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PubMed2006

[Biological therapies of systemic lupus erythematosus].

In recent years new potential therapies of systemic lupus erythematosus, called biological therapies, have been developed. These agents influence some immunological reactions, essential for genesis of systemic lupus erythematosus, ie. T cells and B cells activation, antibodies production, cytokines and complement components activation. Pathogenic basis of using these therapies, their safety and efficacy assessment have been presented.

Antigen-Antibody Complex↗
PubMed1998

Linear cutaneous lupus erythematosus following the lines of Blaschko.

We describe two Japanese girls with discoid lupus erythematosus (DLE) in whom the condition showed a linear configuration following the lines of Blaschko. The clinical appearance was unusual but histological examination established the diagnosis. After reviewing the previous reports, we found that in six of eight patients with linear 'discoid' lesions, the age at onset was under 14 years; no patient has progressed to systemic lupus erythematosus. Patients with 'linear' DLE may compose a certain clinical subset. We propose the term 'linear cutaneous lupus erythematosus' which may be more suitable for the linear lesions of DLE.

Adolescent↗
PubMed1979

Transverse myelopathy in systemic lupus erythematosus.

There have been 28 reported cases of transverse myelopathy associated with systemic lupus erythematosus. In a 6-month period, the authors saw 2 new cases. Both of these patients were young women (29 and 31 years old) who had received a diagnosis of systemic lupus erythematosus prior to the onset of the transverse myelopathy. The case histories of the 2 patients are reported in detail and the findings are compared with those of previously reported cases. The rehabilitation program and outcome are described. Systemic lupus erythematosus should be considered in the differential diagnosis of transverse myelopathy because there may be many unsuspected cases.

Adult↗
PubMed1995

[Clinical and immunologic course of systemic lupus erythematosus in a 12-year-old girl].

The report presents the clinical course of a systemic lupus erythematosus in a 12-year-old girl, when the thrombocytopenia was the only symptom over the period of several months. In the subsequent stage of the clinical observation, in the coexistence of the subacute cutaneous lupus erythematosus with visceral changes were noted. The report emphasises the correlation between the clinical symptoms and the immunological disorders as well as the necessity of the complete immunological diagnostics in cases of isolate symptoms suggesting lupus erythematosus.

Child↗
PubMed1999

Apheresis for lupus erythematosus.

Indications for the use of apheresis technologies in managing lupus erythematosus have undergone numerous conceptual changes since the procedure was automated in the 1960s and first attempted for systemic lupus erythematosus (SLE) in 1974. Once thought to be a promising treatment for a variety of aspects of SLE, apheresis has been relegated to occupying a few important niches as an adjunct in managing the disorder. This article will review the history, physiology and rationale of apheresis pertaining to SLE. A focused critical review of pertinent literature is presented, along with specific recommendations relating to the place of apheresis in the lupus therapeutic spectrum.

Antiphospholipid Syndrome↗
PubMed1999

Antinuclear antibody-keratinocyte interactions in photosensitive cutaneous lupus erythematosus.

Autoimmune diseases are characterized by various circulating autoantibodies, especially antinuclear antibodies (ANA). It has been a long-standing issue as to whether and/or how ANA interact with epidermal cells to produce skin lesions. Of these ANA, the anti-SS-A/Ro antibody is the most closely associated with photosensitivity in patients with systemic lupus erythematosus (SLE) and its subgroups, including subacute cutaneous lupus erythematosus (SCLE) and neonatal lupus erythematosus (NLE). SS-A/Ro antigens are present in the nucleus and cytoplasm, and interestingly, ultraviolet B (UVB) light translocates these antigens to the surface of the cultured keratinocytes. Thus, anti-SS-A/Ro antibodies in the sera can bind to the relevant antigens expressed on the UVB-irradiated keratinocyte surface, and have been speculated to be an important inducer of antibody-dependent keratinocyte damage. This interaction between the anti-SS-A/Ro antibodies and UVB-irradiated keratinocytes may induce the skin lesions through a cytotoxic mechanism. This review will focus on the involvement of antibody-dependent cellular cytotoxicity in the pathogenesis of the skin lesions observed in photosensitive cutaneous lupus erythematosus.

Animals↗
PubMed1976

Renal vein thrombosis, nephrotic syndrome, and systemic lupus erythematosus: an association in four cases.

