Pulmonary haemorrhage and mesenteric vasculitis in a patient with systemic lupus erythematosus

WE Report a 26‐year‐old female patient with systemic lupus erythematosus (SLE) who developed mesenteric vasculitis and pulmonary haemorrhage. This patient initially presented with an acute abdomen and extensive vasculitic rash. While she was being treated for her abdomen she developed fulminant pulmonary haemorrhage requiring mechanical ventilation. With supportive measures and aggressive immunosupression treatment she eventually made a complete recovery.     

Another side of lupus: A case report of lupus presenting with cutaneous vasculitis

Systemic lupus erythematosus (SLE) is a systemic autoimmune complex disease that affects any organ, characterized by immune complex formation and autoantibody production. Lupus vasculitis begins at a young age. These patients generally have a longer disease duration. Ninety percent of cases in lupus-associated vasculitis present with cutaneous vasculitis. Disease activity, severity, organ involvement, response to treatment and drug toxicity determine the frequency of outpatient control in lupus. Depression and anxiety are observed more frequently in SLE than in the normal population. Our case, it is an example of the patient's disruption of controls due to psychological trauma and that lupus can cause serious cutaneous vasculitis. In addition, psychiatric evaluation of lupus cases from the time of diagnosis may have a positive effect on the prognosis.     

Recurrent fractures in an elderly patient with systemic lupus erythematosus

Glucocorticoid‐induced osteoporosis (GIO) is an important problem that remains undertreated, even by rheumatologists. We present a case of an elderly patient with systemic lupus erythematosus diagnosed more than 40 years ago, who suffered from recurrent fractures and attendant complications despite a bone mineral density (BMD) score in the osteopenic range and treatment with bisphosphonates. With improved treatment and outcome of lupus, an increasing number of elderly patients who are susceptible to osteoporotic fractures are expected. This case serves to highlight that rheumatic disease patients on steroids should be screened for GIO, as effective treatment and preventive measures are available. Teriparatide is a promising treatment for patients who have failed bisphosphonate treatment or who are at high risk for fracture. We should also bear in mind that BMD scores alone are not indicative of fracture risk, and other tools such as the WHO‐FRAX (Fracture risk assessment tool), serum vitamin D3 levels and bone turnover markers should be used where appropriate. Other measures including attention to factors that contribute to falls should also be considered, necessitating a multi‐disciplinary approach.     

Two UV-induced episodes of myelitis in a patient with systemic lupus erythematosus.

Two flares of acute myelitis were observed in a 31-year-old woman with previously known mild SLE. The two myelitic episodes both occurred within 1 week after intense sun exposure, and for this reason photobiological induction of the manifestation is considered likely. The diagnostic utility of MRI in the acute situation and the possible influence of sex-steroids on SLE manifestations are also illustrated by this case.     

Massive hepatic infarction in systemic lupus erythematosus

Summary Liver disease in systemic lupus erythematosus, as demonstrated by abnormal histopathology, is rare and usually mild; typically, this hepatic disease is of chronic nature and not related to a hypercoagulable state. A patient is described in whom life-threatening hypercoagulability in association with systemic lupus erythematosus resulted in extensive liver infarction. Follow-up radionuclide liver scintigraphy suggested that regenerative recovery in the infarcted areas of the liver may be delayed or absent, but there was no evident functional hepatic impairment.     

Simultaneous presentation of hemophagocytic syndrome and mesenteric vasculitis in a patient with systemic lupus erythematosus

Abstract

We report an 18-year old female patient with systemic lupus erythematosus (SLE), who developed fever, pancytopenia, abdominal pain, and watery diarrhea. Computed tomography (CT) and bone marrow aspirate revealed lupus mesenteric vasculitis (LMV) and hemophagocytic syndrome (HPS). Serologic tests for Epstein–Barr virus (EBV) indicated its reactivation. This case demonstrates that HPS and concomitant LMV associated with viral reactivation can occur as clinical manifestations of SLE flare.     

