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PubMed1981

Immunofluorescence study of cutaneous blood vessels of 16 patients with systemic lupus erythematosus.

In 16 patients with systemic lupus erythematosus, blood vessels in the affected region of the skin were examined regarding localization of immune complexes. Deposition of IgG, IgA, IgM, IgE, beta 1C and fibrin in the vessel walls was more frequently observed in intramural and perivascular sites than in intimal sites. IgM and fibrin were usually detected in intramural sites only. In large caliber arteries of the skin the intima was the main site of deposition of immune reactants, whereas, in small-caliber vessels, including capillaries, immune reactants were more often found in intramural and perivascular sites.

Adolescent↗
PubMed1982

Methyl prednisolone pulse therapy in the treatment of systemic lupus erythematosus.

Twenty patients with active systemic lupus erythematosus (SLE) were treated with methyl prednisolone pulse therapy (MPPT) and followed up for up to 24 weeks (mean 18 weeks). Beneficial effects of MPPT were observed principally on arthralgia, pleuritic pain, vasculitic skin rash, pyrexia, and lymphadenopathy. The serological tests showing the most improvement were ds DNA binding and the serum C3 level. MPPT was found to be both safe and easy to administer. It may be of value in treating patients with SLE whose disease is not controlled by moderate doses of corticosteroids and may also enable the dose of maintenance corticosteroids to be reduced appreciably.

Female↗
PubMed1992

Human immunodeficiency virus (HIV) and HIV infected cells in saliva and salivary glands of a patient with systemic lupus erythematosus.

A young woman with systemic lupus erythematosus (SLE) was infected with human immunodeficiency virus 1 (HIV-1) and about 6 years later developed persistent bilateral parotid gland enlargement. It was unclear whether this represented salivary gland involvement as a component of her SLE (secondary Sjögren's syndrome) or the initial clinical manifestation of her HIV-1 infection. HIV proviral DNA was found in individual salivary glandular secretions and in whole saliva. Additionally, cells positive for HIV RNA were isolated from whole saliva. A parotid gland biopsy revealed infiltrating lymphocytes containing large amounts of HIV RNA.

Adult↗
PubMed2006

Necrotizing fasciitis resulting from Streptococcus pneumoniae in recently diagnosed systemic lupus erythematosus case: a case report.

Systemic lupus erythematosus (SLE) is a systemic, autoimmune disease. SLE patients are prone to infections, and their hospital admissions and mortality are most commonly associated with infections. Necrotizing fasciitis (NF) is a rare, life-threatening infection of the subcutaneous tissue. In this report, NF associated with Streptococcus pneumoniae (SPN) that developed within hours and resulted in death is presented in a 46-year-old female case who was recently diagnosed as SLE and did not receive any medication (steroid, immunosuppressive, etc.) except for etodolac. This case shows that SLE can generate predisposition to NF, and SPN can play a role in NF etiology.

Disseminated Intravascular Coagulation↗
PubMedDate not supplied

Aseptic leptomeningitis in systemic lupus erythematosus. A case report.

OBJECTIVE: Systemic lupus erythematosus (SLE) with leptomeningeal involvement is a rare condition. We report a case in a 17-year-old woman. CLINICAL PRESENTATION AND INTERVENTION: The patient was admitted to hospital with the complaints of vertigo, nausea, vomiting, headache, diplopia, ptosis on the left and weakness of the left leg. A diagnosis of SLE was established, with diffuse leptomeningeal involvement demonstrated by cranial magnetic resonance imaging. When treated with steroids, the clinical problems resolved almost completely. CONCLUSIONS: Although rare, leptomeningeal involvement can complicate SLE. Cranial magnetic imaging can demonstrate tissue involvement. The condition responds to steroid therapy.

Adolescent↗
PubMed2000

Stenosis of a mechanical mitral prosthetic valve in a patient with systemic lupus erythematosus.

A young woman with systemic lupus erythematosus (SLE) developed progressive heart failure several years after mitral valve replacement with a Björk-Shiley prosthesis for treating mitral stenosis due to Libman-Sacks endocarditis. She was admitted to the hospital with pulmonary oedema. Transoesophageal echocardiography revealed stenosis of the mitral prosthesis, which was covered by fibrous tissue. Replacement of the prosthesis was done but the patient died from cerebral haemorrhage three days later. Although three cases of prosthetic valve dysfunction in SLE have been documented so far, this is to our knowledge the first report of a SLE recurrence on a tilting disc mechanical valve.

