Psychiatric manifestations in systemic lupus erythematosus

Psychiatric abnormalities are common in systemic lupus erythematosus (SLE) with a prevalence of 17% to 75%, reflecting different methods of patient selection and assessment, the different professional orientation of clinicians, and lack of an accepted consensus for diagnosing active neuropsychiatric lupus (NPSLE). The psychiatric syndromes included in the ACR Neuropsychiatric Lupus Nomenclature Committee criteria are cognitive dysfunction, acute confusional state (delirium), anxiety disorder, mood disorder, and psychosis. In SLE patients, identification of psychiatric phenomena and the generation of a differential diagnosis are crucial. Possible mechanisms include vascular injury and pathogenic antibodies. Treatment strategies are based on small case studies. The purpose of this review is to discuss clinical manifestations, pathogenesis and the present therapeutic options in psychiatric lupus.     

Pulmonary vascular disease in systemic lupus erythematosus.

The pulmonary vascular changes in systemic lupus erythematosus (SLE) have been investigated from 20 autopsies performed at the Mount Sinai Hospiral from 1964 to 1973. Acute lesions included fibrinoid necrosis and vasculitis. Chronic lesions consisted of intimal fibrosis, medial hypertrophy, alteration of elastic laminae, periadventitial fibrosis, and, in one case, aneruysmal dilatation. These changes were found variously in arterioles, arteries and veins. The fibrotic and occlusive vascular lesions may account for the syndrome of "unexplained breathlessness" that occurs in SLE. These lesions may progress in certain individuals to overt pulmonary hypertension; the concept of coexisting primary pulmonary hypertension and SLE should be re-examined.     

Neuropsychiatric manifestations in Thai patients with systemic lupus erythematosus

Neuropsychiatric (NP) manifestations in patients with systemic lupus erythematosus (SLE) [NPSLE] and prognostic factors were studied in 91 patients. There were 98 NP episodes, of which 78 (79.6%) occurred within the first year of the disease. Twenty-six patients (6.7%) had NPSLE as an initial presentation of the disease. There were seizures in 53 episodes (54.1%), psychosis in 13 (13.3%), acute confusion state in 11 (11.2%), abnormal consciousness in 6 (6.1%), transverse myelitis in 6 (6.1%), peripheral neuropathy in 5 (5.1%), cerebral infarction in 2 (2.0%) and aseptic meningitis in 2 (2.0%). Most forms of NPSLE responded well to high dose corticosteroids. Anti-convulsant therapy could be discontinued within a median duration of 3 months after the SLE activity was under control, and without significant recurrence of seizures. The 5-year and 10-year survival rates of patients with NPSLE were 75.9% and 50.6%, respectively. Patients with NPSLE had significantly more cutaneous vasculitis and less arthritis than those without.     

Atypical manifestations in a patient with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a chronic systemic inflammatory disease associated with the production of various autoantibodies and involvement of multiple organs. Necropsy findings in a 65 year old woman with SLE who had multiple aortic aneurysms and dissections, as well as other unusual manifestations, are described. The case illustrates the occurrence of and the difficulties encountered in the diagnosis of several diseases, namely aortic aneurysm, aortic dissection, acute pancreatitis, and Penicillium marneffei infection.     

Pulmonary disease in systemic lupus erythematosus and the antiphospholpid syndrome

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by disturbances in innate and adaptive immune mechanisms. Multiple systems and organs may be involved. Tissue damage and dysfunction are mediated by autoantibodies and immune complex formation. The lungs are among the organ systems commonly involved. The pulmonary manifestations usually occur in patients with multisystem disease and include: pleural involvement, parenchymal disease, pulmonary vascular disease and diaphragmatic dysfunction. Manifestations may range from sub-clinical abnormalities to life threatening disorders. Many of the pulmonary manifestations characteristic of SLE are seen in the antiphospholipid syndrome (APS) as well, in both the primary and secondary syndrome. In this review the diverse pulmonary manifestations are described as well as the diagnostic modalities available, including the use of induced sputum evaluation for early diagnosis and follow up. New treatment modalities are referred to.     