免疫球蛋白G在小鼠系统性红斑狼疮肝损害发生中的作用

[目的]探讨免疫球蛋白G(IgG)在系统性红斑狼疮肝损害发生中的作用。[方法]利用空肠弯曲菌诱导建立系统性红斑狼疮小鼠模型18只,设为A组;用乙肝病毒感染造成肝损害的小鼠模型18只,设为B组;取正常对照小鼠18只,设为C组。检测各组各项肝功能指标,利用免疫荧光技术检测IgG在肝脏的沉积情况,同时检测循环中IgG的含量,比较上述各组在肝功能及肝脏IgG沉积方面的不同。[结果]A、B组肝功能各项指标均明显高于C组,但A、B组之间比较差异无统计学意义(P>0.05),A组小鼠肝脏IgG沉积及循环IgG含量均高于B、C组(P<0.05)。[结论]IgG免疫复合物的沉积在系统性红斑狼疮肝损害的发展中可能扮演了重要的角色。     

Infections in patients with systemic lupus erythematosus

Infection is a major contributor to morbidity and mortality in patients with systemic lupus erythematosus (SLE). In most clinical series, infection ranks first or second as the most common cause of death in SLE patients worldwide, including Hong Kong. In this article, the spectrum of infections and their protean manifestations in lupus patients will be reviewed with emphasis on clinical data from Hong Kong and other Asian countries. A high index of suspicion and dedicated work‐up to identify the causative pathogens is pivotal to the early diagnosis and effective management of infective complications in patients with SLE.     

Microvascular involvement in systemic sclerosis and systemic lupus erythematosus

Microvascular changes play central roles in the pathophysiology of SSc and SLE, and represent major causes of morbidity and mortality in these patients. Therefore, clinical tools that can assess the microvasculature are of great importance both at the time of diagnosis and follow‐up of these cases. These tools include capillaroscopy, laser imaging techniques, infrared thermography, and iontophoresis. In this review, we examined the clinical manifestations and pathobiology of microvascular involvement in SSc and SLE as well as the methodologies used to evaluate the microvasculature.     

Anorexia nervosa in a patient with systemic lupus erythematosus

Summary Anorexia nervosa has not been described in patients with systemic lupus erythematosus (SLE). The case of a patient with SLE who developed the classical features of anorexia nervosa is described. Although an impressive interplay between psychosocial and cultural factors was present, the possibility that this syndrome was produced by active CNS vasculitis remained.     

Development of systemic lupus erythematosus in a patient with hypoparathyroidism: a case report and review of the literature

Systemic lupus erythematosus (SLE) is an autoimmune disease in which organs undergo damage. Hypoparathyroidism is a rare disease, which presents in two forms: hereditary and acquired. Cases of hypoparathyroidism and SLE rarely co‐exist. Only six cases have been reported; five of them first presented with lupus and then hypoparathyroidism or simultaneously. We present here developing lupus disease in a woman who had idiopathic hypoparathyroidism. According to increasing data about the autoimmune origin of idiopathic hypoparathyroidism, these case reports suggest that there may be an autoimmune process linking these diseases.     

系统性红斑狼疮脑病36例临床分析

目的研究系统性红斑狼疮脑病患者临床表现特点。方法分析、归纳2000-01-01~2004-10-10中日友好医院神经内科36例系统性红斑狼疮脑病患者症状、体征、辅助检查。结果36例系统性红斑狼疮脑病患者主诉症状9类,其中头痛、意识障碍、肢体无力居发生率前3位;发现体征13类,其中病理反射、意识障碍、肢体瘫痪居前3位。结论(1)系统性红斑狼疮脑病是活动性系统性红斑狼疮的表现之一。(2)系统性红斑狼疮患者无论病史长短,均可发生系统性红斑狼疮脑病。(3)系统性红斑狼疮脑病表现复杂多样。     

Visceral leishmaniasis in a patient with systemic lupus erythematosus: Dilemma in diagnosis and management

Patients with systemic lupus erythemasus (SLE) have an increased risk of bacterial, viral, fungal or parasitic infections, especially if they are receiving immunosuppressive therapy. Leishmaniasis is a group of diseases caused by intracellular flagellate protozoan parasites belonging to the genus Leishmania. We present a 48-year-old female patient, diagnosed with SLE many years ago, who presented with high fever and pancytopenia. We thought that the patient's hematologic findings were related to SLE hematologic involvement. However, we investigated other possible causes when there was no response to drugs for the treatment of SLE. A second bone marrow biopsy showed Leishmania amastigotes and the patient was diagnosed with leishmaniasis. The patient was treated with liposomal amphotericin-B (treatment completed at 40 days). She showed rapid clinical improvement and showed no signs of disease after 4 months.     

