Typical mitral stenosis found in a systemic lupus erythematosus patient with a positive lupus anticoagulant. Is there a pathogenic link?
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Female↗
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Systemic lupus erythematosus with anticardiolipin antibodies and lupus anticoagulant syndrome.
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Adult↗
[Symposium on renal involvement in systemic diseases. III. Lupus erythematosus. (1) Immunopathological study on lupus nephritis (author's transl)].
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Antibodies, Antinuclear↗
[Symposium on renal involvement in systemic diseases. III. Lupus erythematosus. Supplement: the study on lupus nephritis. Serial renal biopsies especially in the patients with non-renal SLE (author's transl)].
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Adolescent↗
Glomerular deposition of renal tubular epithelial antigen in patients with systemic lupus erythematosus: its possible role in lupus nephritis.
Fifty-three renal specimens from 48 patients with SLE were examined for the presence of RTE in the glomeruli. Glomerular RTE, presumably in immune complex form was detected in 60% of the tissues. The deposition of these complexes was related to the severity of histologic changes and activity of SLE. In addition, glomerular localization of RTE was associated with decreased renal function and increased proteinuria. The association between the presence of glomerular RTE antigen, the severity of renal histologic changes and the decreased renal function suggested a possible role for this antigen in the pathogenesis of lupus nephritis.
Adolescent↗
[Lupus erythematosus profundus: two cases with ultrastructural study].
Lupus erythematosus profundus (panniculitis) is an unusual clinical variant of lupus erythematosus in which the lesions occur in deeper corium and panniculus. The diagnosis usually causes difficulty. Both reported cases are similar and concern young men with subcutaneous nodules appearing on the face and leaving depressed areas. Histological examination reveals a marked infiltrate in the dermis especially around the skin appendages, reaching the deep dermis and the fat tissue; this infiltarte is composed mainly of lymphocytes and histiocytes. Some moderate epidermal changes are present: liquefaction degeneration of the basal cell layer, epidermal atrophy, thickening of basement membrane (P. A. S.). Direct immunofluorescence of the lesions showed, in one case IgG deposits at the dermo-epidermal junction. Ultrastructural findingd are similar in both cases and may be an important point of diagnosis for isolated forms: -- tubular structures "paramyxo-virus-like-inclusions" are present in dermal infiltrate, endothelial cells and sometimes in keratinocytes of the basal layer; -- the basal lamina has many folds and villous projections;-- vacuoles and concentric lamellar formations are observed inside keratinocytes. No clinical or immunological signs of systemic lupus were found. Antimalaric drugs clear up the nodules.
Adolescent↗
Combined oral contraceptives in women with systemic lupus erythematosus.
BACKGROUND: Oral contraceptives are rarely prescribed for women with systemic lupus erythematosus, because of concern about potential negative side effects. In this double-blind, randomized, noninferiority trial, we prospectively evaluated the effect of oral contraceptives on lupus activity in premenopausal women with systemic lupus erythematosus. METHODS: A total of 183 women with inactive (76 percent) or stable active (24 percent) systemic lupus erythematosus at 15 U.S. sites were randomly assigned to receive either oral contraceptives (triphasic ethinyl estradiol at a dose of 35 microg plus norethindrone at a dose of 0.5 to 1 mg for 12 cycles of 28 days each; 91 women) or placebo (92 women) and were evaluated at months 1, 2, 3, 6, 9, and 12. Subjects were excluded if they had moderate or high levels of anticardiolipin antibodies, lupus anticoagulant, or a history of thrombosis. RESULTS: The primary end point, a severe lupus flare, occurred in 7 of 91 subjects receiving oral contraceptives (7.7 percent) as compared with 7 of 92 subjects receiving placebo (7.6 percent). The 12-month rates of severe flare were similar: 0.084 for the group receiving oral contraceptives and 0.087 for the placebo group (P=0.95; upper limit of the one-sided 95 percent confidence interval for this difference, 0.069, which is within the prespecified 9 percent margin for noninferiority). Rates of mild or moderate flares were 1.40 flares per person-year for subjects receiving oral contraceptives and 1.44 flares per person-year for subjects receiving placebo (relative risk, 0.98; P=0.86). In the group that was randomized to receive oral contraceptives, there was one deep venous thrombosis and one clotted graft; in the placebo group, there was one deep venous thrombosis, one ocular thrombosis, one superficial thrombophlebitis, and one death (after cessation of the trial). CONCLUSIONS: Our study indicates that oral contraceptives do not increase the risk of flare among women with systemic lupus erythematosus whose disease is stable.
Adolescent↗
Lupus-related advanced liver involvement as the initial presentation of systemic lupus erythematosus.
