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PubMed1990

Glycoprotein specificity of cold-reactive IgM antilymphocyte autoantibodies in systemic lupus erythematosus.

Sera from patients with systemic lupus erythematosus frequently contain IgM antibodies to glycoproteins of Mr 46,000 and approximately 200,000 isolated from nonionic detergent lysates of mature T cells by affinity chromatography with solid-phase wheat germ agglutinin. Autoantibodies of this specificity correlate strongly with the presence of IgM anti-T cell autoantibodies, as determined by independent indirect immunofluorescence and complement-dependent microcytotoxicity assays, and are specifically absorbed by incubation of patient serum with viable T cells. Collectively, the data suggest that gp46 and, to a lesser extent, gp approximately 200 represent major targets of IgM antilymphocyte autoantibodies in systemic lupus erythematosus.

Antibody Specificity↗
PubMed2004

Infective thyroiditis in two cases of systemic lupus erythematosus.

We report on two patients with systemic lupus erythematosus, both of whom developed suppurative thyroiditis. One suffered from Staphylococcus aureus-induced thyroiditis and the other had tuberculous thyroiditis. The occurrence of tuberculous thyroiditis in systemic lupus erythematosus has not previously been reported. The diagnoses were made by fine-needle aspiration biopsy and subsequent bacteriological confirmation. Transient alteration of thyroid function was observed in both patients. In patients with systemic lupus erythematosus who present with fever and anterior neck pain, infection of the thyroid gland should be considered, and appropriate investigations undertaken.

Adult↗
PubMed1982

Bullous systemic lupus erythematosus.

Two patients with established systemic lupus erythematosus developed bullous dermatoses clinically suggestive of bullous pemphigoid (BP). Direct immunofluorescence demonstrated linear staining at the dermoepidermal junction for IgG and C3 in a pattern typical for BP. However, immunoelectron microscopy demonstrated deposits of IgG largely below the basal lamina, with little if any deposit present within the lamina lucida. These findings are consistent with systemic lupus erythematosus (SLE) and effectively rule out BP. In addition, the leukocyte attachment assay, an in vitro assay of the functional activity of tissue-deposited immune complexes, demonstrated strong attachment of leukocytes to the dermoepidermal junction of patients' skin, suggesting that the immune reactants were functional immune complexes of possible importance to the pathogenesis of the bullous lesions. To our knowledge, this is the first time that immunoelectron microscopy has been performed on skin from patients suspected of manifesting concurrent SLE and BP. Our findings cast doubt on previous such reports by demonstrating the existence of a bullous form of SLE resembling BP both clinically and by direct immunofluorescence.

Adolescent↗
PubMedDate not supplied

The utility of the lupus band test on sun-protected non-lesional skin for the diagnosis of systemic lupus erythematosus.

OBJECTIVE: The sensitivity and specificity of the lupus band test was evaluated, using three different criteria, on sun-protected non-lesional skin for the diagnosis of systemic lupus erythematosus (SLE). In addition, the sensitivity and specificity of the lupus band test was compared with those of other laboratory tests used in the diagnosis of SLE. METHODS: Sun-protected non-lesional skin biopsies from 65 patients (F 50; M 15; mean age 41 yrs.) with specific cutaneous manifestations of lupus erythematosus (LE) and from 18 patients with other dermatologic diseases (F 11, M 7; mean age 40 yrs.) were tested using the direct immunofluorescent technique. RESULTS: The sensitivity and specificity of the lupus band test was 10.5% and 97.8% respectively using the strict criterion of the presence of two different immunoreactants. The sensitivity and specificity were 52.6% and 69.5% respectively based on the presence of two different immunoreactants and were 78.9% and 47.8% based on the presence of only one immunoreactant. The highest sensitivity was found for ANA (100%). The specificity of all the laboratory abnormalities was particularly high, varying from 82.8% to 100%, except for ANA antibodies which showed a specificity of 65.2%. CONCLUSIONS: A positive lupus band test on sun-protected non-lesional skin (even if showing the presence of only one immunoreactant at the dermo-epidermal junction) represents a useful and specific criterion for identifying patients with LE. However, this test is not useful in distinguishing between cutaneous lupus patients with systemic involvement and those without systemic involvement.

