Lupus cystitis and performation of the small bowel in a patient with systemic lupus erythematosus and overlapping syndrome

We describe a patient with systemic lupus erythematosus (SLE) and overlapping syndrome who had repeated gastrointestinal (GI) symptoms such as nausea, vomiting and malabsorption. With a subacute process, she developed paralytic ileus and contracted bladder, and died of perforation of the ileum. When 13 reported cases with lupus cystitis were reviewed, an extremely strong correlation between GI and urinary tract symptoms was observed, indicating the presence of a unique subgroup of SLE.     

Management of Evans syndrome

Evans syndrome is an uncommon condition defined by the combination (either simultaneously or sequentially) of immune thrombocytopenia (ITP) and autoimmune haemolytic anaemia (AIHA) with a positive direct antiglobulin test (DAT) in the absence of known underlying aetiology. This condition generally runs a chronic course and is characterised by frequent exacerbations and remissions. First-line therapy is usually corticosteroids and/or intravenous immunoglobulin, to which most patients respond; however, relapse is frequent. Options for second-line therapy include immunosuppressive drugs, especially ciclosporin or mycophenolate mofetil; vincristine; danazol or a combination of these agents. More recently a small number of patients have been treated with rituximab, which induces remission in the majority although such responses are often sustained for <12 months and the long-term effects in children are unclear. Splenectomy may also be considered although long-term remissions are less frequent than in uncomplicated ITP. For very severe and refractory cases stem cell transplantation (SCT) offers the only chance of long-term cure. The limited data available suggest that allogeneic SCT may be superior to autologous SCT but both carry risks of severe morbidity and of transplant-related mortality. Cure following reduced-intensity conditioning has now been reported and should be considered for younger patients in the context of controlled clinical trials.     

系统性红斑狼疮合并Evans综合征临床特点

目的总结系统性红斑狼疮(systemic lupus erythematosus,SLE)合并Evans综合征患者的临床特点。方法回顾性分析北京协和医院2004年1月至2012年7月SLE合并Evans患者的临床表现及实验室特点及治疗和预后。结果 SLE并发Evans综合征患者22例,占同期SLE住院患者3400例的0.65%。其中男3例,女19例,平均35.1岁(16~53岁)。22例患者中以血液系统受累[特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)或自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)]为首发表现的11例(50%),确诊SLE后诊断Evans综合征者6例,二者同时诊断的5例。SLE并发Evans综合征时,患者往往有多系统受累,表现为肾脏受累13例(59.1%),皮肤黏膜受累、关节炎各9例(40.9%),神经系统受累4例(18.2%),胃肠道、肺部受累各2例等。Evans综合征多发生于SLE活动期,患者平均狼疮活动指数评分(11.45±7.6)分(3~30分)。伴发其他结缔组织病5例(22.7%)。经激素联合免疫抑制剂治疗后,20例好转,2例无效者应用利妥昔单抗后好转。结论 SLE合并Evans综合征罕见,发生于SLE多系统受累及活动期。部分患者以ITP或AIHA为SLE首发表现,应及时筛查多种自身抗体,并定期随访密切观察,以期早期诊治。     

Myelodysplastic syndrome accompanied by Evans syndrome

Various cases of myelodysplastic syndrome (MDS) with diverse immunological disorders have been reported by many investigators. In this case report, we present a 70-year-old woman who had been diagnosed as having MDS and liver cirrhosis (LC) type C for 20 months, and who finally developed autoimmune phenomena against autologous blood cells. She was admitted to our hospital in order to evaluate her advanced anemia, thrombocytopenia and fatigue. The laboratory data at admission were as follows: hemoglobin 5.3 g/dl, red blood cell count 109 x 10(4)/microliter, white blood cell count 1,760/microliter, platelet count 4.3 x 10(4)/microliter and reticulocyte count 1.3%. The direct Coombs test was positive. In addition, anti-platelet antibody was positive, using the MPHA method. With these results, Evans syndrome secondary to MDS or LC was diagnosed. The mechanisms for the development of immunological disorders in patients with MDS or LC have not been fully elucidated. To our knowledge, this patient is one of the rare cases with MDS and Evans syndrome reported in the literature. During the generation process of autoantibodies, the role of HCV antigen in the pathogenesis of Evans syndrome was of interest in this patient.     

Lupus cystitis in association with severe gastrointestinal manifestations in an adolescent

Lupus cystitis is a rare interstitial inflammatory disease of the bladder seen in systemic lupus erythematosus that usually occurs in association with gastrointestinal manifestations, and occasionally with central nervous system involvement. There are few reported pediatric cases. We describe this entity as the presenting manifestations of lupus in an adolescent female.     

Lupus cystitis: a possible additive risk factor for emphysematous cystitis in diabetes mellitus: discussion about one case

Emphysematous cystitis (EC) is a rare condition in which gas-forming organisms are active in the bladder wall and lumen. Most of the cases have been described in patients suffering from diabetes mellitus due to glucosuria and subsequent anaerobic fermentation of glucose. To our knowledge this condition has never been described in association with systemic lupus erythematosus (SLE). We report here the first case of EC during the course of a chronic lupus cystitis (LC) in a woman suffering from SLE and type-I diabetes mellitus.     

