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PubMed2005

Long-term thalidomide use in refractory cutaneous lesions of lupus erythematosus: a 65 series of Brazilian patients.

Thalidomide has been reported as efficacious in refractory cutaneous lupus erythematosus (LE). The most fearful side-effects are teratogenicity and neuropathy. We reported clinical efficacy of long-term low-dose use of thalidomide in 65 patients with LE, emphasizing the prevalence of adverse effects, especially of neuropathy and its related factors. Data obtained from medical records included age, sex, disease duration, and the presence of diagnostic criteria for systemic lupus erythematosus (SLE), the extent and activity of cutaneous lesions and previous treatments. Sixty-three patients (98.9%) presented complete or partial improvement with thalidomide therapy. Drowsiness occurred in 50 patients (77%). Twenty-eight patients (43.2%) presented neuropathy symptoms. Nerve conduction studies were done in 21 (75%) of them and were abnormal in 12 (57%). With the interruption of thalidomide, 24 (82.5%) had complete or partial improvement of neuropathy symptoms and 23 (82%) of them had cutaneous relapse. There were no significant differences between those who developed or not neuropathy in treatment duration, age, total dose and systemic versus cutaneous LE. In conclusion, thalidomide can be used in refractory cutaneous LE with great efficacy and relative security. Controlled studies with schemes with lower doses or intermittent usage or alternative drugs are wanted to reduce the burden of cutaneous morbidity of lupus erythematosus.

Adolescent↗
PubMed1989

Endomyocardial biopsy in diagnosis and management of cardiovascular manifestations of systemic lupus erythematosus (SLE).

In recent years, the cardiovascular manifestations of systemic lupus erythematosus have became more apparent as a consequence of both prolonged survival and improvement in diagnostic modalities. We report the case of a 16-year old woman with systemic lupus erythematosus in whom the presence of cardiomyopathy was characterized by endomyocardial biopsy. This diagnostic technique also showed that the histologic hallmark of lupus myocardiopathy persisted despite corticosteroid and plasmapheresis, suggesting the need of a careful cardiac follow-up in these patients.

Adolescent↗
PubMed1980

Cutaneous calcinosis in localized discoid lupus erythematosus.

Cutaneous calcinosis is reported in the lesions of a woman with long-standing discoid lupus erythematosus. Calcium deposits were found in the middle and deep parts of the dermis surrounded by homogeneous collagen staining positively with Alcian blue solution. In contrast to the relative frequency of this event in patients with scleroderma or dermatomyositis, calcinosis cutis occurs very rarely in lupus erythematosus. To the best of our knowledge, this is the first report of calcinosis cutis in discoid lupus erythematosus.

Adult↗
PubMed1950

Lupus erythematosus.

Explore the source record for details and available documents.

Lupus Erythematosus, Discoid↗
PubMed2003

New insights into pregnancy-related complications in systemic lupus erythematosus.

Pregnancy in patients with systemic lupus erythematosus (SLE) presents an additional risk to an already complex clinical situation--overlap in symptoms between changes of pregnancy and SLE, presence of antiphospholipid antibodies, and need for potentially teratogenic medications can all complicate the management of pregnant patients with SLE. Studies demonstrate that, with careful planning, the majority of patients with lupus can complete pregnancy without serious complications. Recent developments are modified instruments to measure disease activity in pregnancy, increasingly common continuation of hydroxychloroquine during pregnancy, more frequent use of in vitro fertilization, and more aggressive fetal monitoring in patients positive for anti-Sjögren's syndrome (SS)-A/Ro or anti- SS-B/La antibody.

Breast Feeding↗
PubMed2006

Association of increased interferon-inducible gene expression with disease activity and lupus nephritis in patients with systemic lupus erythematosus.

OBJECTIVE: To study 5 type I interferon (IFN)-inducible genes (LY6E, OAS1, OASL, MX1, and ISG15) in patients with systemic lupus erythematosus (SLE) and to correlate expression levels with disease activity and/or clinical manifestations. METHODS: Peripheral blood cells were obtained from 48 SLE patients, 48 normal controls, and 22 rheumatic disease controls, and total RNA was extracted and reverse transcribed into complementary DNA. Gene expression levels were measured by real-time polymerase chain reaction, standardized to a housekeeping gene, and summed to an IFN score. Disease activity was determined by the Safety of Estrogens in Lupus Erythematosus: National Assessment-Systemic Lupus Erythematosus Disease Activity Index (SELENA-SLEDAI) composite. RESULTS: Each gene was highly expressed in SLE patients compared with normal controls (P < or = 0.0003) or disease controls (P < or = 0.0008 except for MX1). IFN scores were positively associated with the SELENA-SLEDAI instrument score (P = 0.001), the SELENA-SLEDAI flare score (P = 0.03), and the physician's global assessment score (P = 0.005). Compared with patients without nephritis, lupus nephritis patients had higher IFN scores (overall P < 0.0001), especially during active renal disease. IFN scores were weakly associated with neurologic manifestations. Elevated IFN scores were positively associated with the current presence of anti-double-stranded DNA (anti-dsDNA) antibodies (P = 0.007) or hypocomplementemia (P = 0.007). LY6E expression levels distinguished active from inactive lupus nephritis (P = 0.02) and were positively associated with proteinuria (P = 0.009). CONCLUSION: The 5 IFN-inducible genes were highly expressed in SLE patients, and increased levels were correlated with disease activity defined by several methods. IFN scores, or LY6E levels, were elevated in lupus nephritis patients, especially during active renal disease, and in patients with anti-dsDNA antibody positivity and hypocomplementemia. IFN scores, or LY6E levels, may be useful as a biomarker for lupus nephritis therapy.

