Acute Systemic Lupus Erythematosus Research Articles

Facial edema and crusted patches: A precursor to life-threatening acute systemic lupus erythematosus

To the Editor: Facial edema and crusted patches may be the initial presenting features of acute systemic lupus erythematosus (SLE). A 20-year-old African American man was admitted to the intensive care unit with increasing dyspnea and hemoptysis of 24 hours' duration. He had had fever and facial edema and rash of 1 week's duration. He was tachypneic and hypoxemic; chest radiography revealed extensive diffuse pulmonary bilateral infiltrates (Fig 1). A wide differential diagnosis was considered. Intensive supportive management of the patient was initiated, and he was empirically treated with broad-spectrum antibiotics.

Facial edema and crusted patches: A precursor to life-threatening acute systemic lupus erythematosus

Facial edema and crusted patches: A precursor to life-threatening acute systemic lupus erythematosus

Selected acute phase proteins and interleukin-6 in systemic lupus erythematosus patients treated with low doses of quinagolide

The relationship between endocrine regulation and immune system has recently become the subject of intense investigations. The objective of this study was to determine the extent of selected serum acute phase proteins (APP), IL-6 and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) involvement in systemic lupus erythematosus (SLE) patients during quinagolide therapy. A further aim of this study was to evaluate the relationships between the above mentioned parameters. In 25 SLE patients treated with a low dose of quinagolide (12.5–50 μg per day) and in 25 healthy persons who constituted the control group, serum concentration of C-creative protein (CRP), alpha-1-antitripsin (AAT), ceruloplasmin (CER), IL-6 and prolactin (PRL) were estimated at entry and in patients after 3 months of treatment. Moreover, SLEDAI score was calculated at entry and after 3 months of therapy with quinagolide. IL-6 and PRL levels were significantly higher in SLE group whereas the concentrations of CRP, AAT and CER were higher than in the controls, but without statistical significance. After 3 month therapy statistically significant decrease of serum level of IL-6 and PRL was revealed. Statistically significant lower serum concentration of CER was also obtained after 3 months of therapy whereas serum CRP and AAT concentration was lower compared with the mean pretreatment level but the results did not reach statistical significance. A raised SLEDAI score at entry was significantly reduced after 3 month therapy and positive correlation with PRL level in examined group of patients with SLE was noted at entry. The decreased serum concentration of IL-6, APP and SLEDAI score observed during applied therapy with small dose of quinagolide confirms the hypothesis that quinagolide may become a valuable and safe drug in the therapy of patients with mild SLE.

Clinical Significance of a Small Population of Paroxysmal Nocturnal Hemoglobinuria-Type Cells in the Management of Bone Marrow Failure

Although increased blood cell deficiency of glycosyl phosphatidylinositol-anchored membrane proteins has often been detected in patients with aplastic anemia (AA) and myelodysplastic syndrome (MDS), the clinical significance of such paroxysmal nocturnal hemoglobinuria (PNH)-type cells remains to be elucidated. We established a sensitive flow cytometric assay capable of detecting less than 0.01% of CD59-CD55- blood cells in a sample and used the assay to examine a large number of patients with bone marrow failure. An increase in the proportion of PNH-type cells was detectable in approximately 60% of all AA patients and in 20% of all refractory anemia (RA)-MDS patients. The increase was undetectable in patients with RA with an excessive number of blasts, acute myelogenous leukemia, multiple myeloma, or systemic lupus erythematosus. Our study showed that the presence of an increased number of PNH-type cells was predictive of a good response to immunosuppressive therapy and a favorable prognosis among patients with recently diagnosed AA and RA. A sensitive flow cytometric analysis for detection of a small population of PNH-type cells in peripheral blood cells is one of the most important examinations in the management of bone marrow failure.

What have we learned from a 10-year experience with the lumina (lupus in minorities; nature vs. nurture) cohort? Where are we heading?

Recently, there has been an awareness of the variable phenotypic expression of numerous disorders between individuals from different ethnicities, systemic lupus erythematosus (SLE) one of them. These disparities probably arise from the interaction between genetic and non-genetic (environmental, socioeconomic–demographic, cultural and behavioral) factors. To delineate the influence of these factors on SLE outcome, we established a multiethnic (Hispanic, African American and Caucasian) United States (US) early cohort (<5 years disease duration). Ten years later, interesting data have emerged from the LUMINA (Lupus in Minorities: Nature vs. nurture) cohort. For example, African Americans and Hispanics from Texas have a more severe disease than Caucasians and Hispanics from Puerto Rico. Lack of private insurance, acute SLE onset, expression of HLA-DRB1*01 (DR1) and C4A*3 alleles were associated with higher disease activity, whereas age, the number of American College of Rheumatology criteria met, disease activity, corticosteroid use and abnormal illness behaviors were consistent predictors of damage. In turn, damage and poverty were found to predict mortality. We now plan to apply new approaches (genetic admixture) to deconfound the complex interaction between genetic and non-genetic factors influencing SLE outcome. These data may have impact on the development of policies aimed at eliminating health disparities in the US.

