation factors. The cases are presented of 3 patients with juvenile SLE with unusual haematological manifestations, as well as a review of the literature in relation to them. The haematological manifestations in juvenile SLE are not only related to alterations in cell lines, vascular thrombosis and bleeding disorders should also be suspected. Early diagnosis and treatment reduces morbidity and mortality related to this type of manifestations.

A brief history of antiphospholipid antibodies and antiphospholipid syndrome / Uma breve história dos anticorpos antifosfolípides e da síndrome antifosfolípide

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.

Central retinal arterial occlusion in a patient with pyoderma gangrenosum

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.

Assessment of Antiphospholipid Antibodies, CD4 Count and Some Haematological Parameters in HIV Patients attending a Tertiary Health Institution in South-Western Nigeria

Background: Antiphospholipid antibodies (aPLs) are the serological markers used in the diagnosis of the antiphospholipid syndrome (APS). HIV infection has been associated with an elevated aPls level, but its link to the APS with clinical thrombosis is still been investigated. This study is designed to determine and correlate serum level of antiphospholipid antibodies with CD4 count and some haematological parameters of HIV seropositive subjects in comparison to those of healthy controls and also to compare these parameters between antiretroviral therapy (ART) naïve and treated patients. Methodology: A cohort of 110 patients which consist of 90 HIV positive Patients (22 males and 68 females) and 20 HIV negative patients (10 males and 10 females) which serve as control attending Babcock University Teaching Hospital (BUTH) Ilishan-Remo, Ogun State, Nigeria were recruited for the cross-sectional study. HIV antibodies were detected using 3 rapid diagnostic kits (Determine, Unigold and Stat Pak). CD4+ cells were counted using Partec® Cyflow Counter (Germany). The Full Blood Count was analyzed using the Sysmex® Automated Haematology Analyzer (Kobe-Japan). Antiphospholipid antibodies (aPLs) were assayed using the Human Anti-Phospholipid Screen IgG/IgM ELISA kit (Alpha Diagnostic International, Texas, USA). Results: The present study showed that the mean serum antiphospholipid antibody level was significantly (P<0.001) higher in HIV positive Patients (11.83±7.36u/ml) compared to the control group (7.30±3.95u/ml). While on one hand, there was a strong positive correlation between serum aPLs level and PLT (r= 0.044), MCHC (r= 0.084) and LYM (r= 0.105) in HIV infection; on the other hand, there was a strong negative correlation with CD4 count (r= -0.094), PCV (r= -0.099), Hb (r= -0.072), RBC (r= -0.003), WBC (r= -0.063), MNO (r= -0.213), GRA (r= -0.003), MCV (r= -0.023) and MCH (r= -0.005). Also, there was no significant differences (P>0.05) between the aPLs level of HIV group on ART (11.44±7.74 u/ml) and those not on ART (12.00±7.24 u/ml). Some haematological parameters like PLT, PCV, Hb, RBC and red cell indices of the HIV group on ART did not differ significantly from those not on ART. However, the CD4 count (638.89±119.56 cell/?L), WBC (5.38±1.49X103/?L), LYM (51.43±7.99%) and GRA (46.30±10.18%) of the HIV group on ART were significant higher than those not on ART (465.30±145.92 cell/?L, 4.55±1.57X103/?L, 42.23±10.96% and 39.10±7.81%, respectively). Conclusion: Significant elevated aPLs level is present in HIV infection; however, the information obtained is not sufficient to indicate the occurrence of anti-phospholipid syndrome in HIV infection. There was no strong relationship between aPLs level and indicators of immunohaematological abnormalities in HIV infection. This finding is plausible and would therefore require further investigation.

Systemic Lupus Erythematosus Presenting as Monocular Elevation Deficiency

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.

Childhood Lupus with Microangiopathic Antiphospholipid Syndrome and Pulmonary Hemorrhage.

Background: Concurrent thrombotic and hemorrhagic manifestations are uncommon in patients with Antiphospholipid Syndrome. Case characteristics: A 10-year-old girl with fever, edema, rash and joint pains, who later developed deep venous thrombosis (DVT), stroke, thrombotic microangiopathic hemolytic anemia and pulmonary hemorrhage. Investigations confirmed Antiphospholipid syndrome associated with systemic lupus erythematosus. Outcome: She went into complete remission with intravenous immunoglobulins, plasmapheresis, immunosuppression and anticoagulation. Message: Thrombotic microangiopathic hemolytic anemia and anti-phospholipid syndrome can be the presenting manifestations of systemic lupus erythematosus..

Changes of Treg/Th17 in mice of β2 glycoprotein 1 induced experimental anti-phospholipid syndrome / 中国免疫学杂志

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.

Refractory Mycoplasma pneumoniae pneumonia combined with thrombosis：a case report and literature review / 临床儿科杂志

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.

A Case of Primary Anti-Phospholipid Syndrome with Recurrent Renal Infarction / 대한내과학회지

Anti-phospholipid syndrome is characterized by the occurrence of venous or arterial thrombosis in the presence of anti-phospholipid antibodies and is associated with morbidity during pregnancy. Arterial thromboses are less common than venous thromboses and most frequently manifest with features consistent with ischemia or infarction. Only a few cases of arterial thrombosis with primary anti-phospholipid syndrome have been reported in Korea. We report a 41-year-old man with anti-phospholipid syndrome who had abdominal pain due to a recurrent renal infarction, and we review the literature on anti-phospholipid syndrome.