Adult↗
PubMedDate not supplied

[Seronegative systemic lupus erythematosus (author's transl)].

Seronegative systemic lupus erythematosus is defined as a SLE devoided of antinuclear factors as well as of LE cells when at least 4 out of the 14 ARA criteria are present. We describe herein two case reports and review previous literature concerning this topic. This kind of SLE is characterized by a high incidence of Raynaud's disease, photosensitivity, oral ulcerations, alopecia and perhaps less frequent kidney and central nervous system involvement. The hypotheses about the absence of ANF are discussed. The awareness of such a SLE ("devoided of ANF rather than" seronegative") is to be kept in mind from a chemical, therapeutic and pathophysiologic point of view.

Adult↗
PubMed1988

Autoantibodies to the constitutive 73-kD member of the hsp70 family of heat shock proteins in systemic lupus erythematosus.

Serum from patients with systemic lupus erythematosus (SLE) frequently contain IgM and IgG autoantibodies to the constitutively expressed 73-kD/pI 5.5 member of the hsp70 family of heat shock proteins, as determined by one-dimensional (SDS-PAGE) and two-dimensional (IEF/SDS-PAGE) immunoblotting, and by solid-phase SLE Ig immunoprecipitation experiments using hsp70 protein-specific mAbs as probes. Autoantibodies to hsp70 also were detected in a minority of sera from patients with other rheumatic or viral diseases, but not in normal sera. These data may provide additional insight into etiologic and pathophysiologic mechanisms in this and related autoimmune disorders.

Autoantibodies↗
PubMed1988

Choroidopathy in systemic lupus erythematosus.

Choroidopathy in association with systemic lupus erythematosus (SLE) is a clinically unusual manifestation, previously described in only six patients, to our knowledge. We have followed up six patients with SLE and choroidopathy manifested by multifocal, serous elevations of the retinal pigment epithelium and sensory retina. In four patients, macular involvement was present, and they suffered visual loss. Two eyes of two patients progressed to large, bullous, exudative retinal detachments. In the three patients in whom control of the systemic disease was achieved, the serous detachments resolved. The pathogenesis is most likely related to choroidal vascular disease with resultant pigment epithelial damage and serous fluid leakage beneath the retina.

Adult↗
PubMed2003

The genetics of human systemic lupus erythematosus.

Genetic dissection of human systemic lupus erythematosus (SLE) has been under intense studies during the past decade. Although the complexity inherent to polygenic, multifactorial diseases is challenging, several new insights have been obtained in the past several years using linkage and association studies of families containing SLE patients as well as case-control studies of populations. In addition, recent advances in our understanding of the human genome and emerging technology have been providing new tools in analyses of complex traits, such as SLE. An overview of our current understanding of the genetic basis of SLE and a brief review of findings of linkage and association studies are described here.

Animals↗
PubMed2006

Rembrandt's Maria Bockenolle has a butterfly rash and digital deformities: overlapping syndrome of rheumatoid arthritis and systemic lupus erythematosus.

Rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) are the most common autoimmune disorders, although they each have very different pathophysiology. In general, RA is considered to be a Th1-mediated disease, while SLE is a Th2-mediated disease. Thus, their overlapping, in so called "rhupus", is a rare condition. In Rembrandt van Rijn's (1606-1669) portrait of the middle-aged Maria Bockenolle, we have what may be the earliest depiction of a case of rhupus syndrome: the coexistence of a butterfly rash and digital deformities. This suggests the possible historical importance of an RA epidemic which took place in the early 17th century.

Arthritis, Rheumatoid↗
PubMed2002

Effect of selective posterior rhizotomy on transverse myelitis in a patient with systemic lupus erythematosus.

This report describes the case of a 26-yr-old man experiencing transverse myelitis, a rare but serious complication of systemic lupus erythematosus occurring in less than 1% of patients with systemic lupus erythematosus, 4 yr after the onset of systemic lupus erythematosus. Significant neurologic deficits, including spastic paraplegia, dysthetic pain, and impaired bladder control, which made him completely bedridden and dependent in activities of daily living, continued, despite his immediate diagnosis and treatment. The patient received bilateral L1 to S1 selective posterior rhizotomy 1 yr after the onset of transverse myelitis, and 10 mo after selective posterior rhizotomy, he was completely independent in ambulation and self-care, demonstrating that selective posterior rhizotomy can be safely performed and its goals achieved under different medical conditions, as long as thorough preoperative evaluation and every possible precaution have been taken.

Activities of Daily Living↗
PubMed1981

The role of histones as nuclear autoantigens in drug-related lupus erythematosus.