Adult↗
PubMed2003

Discoid lupus erythematosus presenting as madarosis.

PURPOSE: Discoid lupus erythematosus (DLE) is an autoimmune disorder that usually affects the sun-exposed skin. Periocular involvement occurs uncommonly and may progress from eyelid erythema to scarring and madarosis. METHODS: Observational case report. RESULT: A case of DLE that presented with madarosis alone in the absence of preceding skin erythema and scarring. CONCLUSION: Our case demonstrates that DLE may present with madarosis alone in the absence of a history of preceding erythema and scarring. Discoid lupus erythematosus should therefore be considered as a differential diagnosis in chronic blepharitis that persists despite usual medical management and eyelid hygiene. Biopsy should be considered in the presence of clinical features such as erythematous scale on the face and alopecia and sent for direct immunofluorescence staining.

Diagnosis, Differential↗
PubMedDate not supplied

Neonatal lupus erythematosus mimicking langerhans cell histiocytosis.

Neonatal lupus erythematosus (NLE) is an autoimmune disease characterized primarily by transient skin lesions and/or permanent congenital heart block. Other clinical findings include self-limited cytopenias and liver disease. The syndrome results from the passive transfer of maternal anti-SSA, anti-SSB, or anti-U1RNP autoantibodies to the fetus across the placenta. The cutaneous manifestations are generally analogous to those of subacute cutaneous lupus erythematosus (SCLE) and consist of small, erythematous macules that progress to annular plaques with delicate scaling. The skin lesions usually resolve within the first 6 months of life as maternal autoantibodies are cleared from the infant's circulation. We describe a patient with cutaneous NLE with hepatic and hematologic manifestations. The clinical presentation was atypical, with splenomegaly and petechiae at birth followed by a crusting, papulosquamous skin eruption of the scalp and face mimicking Langerhans cell histiocytosis (LCH).

Antibodies, Antinuclear↗
PubMed1994

[Papular mucinosis. A rare cutaneous manifestation of lupus erythematosus].

The case of a 48-year-old patient with systemic lupus erythematosus is discussed. Under immunosuppressive therapy, symptomless papules occurred on the shoulders, back, chest and face. Histologically, there were deposits of mucinous matter which produced an intensive blue colour in the HALE-PAS and alcian blue colour test. The epidermal and dermal inflammatory changes which typically occur with lupus erythematosus were absent. Whereas mucin is commonly diagnosed in lupus erythematosus, the clinical manifestation of papulous mucinosis is a rare but diagnostically significant phenomenon, since it can occur without further findings which are typical of lupus erythematosus.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed1993

Thyroid autoantibodies in the subsets of lupus erythematosus: correlation with other autoantibodies and thyroid function.

Thirty four sera from: 12 patients with Systemic Lupus Erythematosus (SLE), 9 with Subacute Cutaneous Lupus Erythematosus (SCLE) and 13 with Discoid Lupus Erythematosus (DLE) (disseminatus 3, localised 10) were tested for the presence of: (a) anti-thyroglobulin and anti-microsomal autoantibodies (b) anti-Sm/RNP, anti-doublestranded. DNA (anti-ds. DNA), anti-single-Stranded. DNA (anti-ss. DNA), anti-cardiolipin (anti-Cl), anti-SSA, anti-SSB, Antinuclear Antibodies (ANA). T3, T4, TSH levels were also determined. Five patients with SLE (41.6%), 4 with SCLE (44.4%), and 2 with DLE (15.3%) had thyroid autoantibodies and only three of the 41 controls (7.3%). Five patients (14.7%), especially from SLE and SCLE groups, had biochemical hypothyroidism whereas only one had hyperthyroidism. Statistical evaluation for the possible coexistence of thyroid autoantibodies with a panel of lupus characteristic autoantibodies, revealed highly significant correlations with anti-Sm/RNP, IgG (p = 0.003) and anti-ds. DNA, IgM (p = 0.012). It may be concluded, that not only SLE but also SCLE predisposes to autoimmune thyroid disease and the prevalence of the latter is related to a great extent to the subset of the LE spectrum. From these results and from the inhibition experiments, it seems that some of the specific mono- or polyclonal autoantibodies may be multiple organ reactive.