Tireoidite bacteriana supurativa: relato de caso e revisão da literatura

Neste trabalho é apresentado um caso de tireoidite bacteriana aguda em um criança lúpica de nove anos de idade, em que o diagnóstico precoce foi imprescindível pela gravidade do quadro. Em seguida é apresentada uma revisão da literatura sobre o assunto.

Acute Pneumonia and Systemic Lupus Erythematosus

BUMC PROCEEDINGS 2001;14:88–93 CASE PRESENTATION MICHELLE V. SUN, MD: On March 29, 2000, a 40-year-old black woman with known systemic lupus erythematosus (SLE) presented to Baylor University Medical Center (BUMC) with fevers to 40°C (104°F), yellow to green diarrhea without blood, dyspnea, fatigue, headache, photophobia, cold sores, and a productive cough of 3 days’ duration. She was on chronic prednisone therapy. Five days before admission, she had received her first dose of a course of monthly intravenous cyclophosphamide for type IV lupus nephritis and autoimmune hepatitis. She denied any new rashes, chest pain, or other gastrointestinal or urinary complaints. Her SLE was originally diagnosed in October 1998. She was then lost to follow-up for over a year. On February 1, 2000, she was admitted to an outside hospital with complaints of fatigue, nausea, and jaundice and was found to have elevated liver function tests with an obstructive pattern. An abdominal ultrasound showed gallbladder thickening, and she subsequently underwent a laparoscopic cholecystectomy. At the time of her surgery, her liver was found to be fatty and possibly cirrhotic. The biopsy specimen was consistent with autoimmune hepatitis. During that hospitalization, she also developed acute renal insufficiency with a creatinine of 4.1 mg/dL. She was subsequently transferred to BUMC on February 12, 2000, for further care. Her C3 and C4 levels were both low, indicating active disease. She was started on pulse-dose steroids and underwent renal biopsy, with findings consistent with type IV lupus nephritis. Her hepatic and renal functions remained stable, and she was discharged home on February 23, 2000, on prednisone 40 mg twice a day, with a creatinine of 3.8 mg/dL. Intravenous cyclophosphamide was delayed at that time because of poor healing of her surgical wounds and persistent leak of ascitic fluid. On March 24, 2000, she had been readmitted for elective left subclavian Port-A-Cath placement and intravenous cyclophosphamide therapy (800 mg). She also received intravenous acyclovir for recurrent fever blisters. Her white blood cell count was 11.6 × 103/μL (5% lymphocytes and 88% neutrophils); hematocrit, 28%; platelets, 130 × 103/μL; and creatinine, 1.1 mg/dL. She was discharged home the following day on prednisone and oral acyclovir. The patient’s past medical history was significant only for hypertension and SLE as described above. Her current medications, all taken orally, included lansoprazole, 30 mg daily; furosemide, 40 mg daily; amlodipine, 10 mg daily; prednisone, 40 mg Acute pneumonia and systemic lupus erythematosus

Biological tests of physiologically active compounds (approaches): Communication II

This review considers the methods of investigation proposed by L. A. Piruzyan in the field of preclinical trials of physiologically active compounds: (1) the search for new physiologically active compounds illustrated by analysis of the developmental mechanisms of some diseases, such as jaundice of the newborn, renal and liver insufficiency, acute drug-induced intoxication, discoid and systemic lupus erythematosus, and lupus-like syndrome; (2) experimental studies on safety of medicinal substances—the testing of substances for mutagenicity and embryotoxicity, the preparation of compounds with antimutagenic and anticarcinogenic properties, the determination of preserved blood characteristics; (3) the stabilization of medicinal preparations for use—with drug oxidation inhibitors, and substances contained in liposomes. The ligand pathology concept is presented. The necessity of an individualized approach for assessment of chemical compound safety for humans in emphasized: it should be based on the activity determination of certain enzymes in each subject.