Ascending Aortic Replacement for Acute Type A Aortic Dissection in a Patient with Anti-phospholipid Antibody Syndrome / 日本心臓血管外科学会雑誌

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.

Preliminary approach on application of recombinant human 2-glycoprotein-1 domain Ⅰ dimer as B lymphocyte tolerogen / 吉林大学学报(医学版)

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

Aterosclerose na artrite reumatóide e sua associação com auto-imunidade humoral / Atherosclerosis in rheumatoid arthritis and its relationship with humoral autoimmunity

Objetivos: Muitas questões permanecem sobre as causas da aterosclerose acelerada nos pacientes com doenças inflamatórias sistêmicas como a artrite reumatóide (AR). Estudos na população geral sugeriram que além da inflamação existe uma participação patogênica da auto-imunidade na aterosclerose e discutem a possível associação dos anticorpos contra fosfolípides e proteínas de choque térmico (Hsp). O objetivo deste estudo foi investigar a presença de anticorpos contra fosfolípides, beta2-glicoproteína 1 (beta2-gp1), lipoproteína lipase (LPL) e Hsp em pacientes com AR e avaliar a associação entre estes anticorpos com a presença de aterosclerose subclínica de carótidas. Métodos: Anticorpos contra cardiolipina (aCL) IgG e IgM, beta2-gp1 IgG, IgM e IgA , Hsp 60 e Hsp 65 foram testados por ELISA em um grupo de 71 pacientes com AR comparado com 53 indívíduos controles não portadores de AR, de idade e sexo similar. Foram excluídos os pacientes com HAS, diabetes melitos e os fumantes em ambos os grupos. Níveis de lipoproteínas, parâmetros clínicos da AR, questionário de avaliação de saúde (HAQ), escore de atividade da doença (DAS) 28, velocidade de hemossedimentação (VHS) e proteína C reativa (PCR) foram avaliadas. A associação entre a presença dos anticorpos aCL, beta2-gp1, Hsp 60 e Hsp 65 com os parâmetros clínicos de atividade da doença, com a presença das placas de aterosclerose e com a medida da espessura íntimomedial (IMT) da carótida comum, usando ultra-som (US) modo B de alta resolução foram pesquisadas. Resultados: A idade média no grupo com AR foi 48,93 ± 12,31 vs. 45,37 ± 9,37 no grupo controle saudável (p = 0,20); 90,1% no grupo com AR eram do sexo feminino vs. 86,8% no grupo controle (p = 0,56); índice de massa corporal (IMC) foi 25,72 ± 4,57kg/m² no grupo com AR vs. 26,40 ± 4,52kg/m² no grupo controle (p = 0, 69); Os níveis de colesterol, LDL, triglicerídeos e a relação CT/HDL não foram diferentes quando comparamos os 2 grupos (p > 0,05)...

Purpose: Many questions remain unanswered about the causes of accelerated atherosclerosis in patients with inflammatory systemic diseases such as rheumatoid arthritis (RA). Some studies have suggested the role of autoimmunity besides inflammation in the pathogenesis of atherosclerosis in general population and have also discussed the possible association with antibodies directed to phospholipids and heat shock proteins (Hsp). The aim of this study was to investigate the presence of antibodies against phospholipids, beta2-glycoprotein1 (beta2-gp1), lipoprotein lipase (LPL) and Hsp in RA subjects and evaluate the association between these antibodies with the presence of subclinical carotid atherosclerosis. Methods: Tests to antibodies against cardiolipin (aCL) IgG and IgM, beta2-gp1 IgG, IgM and IgA ,Hsp 60 and Hsp 65 were done by ELISA test in a group of 71 RA subjects compared with 53 age and sex-matched non-RA subjects. Smoking, diabetic and hypertensive patients were excluded in both groups. The lipoprotein levels, clinical parameters of RA, Health Assessment Questionnaire (HAQ), Disease Activity Score (DAS) 28, Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein (CRP) were evaluated. The association between the presence of antibodies against cardiolipin, beta2-gp1 and Hsp 60 and 65 with the clinical parameters of disease activity in RA, and with the presence of plaques and mean intimo-medial thickness (IMT) of common carotid using high-resolution B-mode ultrasound were assessed. Results: Mean age in RA group was 48.93 ± 12.31 vs. 45.37 ± 9.37 in healthy control group (p = 0.20); 90.1% were women in RA group vs. 86.8% in healthy control (p = 0.56); body mass index (BMI) were 25.72 ± 4.57 in RA group vs. 26.40 ± 4.52 in healthy control (p = 0.69). The levels of cholesterol, LDL, triglycerides, CT/HDL didn t have difference between the two groups (p > 0.05)...

Correlation Between Homocysteine and Anti-Phospholipid Antibodies and the Formation and Recurrence of Thrombus in Deep Vein / 中国普外基础与临床杂志

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