Adult↗
PubMed1990

Specificity and sensitivity of diluted aPTT and anticardiolipin antibodies towards thrombosis and miscarriages in patients with systemic lupus erythematosus.

In 36 patients suffering from systemic lupus erythematosus (SLE), lupus anticoagulant (LA), as assessed by aPTT and diluted aPTT, and anticardiolipin antibodies (aCL) were studied. 14 patients, had a clinical history complicated by thrombosis and/or miscarriages. Among patients with thrombosis LA was positive in 42% and in 100% of patients when assessed by aPTT and diluted aPTT respectively; aCL were positive in 85.7% of patients. Among patients without a clinical history of thrombosis, 1 had prolonged aPTT, 3 had prolonged diluted aPTT and 5 had aCL positivity. Diluted aPTT was more sensitive than aPTT and aCL (p less than 0.01) to thrombosis and miscarriages; specificity to thrombosis and miscarriages ranged from 77.3% for aCL and 86.4% for diluted aPTT to 95.5% for aPTT but not significant differences were found. The study suggests that LA, as assessed by a sensitive test like diluted aPTT, is strongly associated to thrombosis and should therefore be considered an important risk factor.

Abortion, Spontaneous↗
PubMed2003

[Cardiovascular risk factors in systemic lupus erythematosus and in antiphospholipid syndrome].

Systemic Lupus Erythematosus (SLE) is an autoimmune disorder affecting multiple organ systems. Treatment of the disease has contributed dramatically in the long-term survival of the patients and now SLE has become a chronic inflammatory disorder. Present data suggest 5, 10 and 20-year survival rates of 93%, 85% and 68% respectively. Accelerated atherosclerosis and early coronary artery disease have become important causes of death and hospitalisation in SLE patients. Many cardiovascular risk factors can be considered: disease activity (particularly kidney involvement), sedentary life (in nearly 70% of the patients), hyperlipidemia, antiphospholipid antibodies, serum homocysteine and many others. Although traditional risk factors are operative in patients with SLE, the risk for myocardial infarction was increased 8.3 folds after controlling these factors in a study, suggesting that SLE itself was the strongest risk factor for cardiovascular disease. Lipid abnormalities may play a major role in increasing cardiovascular risk in SLE patients who are characterized by elevated triglycerides, very low-density lipoprotein cholesterol (VLDL-C), reduced levels of high-density lipoprotein cholesterol (HDL-C) and apolipoprotein (Apo) A-1. Anticardioli-pin antibodies may influence lipid levels in SLE; in particular SLE patients with IgG anticardiolipin antibodies had significantly lower HDL-C compared with patients with no anticardiolipin antibodies. Elevation of serum homocysteine is observed in 15% of SLE patients and is significantly associated with the development of stroke and arterial thrombotic events. The antiphospholipid syndrome (APS) is an acquired thrombotic disorder characterised by recurrent venous or arterial thrombosis or recurrent miscarriages, or both, associated with the presence in the serum of IgG or IgM anticardiolipin antibodies (aCL) and/or lupus anticoagulant (LAC). APS may occur as a primary disorder (PAPS) or associated with connective tissue diseases, mainly systemic lupus erythematosus (secondary APS). Primary and secondary APS are both associated with a significant increase of cardiovascular risk.

Antiphospholipid Syndrome↗
PubMed1975

Systemic lupus erythematosus. Contrasts and comparisons.

Systemic lupus erythematosus, a disease capable of exhibiting virtually any clinical manifestation, is discussed in detail and the findings are compared to those in other, presumably systemic lupus erythematosus-related connective tissue diseases. More emphasis has been placed on the clinical features than on the numerous laboratory manifestations. The conference begins and ends with a brief consideration of current concepts of pathogenesis and suggests some future investigational directions.