Outpatient management of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is often managed by primary care practitioners, who must coordinate the care with the support of subspecialists. The management of patients with such a serious and chronic disease can be both rewarding and challenging. This article reviews common problems and suggests management strategies. Measurement of serum antinuclear antibodies can lead to an erroneous diagnosis if used as the sole basis for diagnosis. Infections are the leading cause of death in lupus patients, and immunizations and antibiotic prophylaxis need to be considered. Acute cutaneous SLE is exacerbated by exposure to ultraviolet light. Patients should avoid sun exposure. Specific treatment of cutaneous SLE includes topical corticosteroids and antimalarial agents. Some of the most perplexing problems seen in SLE relate to neuropsychiatric features. NSAIDs, corticosteroids, and antimalarials are the most commonly used medications for SLE.

Pediatric Rheumatic Diseases

The rheumatic diseases of childhood are a relatively common and extraordinarily diverse group of illnesses; nevertheless, they are at least distantly related by similarities of immunodysregulation. These pathophysiologic relationships are reflected in affected children in similarities of historical, physical, and laboratory data as well as therapeutic intervention.

Clinical relapse in systemic lupus erythematosus: Correlation with antecedent elevation of urinary free light-chain immunoglobulin

This paper reports preliminary evidence suggesting that measurements of free light-chain Ig (FLIg) in urine may represent quantitative markers of in vivo polyclonal B-cell activation. Thus, longitudinal levels of urinary FLIg in patients with systemic lupus erythematosus (SLE) may be used to track or monitor the in vivo immunopathologic B-cell activity of SLE and be helpful in predicting a disease relapse. Our findings showed that dramatic rises in urinary FLIg occurred during asymptomatic intervals that preceded by 4-8 weeks the first symptomatic signs of acute SLE relapse. These results suggest that a sizable lead time may exist between the occurrence of immunopathologic B-cell stimulation and the resultant symptoms and tissue damage of immune complex-induced acute inflammation. In these studies the measurement of urinary FLIg was accomplished by an indirect method using ng-sensitive radioimmunoassays (RIAs) that measured isotypic IgG, IgA, IgM, total kappa-Ig, and total lambda-Ig. As a control for the assessment of renal tubular function and the excretion of low molecular weight proteins in SLE patients, longitudinal measurements of beta-2-microglobulin (B2M) and lysozyme were made using a novel solid-phase 3H-biotin RIA technique.

Functional changes of monocytes due to dialysis membranes

The functional effects directly induced by dialysis membranes on peripheral monocytes were analyzed in a plasma-free model of simulated dialysis using Cuprophan, cuprammonium rayon, polyacrylonitrile, polymethylmetacrilate and polysulphone membranes. A severe reduction of monocyte phagocytosis of IgG-coated erythrocytes was found by using Cuprophan and cuproammonium rayon. The cytofluorimetric analysis of several cell surface receptors, involved in the immune phagocytosis and recognizable by five quoted monoclonal antibodies, did not reveal any significant change. The defective phagocytosis of the IgG-coated erythrocytes by monocytes, due to the exposure to cellulose-derived membranes, was paralleled by an impaired interiorization of heat-aggregated human immunoglobulins, as analyzed by electron microscopy. The cell membrane binding of aggregated immunoglobulins was found to be unaffected. The defect was associated to a remarkably depressed generation of reactive oxygen species after Zymosan stimulation. Therefore, the defective immune phagocytosis induced by exposure of monocytes to cellulosic membranes was not due to a receptor rearrangement or an impaired binding of ingestible particles, but to a reduced internalization capacity probably related to an energy source exhaustion (as shown by the lack of response to stimuli able to induce oxidizing species production). These features are similar to those described in monocytes from acute systemic lupus erythematosus patients.

Cerebral and Retinal Vascular Changes in Systemic Lupus Erythematosus

A 16-year-old white boy with acute systemic lupus erythematosus (SLE) who presented with chorea and florid retinopathy died in renal failure three months after diagnosis. Pathological studies revealed two types of lesion in both the cerebral and ocular vessels. Some meningeal and choroidal vessels showed a typical vasculitis with fibrinoid necrosis, whereas other meningeal and retinal vessels were occluded by amorphous hyaline material in the absence of vasculitis.