Adult↗
PubMed2006

Systemic lupus erythematosus and pregnancy.

Women with systemic lupus erythematosus (SLE) face significant risks when embarking on a pregnancy, but attending a multidisciplinary clinic staffed by an experienced team can improve pregnancy outcome for women and their babies. Pregnancy in SLE should be planned and a management strategy should be agreed in full consultation with the patient, prior to conception. Pregnancy increases the likelihood of a lupus flare. It is not possible to predict when, or if, an individual patient will flare, although flare is more likely if disease has been active within 6 months of conception. Worsening of proteinuria in pregnancy could herald a lupus flare, but the differential diagnosis also includes the physiological response to pregnancy and pre-eclampsia. Corticosteroids, hydroxychloroquine and azathioprine are safe to use in pregnancy, with no adverse fetal effects reported despite many years of experience with their use. Correct identification of patients with antiphospholipid syndrome is important because treatment of affected women during pregnancy can improve fetal and maternal outcome. Neonatal SLE, although rare, carries a significant mortality and morbidity when the fetal heart is the targeted organ. Prophylaxis therapies, including treatment with intravenous immunoglobulin, await larger trials.

Female↗
PubMed1990

Systemic manifestations of systemic lupus erythematosus.

Twenty five cases with systemic lupus erythematosus admitted to a referral service hospital over a period of 6 years have been studied to analyse the pattern of multisystem involvement. Febrile polyarthritis, renal involvement and skin changes dominated the clinical picture. Important serological abnormalities included the presence of antinuclear antibody and anti ds DNA. Renal biopsy carried out in all cases helped to reveal lupus nephritis in subclinical cases. The pattern of renal involvement varied, with diffuse proliferative glomerulonephritis being the commonest. Oral steroids was given to all cases and cyclophosphamide was given to severe and resistant cases. The severity of system involvement, especially renal, influenced the response to treatment. Changes in presentation between Indian and Western patients are highlighted.

Adult↗
PubMed1973

Evidence for complement activation via the alternate pathway in skin diseases, I. Herpes gestationis, systemic lupus erythematosus, and bullous pemphigoid.

A patient with herpes gestationis, 6 of 6 patients with bullous pemphigoid, and 5 of 25 patients with systemic lupus erythematosus were found to have properdin deposited along the skin basement membrane.The patient with herpes gestationis demonstrated by immunofluorescence basement membrane deposition of C3 and C5 in the absence of C1q, immunoglobulins, and light chains. A second patient with herpes gestationis had C3 deposition with no demonstrable immunoglobulins or light chains. A thermolabile humoral factor(s) capable of depositing C3 (without C1q or C4) on normal skin basement membrane was found in the sera of both patients with herpes gestationis. No anti-basement membrane antibodies could be demonstrated in the sera of these patients.The patients with systemic lupus erythematosus and bullous pemphigoid who manifested properdin deposition also showed skin basement membrane deposits of C1q, C4, C3, C5, and immunoglobulins. C3 proactivator (C3PA) was also found deposited along the skin basement membrane of three patients with systemic lupus erythematosus and all six bullous pemphigoid patients. This study provides suggestive evidence that activation of complement is occurring via the alternate pathway in herpes gestationis. In systemic lupus erythematosus and bullous pemphigoid, both the classical (antibody) mediated activation of complement as well as the alternate pathway may be operative.

Adult↗
PubMed2003

Successful long-term treatment of systemic lupus erythematosus with rituximab maintenance therapy.

Systemic lupus erythematosus (SLE) is a chronic, inflammatory autoimmune disease that may involve multiple organ systems. Treatment consists of immunosuppression, cytotoxic treatment, plasmapheresis and immunoglobuline therapy. Treatment of patients refractory to standard treatment approaches is difficult and results are poor. We describe a 39-year old patient with SLE suffering from grand mal epilepsy due to cerebral vasculopathy with positive lupus anticoagulant, who was refractory to standard treatment modalities. The patient was treated with the anti-CD20 monoclonal antibody rituximab (375 mg/m2 x 4, repeated at weekly intervals). Rituximab applications were delivered in October 2000, March 2001 and October 2001. Since March 2002 she has received maintenance therapy with rituximab 375 mg/m2 every three months. A second female with refractory SLE was treated successfully in April 2002 and receives maintenance therapy every three months. Both patients responded well to rituximab therapy. The first patient showed a major improvement of her clinical condition, and 30 months after the beginning of the rituximab therapy she is free of any symptoms. Inflammation parameters, ANA and lupus anticoagulant declined significantly after the treatment. The clinical condition of the second patient improved dramatically, all inflammation parameters normalized and her circulating immunocomplexes disappeared. In conclusion, rituximab maintenance treatment may be a new effective therapy in SLE.