Animals↗
PubMed1992

The sequelae of chronic cutaneous lupus erythematosus.

Eighty-six patients with chronic cutaneous lupus erythematosus were examined. Twelve of these also suffered from systemic lupus erythematosus. The mean duration of the disease was 15.1 years. Fifty-seven percent of patients (49/86) had scarring of some kind producing destruction and deformity; 47% (41/86) had scarring of glabrous surfaces and 35% (30/86) had scarring alopecia; 35% (30/86) were also suffering from pigmentary disturbance. The details and treatment of these and other non-scarring sequelae are discussed.

Adult↗
PubMedDate not supplied

Chronic cutaneous lupus erythematosus--clinical spectrum.

The typical clinical manifestation of chronic cutaneous lupus erythematosus (CCLE) is the chronic discoid lupus erythematosus. This form is characterized by inflammatory, scarring lesions mainly involving the head or neck, but also elsewhere, mostly on the photoexposed areas. The typical lesions in chronic discoid lupus erythematosus appear as round or oval erythematous plaques with scales and follicular plugging. These plaques are well demarcated and spread peripherally. On the face, they can form a butterfly-like rash involving the cheeks and nose. The long-standing lesions are atrophic and scarring, sometimes with hypo-, and hyperpigmentation and teleangiectasias. In 50% of the patients, there is a widespread skin involvement. This form is considered to be the typical clinical picture of CCLE.

Chronic Disease↗
PubMed2002

Indocyanine green angiographic findings in systemic lupus erythematosus choroidopathy.

PURPOSE: To report two patients affected with systemic lupus erythematosus choroidopathy studied with combined fluorescein angiography and indocyanine green angiography. In particular, the presence of choroidal abnormalities at indocyanine green angiography, which could not be detected by fluorescein angiography, was studied. DESIGN: Observational case reports. METHODS: Retrospective review of the clinical and photographic records of two patients with systemic lupus erythematosus in whom choroidopathy developed. RESULTS: Four findings were unveiled by indocyanine green angiography: focal, transient hypofluorescent areas in the very early phase; fuzziness of large choroidal vessels with late diffuse zonal choroidal hyperfluorescence; poorly-defined areas of choroidal hypofluorescence visible up to the late phase; and focal cluster of pinpoint spots of choroidal hyperfluorescence visible from the intermediate to late phase. CONCLUSION: Indocyanine green angiography can provide information that is not detectable by clinical or fluorescein angiographic examination in patients with systemic lupus erythematosus choroidopathy. This information may prove useful in better understanding the pathogenesis of systemic lupus erythematosus choroidopathy.

Adult↗
PubMed1991

Blood rheology in lupus erythematosus.

Blood rheology is one of the determinants of perfusion and might therefore have an impact on the thromboembolic complications of lupus erythematosus. This study aimed at defining the flow properties of blood in patients with various types of lupus erythematosus. Results for 51 patients were compared with those for 20 controls matched for sex. The patients were divided into subgroups--chronic discoid, subacute cutaneous, and systemic lupus erythematosus--according to their clinical or laboratory characteristics. Blood and plasma viscosity, packed cell volume, red cell aggregation, and red cell deformability were used as parameters of blood rheology. Blood and plasma viscosity and red cell aggregation were significantly different in patients compared with controls, indicating reduced blood fluidity in lupus erythematosus. There were no marked sex differences. The rheological effects were greater in those with systemic lupus erythematosus than in those with chronic discoid or subacute cutaneous lupus erythematosus. The presence of a positive antinuclear antibody titre or methods of treatment (systemic steroids or retinoids) had no apparent effect on the parameters tested. It is suggested that a complex haemorheological deficit exists in lupus patients.

Acute Disease↗
PubMed2000

Clinical, histological and immunopathological features of 58 patients with subacute cutaneous lupus erythematosus. A review by the Italian group of immunodermatology.