静脉用免疫球蛋白在系统性红斑狼疮治疗中的应用

静脉用免疫球蛋白(IVIg)主要通过Fc受体介导免疫调节作用治疗系统性红斑狼疮(SLE),其他机制包括抑制补体介导的损伤,调控细胞因子的产生;调控独特型抗体网络,中和病理性自身抗体;调节B细胞和T细胞功能,下调抗体的产生等。IVIg已成功治疗SLE相关的顽固性血小板减少症、中枢神经系统狼疮、狼疮性肾炎及对传统免疫治疗无效或同时合并感染的SLE。     

Chronic intestinal pseudo-obstruction in systemic lupus erythematosus

Background/Aims—Chronicintestinal pseudo-obstruction (CIPO) reflects a dysfunction of thevisceral smooth muscle or the enteric nervous system. Gastrointestinalmanifestations are common in systemic lupus erythematosus (SLE)but CIPO has not been reported. Features of CIPO are reported in fivepatients with SLE.
Methods—From 1988 to1993, five patients with SLE or SLE-like syndrome were hospitalised forgastrointestinal manometric studies. CIPO was the onset feature in twocases. Antroduodenal manometry (three hours fasting, two hours fed) wasperformed in all patients, and oesophageal manometry in four.
Results—Intestinalhypomotility associated with reduced bladder capacity and bilateralureteral distension was found in four patients and aperistalsis of theoesophagus in three. Treatment, which consisted of high dosecorticosteroids, parenteral nutrition, promotility agents, andantibiotics, led to remission of both CIPO and urinary abnormalities inall cases. Antroduodenal manometry performed in two patients afterremission showed increased intestinal motility. One patient died, andpostmortem examination showed intestinal vasculitis.
Conclusions—CIPO inSLE is a life threatening situation that can be reversed by treatment.It may be: (a) a complication or onsetfeature of the disease; (b) secondary tosmooth muscle involvement; (c) associatedwith ureteral and vesical involvement; (d)the result of intestinal vasculitis.

Keywords:chronic intestinal pseudo-obstruction; systemiclupus erythematosus

     

Rapid appearance of severe mitral regurgitation under high-dosage corticosteroid therapy in a patient with systemic lupus erythematosus

Survival of patients with systemic lupus erythematosus has increasedwith corticosteroid therapy. However, adverse effects of corticosteroidtherapy on cardiovascular structures, such as scarring and shrinkingof affected valves, are not well known. We report the case ofa 19-year-old patient who developed severe mitral insufficiencywithin a few weeks after high-dosage corticosteroid therapyhad been instituted for an acute relapse of systemic lupus erythematosus.The rapid development of severe mitral regurgitation was documentedby sequential colour Doppler echocardiography.     

Biomarkers for systemic lupus erythematosus

In recent years, biomarkers have shown significant promise in helping decision‐making in drug development. Systemic lupus erythematosus (SLE) is a complicated and highly heterogeneous disease that involves all organs. Only one drug, belimumab, has been approved by the US Food and Drug Administration to treat SLE during the last 50 years and there remains a high unmet medical need to develop new and effective therapies to benefit different patient populations in SLE. Due to the extreme heterogeneity of the disease and the complex and rigorous process to validate individual biomarkers, there is currently a very limited number of consensus biomarkers to aid the treatment decision‐making in SLE. This review provides a snapshot of some biomarkers in the field that have the potential to make a big impact on drug development and/or treatment decisions by physicians. These include: type I interferon (IFN) gene signature as a pharmacodynamic marker and potential predictive marker for anti‐type I IFN therapy; anti‐double stranded DNA as a disease marker and potential predictive marker for flares; the complements and neutrophil signatures as disease marker of SLE; and TWEAK (a tumor necrosis factor family member produced by macrophages) and MCP‐1 as potential markers to predict renal flares. Most of these markers need carefully planned and prospective studies with high statistical power to confirm their respective utilities. With the development and application of powerful new technologies, more successful biomarkers will emerge in SLE. This could improve the management of patients in the clinic and facilitate the development of novel and more effective therapeutics for this difficult‐to‐treat disease.     

Hydroxychloroquine-induced seizure in a patient with systemic lupus erythematosus

We report on a case of a 17-year-old female with systemic lupus erythematosus (SLE), with a clinical history of complex partial seizure, who developed a tonicoclonic crisis after receiving hydroxychloroquine for 2 weeks at a dosage of 200 mg/day (5 mg/kg). The absence of previous similar episodes and of recurrences after withdrawal of the drug in subsequent months, the short latency after administration and the favourable short-term evolution raised suspicions for a potential role of the drug in the development of the isolated convulsive crisis. It is possible for hydroxychloroquine to be responsible for tonicoclonic seizures in predisposed subjects. Received: 19 March 2000 / Accepted: 16 June 2000