Adult↗
PubMed2004

Tacrolimus- (FK 506) based immunosuppression in severe systemic lupus erythematosus.

In a 30-year-old male patient systemic lupus erythematosus was diagnosed based on the presence of 8 out of 11 ARA criteria. Disease onset was acute and included renal function impairment with biopsy-proven lupus nephritis (WHO class IV) requiring renal replacement therapy. Although conventional immunosuppressive therapy regimens proved effective in controlling disease activity, all of the administered drugs were accompanied by serious side effects: bilateral femur head necrosis with corticosteroids, allergic skin reaction in response to azathioprine, nephrotoxicity with cyclosporine, nausea and abdominal pain with mycophenolate mofetil and life-threatening septicemia with cyclophosphamide treatment. In search for alternative treatment options, tacrolimus (FK506, trough serum levels 3-6 ng/ml) was started. FK506 was well-tolerated and lupus activity completely resolved within 7 months after initiation of therapy. During 36 months of follow-up no arthritic complaints occurred and renal function stabilized at a serum creatinine of 2.1 mg/dl with negative anti-ds-DNA antibodies and ANA titers. In conclusion, FK506 may be considered as alternative immunosuppressive for maintenance treatment in patients with severe lupus erythematosus and side effects to conventional regimens.

Adult↗
PubMed1980

Immune complexes and other laboratory features of pleural effusions: a comparison of rheumatoid arthritis, systemic lupus erythematosus, and other diseases.

We have evaluated the clinical usefulness of certain diagnostic tests in consecutive patients with pleural effusions, including 12 who had rheumatoid arthritis, nine who had systemic lupus erythematosus, and 39 who had other diseases. Effusions from patients with rheumatoid arthritis and systemic lupus erythematosus differed with respect to glucose level, lactic dehydrogenase activity, and pH. Complement levels differentiated rheumatoid from control effusions but did not segregate rheumatoid from systemic lupus erythematosus. Immune complexes were detected in all the rheumatoid pleural fluids by radioassays using monoclonal rheumatoid factor, C1q, and Raji cells. In most of the fluids, these complexes were reactive in all three assay systems and their levels higher than those found in paired serum samples. In effusions from patients with systemic lupus erythematosus, however, immune complexes were detected mainly by the Raji cell radioimmunoassay, and levels by all three assays were similar to those of paired serum samples.

Adult↗
PubMedDate not supplied

Immunology of cutaneous lupus erythematosus.

The cause and effect between ultraviolet light and cutaneous lupus erythematosus (CLE) is clear. In LE patients indeed, photosensitivity is one of the major diagnostic criteria of the systemic form of lupus erythematosus. This strong clinical association has led to the postulate that abnormal photosensitivity participates in the pathogenesis of cutaneous lesions in LE. What is not clear is how the ultraviolet radiation (UVR) induces cutaneous lesions in susceptible individuals despite the fact that profound effects of UVR on the cellular components of the skin have been extensively studied. The whole scenario is complicated by the relationship between sunlight and the cutaneous immune system. Pronounced effects of UVR on the cutaneous immune response further complicate the understanding of photosensitivity in LE. In addition, the network of cutaneous cytokines, chemokines, and adhesion molecules has become increasingly intricate, thus contributing to the genetic substrate of each individual, and to the tremendous complexity of the pathogenesis of CLE.

Humans↗
PubMed1988

Antimalarial drugs, systemic lupus erythematosus and pregnancy.

Antimalarial drugs containing the 4-amino quinoline radical are used to help control disease activity in discoid lupus erythematosus and systemic lupus erythematosus (SLE). Many patients with these complaints are young women, some of whom will become pregnant. The use of these substituted 4-amino quinoline compounds in pregnancy is controversial. We studied the full obstetric histories of 8 women with SLE who had taken either chloroquine phosphate or hydroxychloroquine sulphate (Plaquenil) throughout the entire length of at least 1 pregnancy. These 8 women had 14 pregnancies while receiving antimalarial drugs. Fetal wastage was high in these patients, regardless of antimalarial therapy, and was almost 100% in patients who were clinically active. Six normal full term spontaneous deliveries resulted from these pregnancies with clinically healthy normal babies born despite exposure to antimalarial therapy throughout the pregnancies.

Abortion, Spontaneous↗
PubMed1977

Lupus erythematosus profundus (Kaposi-Irgang) with monoclonal gammopathy.

The appearance of subcutaneous nodules of lupus erythematosus profundus on the face and neck of a woman with a long history of lupus erythematosus (LE) is reported. Of note were the three distinct stages in the course of the disease: (1) discoid LE, first appearing at the age of 42; (2) systemic LE, appearing 10 years later; (3) LE profundus, appearing 21 years after the diagnosis of discoid LE was made. The finding of monoclonal gammopathy is of particular interest, although its relationship to the basic disease has not been clarified.

Female↗