BACKGROUND AND PURPOSE: Systemic lupus erythematosus (SLE), a prototype of systemic autoimmune disease characterized by multiorgan involvement with diverse clinical and serological manifestations, principally affects women in their child-bearing years. Clinically significant hepatic abnormality as the initial presentation of SLE has rarely been reported. METHODS: Eleven patients with lupus with initial presentation of lupus-related hepatitis were included in this retrospective review. Clinical manifestation, immunological profiles, and risk factors for poor prognosis were analyzed. RESULTS: The most commonly associated clinical manifestations were found to be thrombocytopenia, leukopenia, advancing age, and presence of anti-SSA/Ro antibody and anti-thyroid antibodies. The diagnosis of SLE was delayed due to dominant hepatic abnormalities. Age greater than 50 years and marked hepatic decompensation in accordance with Child classification B and C might suggest poor prognosis (p=0.06). However, the p value was not statistically significant because of the small sample size. CONCLUSIONS: Lupus-related hepatitis, particularly in late-onset lupus, is common. In addition, the presence of anti-SSA, anti-thyroglobulin, and anti-microsomal antibodies is indicative of hepatic involvement in patients with SLE.
Adult↗
Interleukin-6 expression in the skin of patients with lupus erythematosus.
It has been proposed that interleukin-6 may play a role in the pathogenesis of autoimmune diseases like lupus erythematosus. We have therefore investigated the immunoreactivity of IL-6 in 32 skin biopsies of 23 patients suffering from chronic discoid lupus erythematosus (n = 16), subacute cutaneous lupus erythematosus (n = 5) and systemic lupus erythematosus (n = 5) as well as in uninvolved skin (n = 6) and in normal skin from healthy volunteers (n = 3). Increased immunohistochemical staining was detectable in 14 of 26 biopsies from lesional skin. The remaining biopsies from lesional, non-lesional and normal skin displayed only minimal or no reactivity, but 8 out of 12 lupus erythematosus patients had been pretreated with local or systemic antiinflammatory drugs. Irrespective of the LE subtype, immunolabelling was generally most intense in the basal layer of the epidermis, with additional intense suprabasal staining in sections from 2 of 5 SLE patients. Preferential production of IL-6 in the lower parts of the epidermis was confirmed by RNA in situ hybridization. No correlation was found between the deposition of immunoglobulins and complement at the dermo-epidermal junction and IL-6 expression in keratinocytes. These data suggest that IL-6 may be involved in LE although its exact role in the pathogenesis of the disease needs to be further elucidated.
Adult↗
Sources of autoantigens in systemic lupus erythematosus.
PURPOSE OF REVIEW: A hallmark of systemic lupus erythematosus is the production of autoantibodies that recognize nuclear antigens. However, the underlying events and mechanisms that lead to the selection of these molecules for the autoimmune response remain poorly understood. In this review, we will examine some of the proposed explanations for sources of systemic lupus erythematosus-specific autoantigens. We will focus on events related to apoptosis, viral infection, cytokine production, innate immune system components, and alternative splicing of pre-mRNA transcripts. RECENT FINDINGS: Definitive proof of a viral etiology for lupus remains elusive. However, recent observations have added to increasing evidence that viruses contribute to the bypass of tolerance in systemic lupus erythematosus. Also, events associated with apoptosis - most notably proteolytic autoantigen cleavage by caspases and granzyme B - have been implicated in the initiation of autoimmune responses for over a decade. Results obtained from animal models and human systems suggest complex functions for pro-apoptotic pathways in the regulation of immune responses. Inducible antigen expression and alternatively spliced transcripts may represent additional ways of generating autoantigenic material. Finally, toll-like receptor family members may play critical roles in the induction of antibody responses to nucleic acids in systemic lupus erythematosus. SUMMARY: Several factors may contribute to the generation of systemic lupus erythematosus-specific autoantigens. Determining the underlying causes of autoantibody production may provide important insight into the etiology and pathogenesis of this disease.
Animals↗
Topical tacrolimus therapy of resistant cutaneous lesions in lupus erythematosus: a possible alternative.
OBJECTIVE: To determine the efficacy of tacrolimus ointment 0.1% on resistant cutaneous lesions in patients with lupus erythematosus. METHODS: Twelve patients with skin manifestations were studied. Six had discoid lupus (DL), four subacute cutaneous lupus erythematosus (SCLE) and two systemic lupus erythematosus (SLE). All patients had extensive skin lesions refractory to previous treatment. Patients received topical tacrolimus 0.1% for a minimum of 6 weeks and response was evaluated by physicians' and patients' assessment and documented with photographs at baseline and at the end of the treatment. RESULTS: Eleven of 12 patients completed the therapy. One patient with DL discontinued because of side--effects-peeling and a burning sensation. Six patients were clearly improved, one patient had a minor remission of his face lesion while in four the rashes remained the same. Two patients with SCLE had significant regression of their lesions while the other two had no improvement. In DL, two had certain improvement, one minor improvement and two were without response. The patients with SLE had significant amelioration of their extensive photosensitive rash. CONCLUSION: Tacrolimus ointment 0.1% may be an effective alternative in patients with severe resistant cutaneous manifestations in lupus erythematosus.