Patients with drug-related lupus erythematosus produce antibodies to nuclear histones which can be detected by a three-step indirect immunofluorescence technique. Procainamide-related antinuclear antibodies were detected by this technique, but hydralazine-related antinuclear antibodies were not. Certain evidence suggests that antibodies induced by the two drugs are reactive with different subclasses of histones. Hydralazine was shown to interact with a soluble DNA-histone complex, and the resulting interaction rendered the histone moiety resistant to trypsin digestion. This mechanism may help to maintain DNA-histone complexes in a potentially immunogenic form and result in the production of autoantibodies.

Animals↗
PubMed2005

Altered regulation of IL-2 production in systemic lupus erythematosus: an evolving paradigm.

In systemic lupus erythematosus (SLE), IL-2 production by T lymphocytes in vitro is impaired. Deficient IL-2 production may be an outcome of a primary SLE T cell disorder that is due to impaired signal transduction. In this issue of the JCI, evidence is presented that an anti-TCR/CD3 complex autoantibody present in SLE sera can bind to T cells and activate the Ca(2+)-calmodulin kinase IV (CaMKIV) signaling cascade, resulting in downregulation of IL-2 transcription and IL-2 production. Because IL-2 may contribute to the maintenance of T cell tolerance, deficient IL-2 production could promote a breach of T cell tolerance that results in autoantibody production in SLE.

Autoantibodies↗
PubMedDate not supplied

[Thrombosis in disseminated lupus erythematosus].

In the course of disseminated lupus erythematosus (DLE), the development of arterial or venous thrombosis is sometimes observed. They are rare in the course of hematological forms of DLE, in which the more or less constant leucopenia is accompanied to variable extent by the affection of the other blood-lines: thrombocytopenia and/or thrombocytopathy and/or hemolytic anemia. The discovery of a circulating anticoagulant seems to be correlated with the existence of thrombosis. In fact it involves antiphospholipid antibodies, responsible for the protraction of certain coagulation tests, which shows up their existence. Recent studies have shown recognition of the membrane phospholipids of the vascular endothelial cell. A functional imbalance in this would be observed to encourage the synthesis of factors favorable to thrombosis.

Abortion, Spontaneous↗
PubMed1993

A controlled study of peripheral neuropathy in systemic lupus erythematosus.

In 34 patients with systemic lupus erythematosus (SLE) and 34 age- and sex-matched healthy controls we performed standardized quantifiable neurological testing for neuropathic symptoms (Neuropathic Symptom Score) and deficits (Neurological Disability Score), as well as nerve conduction velocity studies (NCV) and determination of vibration thresholds (VT). SLE patients had more neuropathic symptoms and deficits than controls. Most quantitative and qualitative NCV attributes showed no difference between patients and controls. However, if categorized as NCV abnormalities of 2 or more nerves in each individual, a frequency of polyneuropathy of 21% was seen in SLE patients compared to 6% in controls. VT indicated a slight, but widespread, diffuse polyneuropathy in patients compared to controls.

Adolescent↗
PubMed2002

Controversies and advances in the management of systemic lupus erythematosus in children and adolescents.

Systemic lupus erythematosus (SLE) begins in childhood or adolescence in approximately 15% of cases, and although short- and long-term outcomes have improved considerably in the past 20 years there remain many areas of controversy regarding optimal management of these patients. In this chapter, areas of debate reviewed include: the appropriate timing of adding immunosuppressive therapy to corticosteroids, the optimal choice of immunosuppressive medication, the management of patients who fail 'standard' therapies, the significance and appropriate treatment of antiphospholipid antibodies in children with SLE and the emerging morbidities of childhood SLE. The goal of this chapter is to provide a balanced literature review and clinical approach to children and adolescents with SLE and to highlight areas requiring studies in paediatric clinical research.

Adolescent↗
PubMed1997

Systemic lupus erythematosus: demographics, prognosis, and outcome.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disorder with an annual incidence of 50 to 70/million and a prevalence of 500/million population. The highest incidence is observed in women aged 20 to 40 years. The clinical manifestations of SLE are remarkably heterogeneous. Major organ system involvement may occur in the heart, lungs, kidneys, and central nervous system and is responsible for most of the mortality and morbidity caused by the disease. Complications of drug treatment, in particular corticosteroid side effects, contribute to longterm morbidity. The major causes of death are directly related to the disease and include acute vascular neurologic events, renal failure, cardiovascular or pulmonary involvement, infection, and coronary artery disease.

Adolescent↗