We report here four cases of systemic lupus erythematosus associated with the nephrotic syndrome and renal vein thrombosis and review six familiar cases previously noted in the literature. Renal biopsy in each of our cases showed changes consistent with the membranous type of lupus nephropathy. We discuss the exclusive appearance of this pattern in relation to renal vein thrombosis in other forms of renal disease. The occurance of renal vein thrombosis in patients with systemic lupus erythematosus and the nephrotic syndrome supports the evidence that the thrombosis is a complication rather than a cause of the nephrotic syndrome. The presence of pleuritic pain in a patient with systemic lupus erythematosus and the nephrotic syndrome should alert the clinician to the possibility of renal vein thrombosis and pulmonary emboli. With appropriate diagnosis and anticoagulation therapy, our patients had a benign course during 7 to 48 months of follow-up.

Adult↗
PubMed1995

Minocycline-associated lupus erythematosus.

We report a patient who developed symptoms of lupus erythematosus which was apparently related to minocycline therapy for acne vulgaris. To our knowledge, this is only the second reported case of minocycline-associated lupus erythematosus.

Acne Vulgaris↗
PubMed1978

Coexistent bullous pemphigoid and systemic lupus erythematosus.

A case of coexistent bullous pemphigoid and systemic lupus erythematosus is reported. Evidence for an immunologic pathogenesis is reviewed for both disorders. It is suggested that the coexistence of bullous pemphigoid and systemic lupus erythematosus may represent a similar pathomechanism.

Adult↗
PubMed1995

Classification of lupus erythematosus based upon cutaneous manifestations. Dermatological, systemic and laboratory findings in 191 patients.

BACKGROUND: Lupus erythematosus (LE) is a multi-organ-system disease, the characteristics of which are reflected in the 1982 American Rheumatism Association (ARA) criteria for systemic lupus erythematosus (SLE). From a dermatological point of view, only the presence of LE-specific histopathology is necessary and sufficient for the diagnosis of LE. The association between the type of LE-specific skin lesion and the severity of extracutaneous manifestations of LE has not yet been investigated systematically. OBJECTIVE: The aim of this study was to evaluate, according to the type of LE skin lesions, the prevalence of the 1982 criteria for SLE. METHODS: We selected 191 patients whose skin lesions were histologically diagnosed as LE specific. Patients were classified on the basis of skin disease, and their clinical and laboratory data were analyzed. RESULTS: Of 191 patients, 130 (68%) exhibited only one type of LE-specific skin lesion (monomorphic), 55 (29%) had two types (bimorphic) and the remaining 6 (3%) displayed three types (trimorphic). Nineteen of 22 (86%) patients who presented discoid lupus skin lesions above the neck without other eruptions were classified in the cutaneous-limited LE spectrum. Of 116 patients with acute lupus skin lesions (malar rash), 83 (72%) clearly fulfilled the 1982 ARA criteria for SLE. In skin lesions of LE profundus, chilblain lupus, subacute lupus (annular-polycyclic erythema and the papulosquamous variant), there were no significant correlations between the type of eruption and the severity of extracutaneous manifestations. CONCLUSION: Patients with acute lupus skin lesions could usually be classified as suffering from SLE, whereas monomorphic patients with localized discoid lesions rarely exhibited extracutaneous manifestations. This tendency was less distinct in bimorphic patients. Almost all patients with subacute skin lesions were bimorphic or trimorphic, which might be due to genetic or racial differences between Japanese and other populations.

Adult↗
PubMed1986

Bullous systemic lupus erythematosus: an unusual clinical course and detectable circulating autoantibodies to the epidermolysis bullosa acquisita antigen.

Bullous systemic lupus erythematosus is a newly recognized form of systemic lupus erythematosus characterized by a skin eruption clinically and histologically resembling dermatitis herpetiformis and responsive to dapsone. We report on a patient with bullous systemic lupus erythematosus who initially presented with lesions clinically resembling erythema multiforme, experienced exacerbation of her disease with dapsone, and had detectable circulating autoantibodies to the epidermolysis bullosa acquisita antigen.

Adolescent↗
PubMed1998

[Clinical significance of antiphospholipid autoantibodies in lupus erythematosus].

