Immunotherapies application in active stage of systemic lupus erythematosus in pregnancy: A case report and review of literature

BACKGROUNDPregnancy in the setting of systemic lupus erythematosus can worsen the condition from the stable to active stage, with quality of life and fertility desire being particular concerns. Pregnancy in the active stage of systemic lupus erythematosus (ASLE), although rare and complicated to manage, can be treated favorably with immunotherapies ifs used properly. Here we report such a success case.CASE SUMMARYA 31-year-old primigravida patient, diagnosed with SLE seven years ago, was induced ASLE after a cold at 21 + weeks. The patient’s vital signs on presentation were normal. Her laboratory exam was remarkable for significant proteinuria, liver and renal dysfunction, and low C3 and C4 levels. Infectious work-up was negative. The patient was diagnosed with ASLE. She was given immunosuppressive agents (methylprednisolone, gamma globulin and azathioprine etc.) and plasma adsorption therapy, monitoring blood pressure every 8 h, fetal heart rate twice a day, and liver and renal function at least twice a week. Successful maternal and fetal outcomes are presented here.CONCLUSIONChild-bearing in ASLE has become more promising, even for this difficult case of ASLE with multiple organ damage. Thorough antepartum counseling, cautious maternal-fetal monitoring, and multi-organ function monitoring by multidisciplinary specialties are keys to favorable pregnancy outcomes.     

以肺部表现首发的系统性红斑狼疮4例及文献复习

目的：研究系统性红班狼疮(SLE)首发肺部表现的临床特征及诊治方法。方法：回顾分析2002年10月～2004年4月4例SLE首发肺部表现患者的临床资料，并复习相关文献。结果：4例病人，女性3例，男性1例，年龄29～52岁，从肺部首发症状到出现其它系统症状时间为1周～1年，所有病例均符合美国风湿病学会1982年制订的SLE诊断标准。X线检查示两下肺间质纤维化1例，双侧胸腔积液伴心包积液1例，间质性肺炎1例，狼疮性肺炎1例。肾活检病理为Ⅱ型狼疮性肾炎2例，Ⅲ型1例，Ⅳ型1例。经甲基泼尼松龙及环磷酰胺等治疗，24h尿蛋白定量均较人院时下降50％以上，复查X线胸片示1例间质纤维化无变化，另3例病灶均有吸收。结论：以肺部症状为首发表现病例尤其是间质炎症或纤维化为主者，必须仔细除外SLE，不能简单地以抗感染治疗处理，需密切随访；必须强调急性狼疮性肺炎早期的正确诊断和治疗。     

多靶点治疗难治性肾小球疾病临床观察

目的 观察激素加霉酚酸酯(mycophenolate mofetil,MMF)和他克莫司(taerolimus,FK506)的多靶点方案治疗难治性肾小球疾病的疗效及安全性.方法 2008年5月-2010年3月收治的15例狼疮性肾炎(lupus nephritis,LN)、3例膜增生性肾小球肾炎(membranoprol...     

系统性红斑狼疮患者超声心动图改变的研究

本文采用二维多普勒超声心动图（超声心动图）对１２９例系统性红斑狼疮（ＳＬＥ）患者进行前瞻性研究。有超声心动图异常的７９例（６１．２４％）。其中瓣膜异常５２例，８２．６％（４３／５２）的瓣膜异常患者无临床症状及血液动力学改变；心包积液３６例，其中轻度２３例无血液动力学改变；心肌异常２４例。兼有不同程度的瓣膜、心肌或心包病变者３４。有抗磷脂抗体的患者心瓣膜损害的发病率较高（３３／４９），提示这种抗体在ＳＬＥ患者心瓣膜病变的发病机制中可能有突出促进作用     

Systemic lupus erythematosus combined with primary hyperfibrinolysis and protein C and protein S deficiency: A case report

BACKGROUNDSystemic lupus erythematosus (SLE) is an autoimmune disease characterized by systemic involvement and multiple autoantibodies in the serum. Patients with protein C (PC) and protein S (PS) deficiency are prone to thrombosis. In contrast, patients with primary hyperfibrino-lysis tend to bleed.CASE SUMMARYA 52-year-old female patient with bilateral pleural effusion was diagnosed with "tuberculous pleurisy" and treated with anti-tuberculosis drugs and prednisone. The coagulation-related laboratory results showed decreased fibrinogen, PC activity, PS activity, and antithrombin Ш activity. The immune-related laboratory results showed positive antinuclear antibody, anti-Smith antibody, anticardiolipin antibody (ACL), anti-β2-glycoprotein I antibody (aβ2GPI) and direct Coomb’s test and decreased complement 3 and complement 4. Thoracoscopy was performed and bloody pleural fluid was drained. Pathology of the pleural biopsy showed lymphocytes, plasma cells, and a few eosinophils in adipose and fibrous connective tissue. Results of whole exome sequencing of blood showed no genetic mutations suggesting the presence of hereditary hematological diseases. The patient was finally diagnosed with SLE and primary hyperfibrinolysis, and was treated with prednisolone, hydroxychloroquine, and compound cyclophosphamide. CONCLUSIONPC and PS deficiency in SLE might be related to ACL and aβ2GPI. SLE and primary hyperfibrinolysis can coexist in one patient, with both a risk of thrombosis and a risk of bleeding.     

Systemic lupus erythematosus and antineutrophil cytoplasmic antibody-associated vasculitis overlap syndrome in a 77-year-old man: A case report

BACKGROUNDSystemic lupus erythematosus (SLE) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV) are classically thought to cause renal impairment and small vessel vasculitis with different pathophysiologies. Their overlap constitutes a rare rheumatologic disease. To date, only dozens of such cases with biopsy-proven glomerulonephritis have been reported worldwide typically in women of childbearing age. Here, we present a unique clinical case due to its rarity and individualized treatment of a Chinese man in his eighth decade of life.CASE SUMMARYA 77-year-old man was admitted to several hospitals for shortness of breath and received nonspecific treatments over the past 3 years. As his symptoms were not completely relieved, he visited our hospital for further treatment. Laboratory examinations revealed kidney dysfunction, severe anaemia, hypocom-plementemia, glomerular proteinuria, and microscopic haematuria. Antinuclear antibodies, as well as anti-dsDNA antibodies, were positive. Computed tomography of the chest showed right pleural effusion. Renal biopsy was performed, and histology suggested crescentic glomerulonephritis, pauci-immune type. After treatment with plasmapheresis, glucocorticoid, and cyclo-phosphamide, the disease was in remission, and the patient remained in a stable condition for over 3 years post-hospital discharge.CONCLUSIONDue to its complexity and rarity, SLE and AAV overlap syndrome is easily misdiagnosed. An accurate diagnosis and treatment at the earliest stage may significantly improve the condition and reduce irreversible organ injury.