Patients with SLE show in a variety of neuropsychiatric symptoms, although we could not use standardized methods for evaluating and making diagnosis of the syndromes. ACR felt to develop objective and valuable tools for the diagnosis and classification of neuropsychiatric lupus, therefore, they proposed a new tentative set of nomenclatures describing neuropsychiatric lupus syndromes in accordance with 4th edition of Diagnostic and Statistical Manual of Mental Disorders provided by American Psychiatric Association. For this purpose, The Ad Hoc Committee collected and evaluated 108 case presentations of neuropsychiatric lupus from USA, Canada, and UK. Re-evaluation of the tentative nomenclatures selected 19 neuropsychiatric syndrome to facilitate and enhance clinical research. They grouped into peripheral and central nervous system lupus, and central nervous system lupus was divided into neurologic syndromes and diffuse psychiatric/ neuropsychological syndromes. Instead of organic brain syndrome, a term of acute confusional state was introduced.

Dermatologic manifestations are among the most common signs of systemic lupus erythematosus (SLE). The Hopkins Lupus Cohort study is a prospective study in which patients with SLE are seen on a quarterly basis for measurement of disease activity, laboratory tests, and assessment of morbidity and quality of life. This cohort has allowed unique insights into the epidemiologic factors of SLE, the presentation of dermatologic lupus, and morbidity, all of which are presented in this report. In addition, the dermatologic signs of antiphospholipid antibody syndrome (APS) are reviewed. Approaches to treatment of dermatologic lupus and APS are discussed.

BACKGROUND

Systemic lupus erythematosus (SLE) occurs predominantly in young women, but also in children. The prevalence of SLE varies worldwide, ranging from about 1 in 3500 women (regardless of race) in the UK, to 1 in 1000 women in China, to 1 in 250 African-American women in the US.

METHODS

We conducted a systematic overview, aiming to answer the following clinical questions: What are the effects of immunosuppressants in people with proliferative lupus nephritis? What are the effects of different immunosuppressants compared with each other in people with proliferative lupus nephritis? We searched: Medline, Embase, The Cochrane Library, and other important databases up to April 2014 (Clinical Evidence overviews are updated periodically; please check our website for the most up-to-date version of this overview).

RESULTS

At this update, searching of electronic databases retrieved 448 studies. After deduplication and removal of conference abstracts, 120 records were screened for inclusion in the review. Appraisal of titles and abstracts led to the exclusion of 53 studies and the further review of 67 full publications. Of the 67 full articles evaluated, four systematic reviews and one RCT were added at this update. We performed a GRADE evaluation for 13 PICO combinations.

CONCLUSIONS

In this systematic overview, we categorised the efficacy for 10 interventions based on the effectiveness and safety of immunosuppressants plus corticosteroids compared with corticosteroids alone, and immunosuppressants plus corticosteroids compared with each other in people with proliferative lupus nephritis (WHO grades III-V).

Mucocutaneous manifestations occur frequently in systemic lupus erythematosus (SLE). Common non-lupus dermatoses may be confused with lupus rashes, with important clinical consequences. A study of 84 consecutive patients with SLE was performed to determine the comparative frequency of lupus and non-lupus mucocutaneous abnormalities, the comparative sensitivity of routine histology and immunofluorescence in the diagnosis of lupus rashes, and the association of skin manifestations with other clinical and serological features. Thirty-five patients had dermatoses attributable to SLE (mean 3.7 per patient) and 58 had dermatoses which were not directly attributable to SLE (mean 2.1 per patient), highlighting the need for accurate diagnosis of skin rashes in SLE patients. Routine histology confirmed the clinical diagnosis of typical cutaneous lupus in 100% of malar lupus rashes and in approximately 90% of subacute cutaneous and discoid lupus rashes. Direct immunofluorescence of the affected skin provided supportive evidence of cutaneous lupus in 60% of malar lupus rashes and approximately 50% of subacute cutaneous and discoid lupus rashes. This reaffirmed the poor sensitivity of immunofluorescence, compared with routine histology, in the diagnosis of lupus rashes. The association of subacute cutaneous lupus with anti-Ro antibodies was confirmed.

BACKGROUND

Lupus erythematosus is a multisystemic disease that is characterized by autoantibody production and immune complex deposition in such tissues as the mucosa, joints, the central nervous system, and skin. Cutaneous lupus erythematosus is categorized as acute, subacute, and chronic. Chronic cutaneous lupus erythematosus comprises discoid lupus erythematosus (DLE) and lupus profundus (LP).

OBJECTIVE

To analyze the expression of proapoptotic molecules in patients with lupus erythematosus discoid and lupus profundus.

METHODS

Descriptive study, the study groups comprised 10 cases of LP and 10 cases of DLE, and a control. Skin samples of cases and controls were processed for immunohistochemistry and by TUNEL technique. The database and statistical analysis was performed (statistical test X(2)) SPSS (Chicago, IL, USA).

RESULTS

Apoptotic features were broadly distributed along the skin biopsies in epidermal keratinocytes as well as at dermis. By immunohistochemistry the expression of Fas receptor and Fas-L was higher in the skin of lupus patients compared with controls. We also noted differences in Fas-L, -Fas, and -Bax proteins expression intensity in discoid lupus erythematosus patients in the epidermis, and hair follicles.

CONCLUSIONS

Fas and Fas-L are expressed similarly in LP and DLE.

Twenty-seven patients with histologically confirmed lupus nephritis were studied to identify the incidence of lupus band and its significance to histological patterns of nephritis and complement levels. It was found that the kidney involvement is 2.5 times more frequent in lupus band positive patients. The association of a positive lupus and low C3 level signifies the presence of diffuse proliferative glomerulonephritis rather than membranous glomerulonephritis. The results, together with earlier reports, are discussed.

A case of discoid lupus erythematosus (DLE) in a 20 year old female patient is presented. The, case clinically resembled lupus causing bilateral ectropion and exposure keratitis in both eyes.

The cutaneous manifestations of lupus erythematosus (LE) are extremely varied, and they predominate among the clinical signs of chronic LE and subacute cutaneous LE. In chronic LE, typical lesions are found in regions exposed to sunlight and consist of erythema, hyperkeratosis and skin atrophy. The skin of patients with subacute cutaneous LE is also highly sensitive to light; the lesions consist of an annular or polycyclic or sometimes psoriasiform erythemato-squamous eruption observed mainly in uncovered areas. These dermatological forms are part of the wide spectrum of "lupus". Cutaneous manifestations are often associated with systemic signs of varying type and severity and with immunological abnormalities, such as antibodies to Ro in subacute cutaneous LE, which act as markers of the disease.

A growing body of evidences reinforces the close link between systemic lupus erythematosus (SLE) and atherosclerosis which is due to traditional and nontraditional risk factors for cardiovascular diseases. It is now recognized that SLE has a particular pattern of dyslipoproteinemia characterized by low HDL levels and increased triglycerides, which is aggravated by flares. Multiple mechanisms can induce an altered lipoprotein metabolism in SLE such as cytokines produced during systemic inflammation, autoantibodies and therapy.