BACKGROUND: Subacute cutaneous lupus erythematosus (SCLE) is a distinct subset of cutaneous lupus erythematosus clinically characterized by psoriasiform and/or annular lesions and by a mild or absent systemic involvement. OBJECTIVE: The Italian Group of Immunodermatology of the Italian Society of Dermatology and Venereology reviewed the cases of SCLE seen in 10 years (1987-1996). PATIENTS: Forty-six women and 12 men have been retrospectively studied, 42% had annular lesions, 39% psoriasiform ones and 16% both. RESULTS: Lesions were mainly localized on the neck and face and relapsed in spring and autumn. Seventeen patients had 4 or more American College of Rheumatology criteria and could be classified as having systemic lupus erythematosus. The most frequent histopathological alterations were epidermal atrophy, hydropic degeneration of the basal layer and perivascular lymphocytic infiltrate. Deposits of immunoglobulins and C3 at the dermo-epidermal junction on the clinically involved skin were present in 86% of the patients. Dust-like particles in the epidermis were only found in 3% of cases. Anti-Ro/SSA antibodies were found in 71% of the cases and anti-dsDNA only in 5% of cases. CONCLUSIONS: SCLE is a particular subset of cutaneous lupus erythematosus with peculiar clinical and immunopathological features.

Adolescent↗
PubMed2005

[Thalidomide in the treatment of chronic discoid lupus erythematosus].

INTRODUCTION: Thalidomide is the second line treatment of chronic lupus erythematosus. The efficacy of this treatment, the minimal effective doses and tolerance are poorly documented in the literature. PATIENTS AND METHODS: We present the data of a single-center retrospective studied among 18 patients with chronic lupus erythematosus, treated with thalidomide from 1998 to 2003. Inclusion criteria were: the presence of clinical lesions evoking the disease, confirmed by histological examination and direct immunofluorescence and treatment with thalidomide for more than 2 months. RESULTS: Mean age on diagnosis was of 35.8 years. Thalidomide had been initiated a mean of 10.6 years after diagnosis of chronic lupus erythematosus. In 13 out of 18 patients, thalidomide had been prescribed because of failure with prior treatments. Fifteen patients were improved by thalidomide (83.3 p. 100), with 11 (61 p. 100) complete and 4 (22 p. 100) partial remissions. Two (11 p. 100) patients were stabilized and treatment failed in one. The mean initial dose was of 100 mg/d (50-150), and maintenance dose was of around 50 mg/d (56 mg/d). The mean follow-up with thalidomide was of 19.4 months. Only one withdrawal due to side effects was reported. The most frequent side effects were: asthenia (33 p. 100), paresthesia (22 p. 100) and weight gain (16.6 p. 100). No side effects were reported in 10 out of 18 patients. DISCUSSION: This study confirms the efficacy of low dose thalidomide in the treatment of chronic lupus erythematosus. In our experience, tolerance to this treatment is good, the most frequent side effect was asthenia, but usually mild. No significant peripheral neuropathy was noted. The fear of side effects, notably neurological, should not delay initiation of thalidomide in the case of failure with current treatments.

Adolescent↗
PubMed1993

End-stage renal disease in systemic lupus erythematosus.

The progression of lupus nephritis severe enough to require dialysis does not necessarily indicate that it is "end-stage." Ten percent to 28% of patients with lupus nephritis who develop renal failure requiring dialysis will recover enough function to come off dialysis. The clinical activity of systemic lupus erythematosus (SLE) is quiescent in most patients with end-stage lupus nephritis, regardless of the modality of dialysis treatment. Clinical and serologic remission of SLE permits judicious withdrawal of immunosuppressive therapy, as well as a favorable long-term outcome for patients that is comparable to that of nonlupus patients. The great majority of deaths in patients with end-stage lupus nephritis occur in the first 3 months of dialysis and most often result from infection. Later, infection and cardiovascular complications are common causes of death. Patients with lupus nephritis should wait at least 3 months on dialysis before receiving a kidney transplantation. Immunosuppressive therapy and graft survival rates for lupus patients are not different from those of nonlupus patients. Recurrence of lupus nephritis in the allograft is exceedingly rare.

Humans↗