Basedow disease associated with Evans syndrome

A 60-year-old woman was admitted to a hospital complaining of dizziness and general fatigue in October, 2004. Because of heart failure and severe anemia, she was referred to our hospital. Based on a positive direct Coombs test and an elevated level of platelet-associated IgG (PAIgG), the patient was diagnosed as having autoimmune hemolytic anemia (AIHA) associated with idiopathic thrombocytopenic purpura (ITP), i.e., Evans syndrome. Basedow disease was also diagnosed due to hyperthyroidism with an elevation of anti-thyroid stimulating hormone (TSH) receptor antibodies. Both the Evans syndrome and Basedow disease were considerably ameliorated with plasma exchange, corticosteroid and thiamazole therapy. Although Basedow disease is known to be associated with hematological disorders such as AIHA or ITP, the combination of Basedow disease and Evans syndrome is rare. We report here a case of Basedow disease associated with Evans syndrome.     

Evans syndrome and primary biliary cirrhosis

Primary biliary cirrhosis associated with autoimmune haemolytic anaemia and thrombocytopaenia has rarely been reported in adults. We are presenting an 83-year-old woman with primary biliary cirrhosis who was also diagnosed with autoimmune haemolytic anaemia and idiopathic thrombocytopaenic purpura.     

Rituximab therapy for childhood Evans syndrome

The safety and efficacy of rituximab have been retrospectively assessed in 17 children with Evans syndrome. Patients received 4 or 3 weekly doses of rituximab (375 mg/m(2) per dose) associated with prednisone, alone (14 patients) or associated with other immunosuppressive drugs. Complete or partial remission of at least one cytopenia was achieved in 13 out of the 17 patients (76%), and lasted in 11 of them with a mean follow-up of 2.4 years (range 0.5-7 years). Steroid therapy was stopped or tapered at 50-100% of the baseline dosage in all long-term responders. Moderate side effects and infection occurred only in 4 and 1 children respectively.     

The Evans syndrome: characterization of the responsible autoantibodies

The immunofluorescence test on paraformaldehyde-fixed cells was used for the detection of antibodies bound to the platelets and granulocytes and present in the sera of 24 patients with Evans syndrome and a further 29 patients with both idiopathic thrombocytopenia (ITP) and idiopathic neutropenia (INP), but without autoimmune haemolytic anaemia (AIHA). The direct immunofluorescence test on platelets and/or on granulocytes was positive in all patients with a cytopenia, but the sera of only 17 patients with the Evans syndrome and 15 of the other patients contained platelet- or granulocyte-specific autoantibodies.
From absorption and elution experiments, it appeared that the autoantibodies were directed against antigens specific for the various peripheral blood cells, i.e. erythrocytes, platelets and granulocytes and that they were not cross-reacting.     

IgA anti-red blood cell auto-antibodies in Evans syndrome

In Evans syndrome, IgG auto-antibodies (Abs) and/or complement components are frequently detected on red blood cells (RBC) in the direct antiglobulin test (DAT). A 70-year-old man with Evans syndrome diagnosed four years previously presented with a persistent autoimmune haemolytic anaemia, despite immunosuppressive treatment and normalization of platelet count. The RBC allo- and auto-Abs screening and identification were performed by indirect antiglobulin test (IAT) and DAT. In March 2006, no circulating anti-RBC auto-Abs were found in IAT but the DAT was positive with anti-IgG (++),-C3d (weak) and -IgA (++). Follow up for 11 months revealed anti-RBC IgA auto-Abs on five out of six samples. IAT was positive for RBC auto-Abs on three samples. No correlation between the haemoglobin level and the strength of reactivity of IgG and IgA auto-Abs was observed. IgA anti-RBC auto-Abs are present in Evans syndrome. To detect these Abs and characterize their role, DAT procedures should systematically include anti-IgA.     

IgA anti-red blood cell auto-antibodies in Evans syndrome

In Evans syndrome, IgG auto-antibodies (Abs) and/or complement components are frequently detected on red blood cells (RBC) in the direct antiglobulin test (DAT). A 70-year-old man with Evans syndrome diagnosed four years previously presented with a persistent autoimmune haemolytic anaemia, despite immunosuppressive treatment and normalization of platelet count. The RBC allo- and auto-Abs screening and identification were performed by indirect antiglobulin test (IAT) and DAT. In March 2006, no circulating anti-RBC auto-Abs were found in IAT but the DAT was positive with anti-IgG (++), -C3d (weak) and -IgA (++). Follow up for 11 months revealed anti-RBC IgA auto-Abs on five out of six samples. IAT was positive for RBC auto-Abs on three samples. No correlation between the haemoglobin level and the strength of reactivity of IgG and IgA auto-Abs was observed. IgA anti-RBC auto-Abs are present in Evans syndrome. To detect these Abs and characterize their role, DAT procedures should systematically include anti-IgA.