Childhood-onset systemic lupus erythematosus

OBJECTIVES: To describe the initial clinicolaboratory manifestations and short-term outcome in a series of Nigerian children with systemic lupus erythematosus (SLE). METHODS: A nonrandomized prospective study of consecutive cases of childhood-onset SLE. Baseline and follow-up clinicolaboratory data were collected and analyzed. Each patient was followed up for 12 months. RESULTS: Eleven children were studied. There were seven girls (F:M, 1.75). Mean ages at lupus onset and diagnosis were 10.0 +/- 2.53 years and 11.2 +/- 2.53 years, respectively. Mean time at onset of renal disease following SLE symptoms onset was 1.22 +/- 0.93 years. All cases were misdiagnosed prior to presentation; diagnosis was delayed in nine patients. Lupus activity was mild, moderate and severe in two, five and four patients, respectively. Hypertension (n = 5), nephrotic syndrome (n = 6), microerythrocyturia (n = 6) and acute renal failure (n = 7) were associated morbidities. Of the 27 presenting clinical features, 17 were nondiagnostic, while 10 were diagnostic. Fever (n = 9) was a major nondiagnostic symptom; major diagnostic manifestations were lupus nephritis (n = 11), arthritis (n = 10) and serositis (n = 7). Catastrophic antiphospholipid syndrome was diagnosed in three. The glomerular lesions were nonproliferative (n = 1), focal (n = 3) and diffuse (n = 7) proliferative lupus nephritis. Complete remission rate at end-point was 71.4%. Fourteen percent of the patients relapsed. Renal survival and mortality rates were 86.0% and 30.0%, respectively. CONCLUSION: In this study, severe renal and extrarenal comorbidities were common; mortality rate was also high. High frequency of misdiagnosis and delayed diagnosis were probably responsible for these.     

Pulmonary function in systemic lupus erythematosus patients without respiratory symptoms.

Pulmonary function was studied in 22 patients with systemic lupus erythematosus without pulmonary clinical symptoms. The most striking features were: a) a restrictive functional pattern with hyperinflation, characterized by a decreased vital capacity and increased residual volume; b) alteration of the elastic properties of the lung, with increased pulmonary elastance; c) impairment of the alveolar-capillary gas transfer capacity, with very significant changes of the CO diffusion and arterio-alveolar gradients for O2 and CO2. No marked differences were found in functional disturbance among patients in the active or inactive phase of the disease.     

RhoA参与系统性红斑狼疮发病的相关性研究

本研究旨在探索RhoA在SLE患者中的表达及临床意义。通过RT-PCR方法,检测诊断明确的SLE患者(n=40)与健康对照者(n=58)外周血细胞中RhoA的表达水平,分析其与SLE临床特征及IFN积分的相关性。同健康对照组相比,RhoA在SLE患者的外周血细胞中的表达水平显著升高(P0.000 1),结合临床资料分析提示RhoA的表达水平与SLE疾病活动指数(SLEDAI)及肾脏损害活动指数(Renal-SLEDAI)呈正相关,通过干扰素积分相关性研究,发现RhoA的基因表达与干扰素积分显著正相关。课题组的研究结果表明RhoA与SLE疾病活动性相关,RhoA可能参与了SLE的发病。     

Pulmonary alveolar hemorrhage in a pregnancy complicated by systemic lupus erythematosus

We present a case of a pregnant patient with fulminant systemic lupus erythematosus complicated by alveolar hemorrhage, a rare and potentially fatal manifestation of lupus. It typically presents in the context of a pulmonary-renal syndrome. Active lupus nephritis with hypoalbuminemia is a major risk factor for alveolar hemorrhage. Treatment with high-dose corticosteroids is the mainstay of therapy.     