The authors have determined the prevalence of antibodies of cofactor dependent anticardiolipin and beta 2-glycoprotein I and lupus anticoagulant and the frequency of false positive VDRL test in systemic lupus erythematosus. The aim of this retrospective study was to assess the presence of these antibodies and symptoms of antiphospholipid syndrome. The serum samples were examined by modified ELISA method for detecting of cofactor dependent anticardiolipin. The antibodies to beta 2-glycoprotein I were examined by ELISA. The lupus anticoagulant and VDRL test were performed by routine laboratory method. The authors have found that 19 of 58 patients with systemic lupus erythematosus had cofactor dependent anticardiolipin, 10 patients had antibodies to beta 2-glycoprotein I and 4 patients had positive VDRL test. 5 of 34 plasma samples were lupus anticoagulant positive. 19 patients with systemic lupus erythematosus had 14 neuropsychiatric disorders, 9 cardiovascular diseases, 7 thrombocytopenia, 6 histories of recurrent abortion and fetal loss, 5 livedo reticularis and 3 thromboembolic events in all of them had detected antibodies to cofactor dependent anticardiolipin, while these complications were diagnosed in 39 anticardiolipin negative patients much more rarely. The results of this retrospective study suggest that significant association exists between the presence of cofactor dependent anticardiolipin and symptoms of antiphospholipid syndrome in systemic lupus erythematosus.

Antibodies, Antiphospholipid↗
PubMed1999

Hypertrophic discoid lupus erythematosus of the conjunctiva.

PURPOSE: To report the ophthalmic manifestations of hypertrophic discoid lupus erythematosus of the conjunctiva. METHOD: Case report and review of biopsy results. RESULTS: A 58-year-old woman with a history of chronic blepharoconjunctivitis presented with an unusual raised conjunctival lesion. Previous biopsy slides were reviewed and interpreted as diagnostic of discoid lupus erythematosus, hypertrophic or verrucous type. Both blepharoconjunctivitis and the raised conjunctival lesion resolved with hydroxychloroquine therapy. CONCLUSIONS: A raised conjunctival mass in the context of refractory blepharoconjunctivitis should elicit suspicion for discoid lupus erythematosus. The hypertrophic variant of this disease can affect the conjunctiva.

Biopsy↗
PubMed2000

Association of a promoter polymorphism of tumor necrosis factor-alpha with subacute cutaneous lupus erythematosus and distinct photoregulation of transcription.

Ultraviolet irradiation stimulates keratinocytes and dermal fibroblasts to release cytokines involved in apoptosis and immunomodulation, such as tumor necrosis factor-alpha and interleukin-1alpha. Recent work has associated the -308A polymorphism of the human tumor necrosis factor-alpha promoter with systemic lupus erythematosus and adverse outcomes in several infectious diseases. To explore the role of this polymorphism in ultraviolet-induced disease, we used two approaches. First, we examined its prevalence in individuals with different ultraviolet sensitivity. Compared with healthy controls, there was a substantially increased prevalence of -308A in subacute cutaneous lupus erythematosus, an extremely photosensitive form of cutaneous lupus erythematosus, but not in discoid lupus erythematosus, a less photosensitive form. Next, to examine molecular regulation by tumor necrosis factor -308A, cultured 3T3 fibroblasts were transiently transfected with chloramphenicol acetyl transferase reporter constructs under the control of either -308A or the wild-type -308G promoter. Without added interleukin-1alpha the two constructs produced similar baseline chloramphenicol acetyl transferase activity and similar responses to ultraviolet. The responses to interleukin-1alpha, a photoinduced cytokine, were markedly different: interleukin-1alpha without ultraviolet produced a 15-fold increase in chloramphenicol acetyl transferase transcription from the -308A construct without affecting the wild-type -308G. Interleukin-1alpha plus ultraviolet B caused a remarkable 300-fold increase in -308A chloramphenicol acetyl transferase transcription over baseline, while increasing the wild type to <15% of this level. These results indicate a clear difference between the two promoters, including a striking synergy between ultraviolet B and added interleukin-1alpha in the induction of transcription by the tumor necrosis factor-alpha -308A promoter. Overall, our findings indicate a strong linkage between the -308A polymorphism and subacute systemic lupus erythematosus, which is likely to directly contribute to the photosensitivity of these patients.

Chloramphenicol O-Acetyltransferase↗
PubMed1994

Renal transplantation in anticardiolipin antibody-positive lupus erythematosus patients.