Adult↗
Subacute cutaneous lupus erythematosus in Iceland.
Identification of patients with subacute cutaneous lupus erythematosus (SCLE) in Iceland was performed by means of a survey based on the histopathologic or immunofluorescent diagnosis of lupus erythematosus. Respondents underwent serologic studies, gave histories, and, dependent upon the latter, were examined. The resultant proportional rate of patients with SCLE approached 10% of all lupus erythematosus cases.
Antibodies, Antinuclear↗
Phototesting in lupus erythematosus.
Ultraviolet (UV) irradiation is a major factor in the pathogenesis of certain variants of cutaneous lupus erythematosus. Photosensitivity constitutes one of the criteria of the American Rheumatism Association for the diagnosis of systemic lupus erythematosus, which further emphasizes its importance. The pathomechanism of UV-induced lupus erythematosus remains unknown. The characterization of photosensitive subacute cutaneous lupus erythematosus (SCLE) by Gilliam and Sontheimer has led to a new approach. Through the development of standardized test methods it has became possible to reproduce cutaneous lesions in the UV-A and UV-B spectrum. These standardized test methods allow a better definition of photosensitivity than clinical history does. Recent clinical data show that besides SCLE another variant, lupus erythematosus tumidus, also reveals pronounced photosensitivity. In this review article phototest procedures, phototest results, and clinical correlations in different subgroups are discussed.
Fluorescent Antibody Technique↗
Cutaneous neonatal lupus erythematosus in Chinese neonates.
Neonatal lupus erythematosus (NLE) is a well established subset of Lupus erythematous (LE). Three chinese female neonates presented to our Skin Centre, from May 1990 to May 1991 with NLE. All had skin lesions without congenital heart block and systemic problems. Two had photosensitive annular erythematous lesions on scalp, urticarial lesions on body, and one with facial atrophy, with aplasia cutis congenita. The biopsies were non specific while one had C3 in vessel walls. The major serogical marker was anti La antibody/SSB in two babies and anti Ro antibody/SSA in one. Two mothers were known cases of LE and one, was undiagnosed. Two infants were treated with short term low dose prednisolone. The infants will require long term follow up with paediatricians, in view of the fact that they can develop SLE later. The diagnosis of NLE is emphasised in infants with facial lesions to avoid delay in diagnosis, and full work up in both infants and mother is necessary.
Antibodies, Antinuclear↗
Crusted scabies in a child with systemic lupus erythematosus.
A child with systemic lupus erythematosus who has been treated with prednisone for three years, developed crusted scabies. Scrapings from lesions revealed Sarcoptes scabiei adult mites mad eggs. The patient died with septicemia and renal failure soon after starting topical 20% sulfur. A marked improvement was observed in the cutaneous lesions.
Biopsy↗
Ganciclovir-resistant, cytomegalic interstitial lung disease in a patient with systemic lupus erythematosus.
A patient with systemic lupus erythematosus developed interstitial lung disease initially felt to be a manifestation of the disease but that, on further workup, proved to be a manifestation of cytomegalic disease resistant to ganciclovir. Treatment with foscarnet was associated with prompt improvement.
Antiviral Agents↗
Neonatal lupus erythematosus.
The infant of a mother with systemic lupus erythematosus (SLE) developed an extensive cutaneous eruption at 5 weeks of age. Biopsy findings were consistent with cutaneous lupus erythematosus (LE). Splenomegaly, anemia, neutropenia, and depressed total hemolytic complemtnt levels were additional findings. The course was benign, and all manifestations disappeared by 4 months of age. Fifty-two previously reported infants with cutaneous lesions, congenital atrioventricular heart block, or hematologic manifestations of neonatal LE are reviewed.
Female↗
Conversion of discoid lupus erythematosus to mixed connective tissue disease.
The progression from discoid lupus erythematosus (DLE) to severe systemic lupus erythematosus (SLE) is rare. Two patients with DLE for five and 10 years eventually developed systemic involvement with clinical features of mixed connective tissue disease (MCTD). Both patients had high titer serum antibody to ribonucleoprotein (RNP) and epidermal nuclear staining on direct immunofluorescence of normal skin. Neither patient had renal disease but one patient developed pulmonary involvement. This observation suggests that patients with DLE and the Raynaud phenomenon may have a connective tissue disease subset characterized by anti-RNP, the immunologic marker for MCTC.
Adult↗