Epidemiology of systemic lupus erythematosus

Introduction: Systemic lupus erythematosus (SLE) is a disease distributed worldwide, which occurs in both genders, and across racial/ethnic and age groups; however, higher rates are observed in adults, in women and in non-Caucasians. Genetic, environmental, sociodemographic and methodological issues are responsible not only for these differences but for the variable course and outcome of the disease. Non-Caucasians have a more severe disease with a higher risk for early mortality and damage accrual. Males also have a more severe disease; however, a negative impact of male gender on lupus outcomes has not been firmly established. Childhood-onset is associated with a more severe disease; moreover, it is also associated with higher damage and diminished survival; finally, late-onset lupus is mild but it is associated with higher damage accrual and a diminished survival.

Areas covered: In this review, we discuss the incidence and prevalence of SLE, the impact of age, gender and race/ethnicity in SLE and in the survival of those affected.

Expert commentary: Age, gender and race/ethnicity impact disease expression in SLE patients; despite improvements in survival, mortality in SLE remains almost three times higher than in the general population.     

Immunotherapy of systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a remarkably heterogeneous multisystem autoimmune disease. Improved understanding of the immunopathogenesis of this disorder over the past two decades is now being applied to therapy, with treatment being directed at specific immunological targets. This review will focus on both new uses for established therapies as well as on the introduction of novel agents. It is premature to claim that any one agent has superior efficacy to current therapies. However, the willingness of investigators supported by the pharmaceutical and biotechnology industries to evaluate the efficacy and safety of new products in controlled clinical trials may provide answers.     

Etiopathogenesis of systemic lupus erythematosus

Systemic lupus erythematosus is an autoimmune disease of unknown etiology. Research efforts of the last few years have mainly focused on basic molecular and cellular pathogenetic processes of the disease. Consequently, this paper reviews the etiopathogenetic hallmarks, such as impaired amount and presentation of nuclear antigens, production of antinuclear antibodies by T-cell-dependent B cell stimulation and organ damage by anti-dsDNA antibodies or immune complexes that are discussed at the present time. In summary, the hypothesis of a dysregulation of apoptotic cell clearance is strongly supported and broadly discussed.     

Clinical manifestations of cutaneous lupus erythematosus

The skin findings seen in lupus erythematosus can present with either lupus-specific or lupus-nonspecific findings, with lupus-specific skin disease showing findings histopathologically distinct for cutaneous lupus erythematosus. Lupus-specific skin diseases include chronic cutaneous, subacute cutaneous, and acute cutaneous lupus erythematosus. The types of skin lesions in each group are clinically distinct and recognizing the specific subsets helps in prognosticating the likelihood of underlying systemic lupus. A number of medications are associated with cutaneous lupus, in particular with subacute cutaneous lupus erythematosus. Lupus nonspecific skin lesions are not histopathologically distinct for cutaneous lupus and/or may be seen as a feature of another disease process. Nonspecific disease-related skin lesions are frequently seen in patients with SLE, usually in the active phase of the disease. The current ACR classification criteria for SLE include four somewhat overlapping dermatologic criteria, butterfly rash, discoid lupus, photosensitivity, and oral ulcers and thus patients can be classified as having SLE with only skin manifestations.     

Pathogenesis of systemic lupus erythematosus

The exact patho-aetiology of systemic lupus erythematosus (SLE) remains elusive. An extremely complicated and multifactorial interaction among various genetic and environmental factors is probably involved. Multiple genes contribute to disease susceptibility. The interaction of sex, hormonal milieu, and the hypothalamo-pituitary-adrenal axis modifies this susceptibility and the clinical expression of the disease. Defective immune regulatory mechanisms, such as the clearance of apoptotic cells and immune complexes, are important contributors to the development of SLE. The loss of immune tolerance, increased antigenic load, excess T cell help, defective B cell suppression, and the shifting of T helper 1 (Th1) to Th2 immune responses leads to B cell hyperactivity and the production of pathogenic autoantibodies. Finally, certain environmental factors are probably required to trigger the disease.