Up to 44% of patients with systemic lupus erythematosus have antiphospholipid antibodies, which in some patients have been associated with thrombocytopenia, in vitro coagulation abnormalities, and a thrombotic tendency. Patients with lupus nephritis who reach end-stage renal disease may continue to have anticardiolipin antibodies (ACL-Ab) at the time of renal transplantation despite a lack of clinical or serologic activity of systemic lupus. The risk of thrombotic and other complications has not been previously reported in these patients. To evaluate the effect of ACL-Ab on the course of renal transplantation, the clinical course of eight ACL-Ab-positive lupus patients transplanted in the period from January 1990 to June 1992 were compared with five ACL-Ab-negative lupus patients transplanted during the same period. All patients had lupus nephritis as the cause of renal failure. There were four thrombotic episodes in the patients who were ACL-Ab-positive but none in the control group. Neither of the two groups differed in the number of rejection episodes, the rate of graft loss, or renal function at last follow-up. Patients with systemic lupus erythematosus who are ACL-Ab-positive can be successfully transplanted. However, these patients require attention to possible thrombotic events with a potential role for prophylactic anticoagulation.

Adult↗
PubMed1980

Procainamide-induced lupus erythematosus pericarditis encountered during coronary bypass sugery.

Procainamide is probably the most common offending drug responsible for the drug-induced lupus erythematosus syndrome today. Pericarditis has been reported to occur in from 14 to 18 per cent of the cases of procainamide-induced lupus erythematosus, and occasional reports of massive pericardial effusion, pericardial tamponade and constrictive pericarditis have appeared in the literature. We describe a patient who presented with features of procainamide-induced lupus erythematosus without any clinical evidence of pericarditis. He underwent coronary bypass surgery 12 days after administration of the drug was stopped and was found to have a significant pericardial effusion at the time of surgery; histologic examination of pericardial tissue and pericardial fluid confirmed that the pericardial effusion was related to the procainamide-induced lupus syndrome. The incidence of pericarditis in procainamide-induced lupus erythematosus may be higher than presently accepted figures would indicate. Symptoms and signs related to procainamide-induced lupus pericarditis may cause diagnostic confusion with common postoperative bypass complications; the full implications of this disease entity to the patient undergoing coronary bypass are unknown.

Biopsy↗
PubMed1993

Periparotid lupus erythematosus panniculitis. Clinicopathologic correlation of two cases presenting as primary parotid disease.

Lupus erythematosus (LE) panniculitis, also called lupus profundus, is an uncommon but well-established manifestation of both chronic cutaneous (discoid) LE and systemic LE. Although LE panniculitis characteristically may involve the face, specific parotid and periparotid involvement has not, to the best of our knowledge, been previously reported. We describe the clinicopathologic correlation of two cases of LE panniculitis that presented as apparent primary parotid parenchymal disease; one as a neoplasm, the other as parotiditis. Failure to recognize LE panniculitis resulted in prolonged periods of inappropriate therapy and significant morbidity, including an unnecessary parotidectomy. Lupus erythematosus panniculitis has well-established histologic criteria that should be considered in the differential diagnosis of an unexplained parotid mass.

Adult↗
PubMed2005

Familial aggregation of systemic lupus erythematosus, rheumatoid arthritis, and other autoimmune diseases in 1,177 lupus patients from the GLADEL cohort.

OBJECTIVE: To determine whether there is familial aggregation of systemic lupus erythematosus (SLE) and/or other autoimmune diseases in SLE patients and to identify clinical differences between patients with and those without familial autoimmunity. METHODS: We interviewed members of the Grupo Latinoamericano de Estudio del Lupus Eritematoso (GLADEL) inception cohort of 1,214 SLE patients to ascertain whether they had relatives with SLE and/or other autoimmune diseases. Identified relatives were studied. Familial aggregation was tested using reported highest and intermediate population prevalence data for SLE, rheumatoid arthritis (RA), or all autoimmune diseases, and studies were performed to identify the genetic model applicable for SLE. RESULTS: We identified 116 first-, second-, or third-degree relatives with SLE, 79 with RA, 23 with autoimmune thyroiditis, 3 with scleroderma, 1 with polymyositis, and 16 with other autoimmune diseases, related to 166 of the 1,177 SLE patients in the GLADEL cohort who agreed to participate. Forty-two SLE patients had 2 or more relatives with an autoimmune disease. We found a lambda(sibling) of 5.8 and 29.0 for SLE and of 3.2-5.3 for RA, when comparing with their reported high or intermediate population prevalence, respectively. We also found familial aggregation for autoimmune disease in general (lambda(sibling) = 1.5) and determined that for SLE, a polygenic additive genetic model, rather than a multiplicative one, is applicable. CONCLUSION: In SLE there is familial aggregation of SLE, RA, and autoimmune disease in general. A polygenic additive model applies for SLE. American Indian-white Mestizo SLE patients and those with higher socioeconomic level were more likely to have familial autoimmunity.

Arthritis, Rheumatoid↗