The role of hypertension in the vascular disease and myocardial infarcts associated with murine systemic lupus erythematosus

The several kinds of mice that spontaneously develop acute systemic lupus erythematosus (SLE)--BXSB males, MRL/l males and females, and (NZB X W)F1 females--have a 15-20% incidence of degenerative vascular disease (DVD) and myocardial infarcts (MI) in which the affected coronaries contain deposits of immunoreactants, presumably in the form of immune complexes. Among the F1 hybrid crosses of SLE mice, only the (NZW X BXSB)F1, (W X B)F1 male has a significantly higher incidence of DVD/MI (80%). Search for possible causes of this high incidence of myocardial infarcts revealed several unique features of this mouse: hypertension, thrombocytosis, and early onset of circulating immune complexes and glomerulonephritis. Our attempts to prevent this DVD/MI focused on: reduction of hypertension, prevention of thrombosis, and immunosuppression. Immunosuppression by Cytoxan resulted in almost complete prevention of both the SLE disease and DVD/MI. Administration of bretylium, an antihypertensive and anti-arrhythmic agent, resulted in reduction of blood pressure and the severities of glomerulonephritis, DVD, and MI; it also slightly reduced the levels of circulating immune complexes and leukocytosis. Of the 4 antithrombotic agents used, only aspirin showed some reduction in the incidence of DVD/MI and delay of glomerulonephritis-associated mortality.

An immunoenzymatic assay for the detection and quantitation of platelet antibodies: The platelet β-galactosidase test (PGT)

An immunoenzyme assay for detection of platelet antibodies and suitable for routine use is described. Purified rabbit IgG anti-human IgG antibodies are conjugated to β-galactosidase with meta-maleimidobenzoyl-hydroxysuccinimide ester as a bifunctional reagent and o-nitrophenyl- β-galactopyranoside as a substrate to evaluate the enzymatic activity of the labeled antiglobulin. The sera of 26 patients suffering from various diseases (acute leukemia, aplastic anemia and systemic lupus erythematosus) and 40 control subjects were assayed with the enzyme-labeled reagent and, for comparison, with an indirect immunofluorescence technique. Half of these patients had never been transfused. Platelet antibodies were detected by both assays in all the transfused patients except one, and in 3 out of 13 non-transfused patients. The sera of all the control subjects were negative. Quantitation of platelet antibodies was obtained by a sensitive antiglobulin absorption technique. A method for standardization of the reagents allowing comparison of results obtained in the same patient at different times and suitable for long-term follow-up studies is also described.

Intravascular Coagulation in Acute Lupus Erythematosus

WE DESCRIBE a young woman with acute systemic lupus erythematosus (SLE) who also had complicating disseminated intravascular coagulation (DIC) that was both responsive to heparin and sensitive to its continued administration. Eventual response to simultaneously administered steroid therapy permitted successful discontinuance of anticoagulation treatment after 15 days. When last seen 1 1/2 years later, the patient was well while taking prednisone, 15 mg on alternate days. Report of a Case Following an attack of pharyngitis, a 19-year-old woman noticed small purpuric lesions on the fingers, cheeks, forehead, and ears. In the emergency department, her hematocrit value was 28.6%; white blood cell (WBC) count, 3,100/cu mm with 43 segmented neutrophils, 3 band cells, 49 lymphocytes, and adequate platelets; the corrected erythrocyte sedimentation rate was 32 mm/hr, and antistreptolysin O titer, 50 Todd units. A lupus erythematosus preparation was obtained and later reported as positive. She was instructed to return to the

Lupus erythematosus: Histopathology of cutaneous lesions

This paper describes the active and healing aspects of the pathology of chronic discoid and acute systemic lupus erythematosus. Emphasis is placed upon pathology of the basal laminar region, including especially thickening and vacuolopathy. The relationship between the two forms of the disease is discussed; the processes are usually separate in their evolution and course, but overlap between the two occurs. Problems in distinguishing acute systemic lupus erythematosus and dermatomyositis are also discussed.

Clinical study of 37 collected cases of acute systemic lupus erythematosus in Chulalongkorn medical school

Clinical study of 37 collected cases of acute systemic lupus erythematosus in Chulalongkorn medical school

Hemorrhagic bullae in gonococcemia.

CHARACTERISTIC cutaneous lesions can be the clue to the diagnosis of gonococcal septicemia. Frequently, these sparse, acral lesions occurring in young adults, especially women, who have fever, polyarthralgia, and tenosynovitis, are dismissed as folliculitis or regarded as manifestations of flu, acute rheumatic fever or systemic lupus erythematosus. The skin lesions of gonococcemia are virtually specific.1 Heralded by splinter-like pain, the fully developed lesions have been emphasized as being of two types: purpuric and vesiculopustular on broad erythematous bases.2 , 3 A third variant, hemorrhagic bullae, has not been described since the advent of antibiotics.4 The most prominent skin lesions in two recently . . .

Systemic Lupus Erythematosus Complicated by Coccidioidomycosis

In 1950 this patient was treated with cortisone acetate for acute systemic lupus erythematosus (SLE). A state of remission was obtained until June 1963 when the patient moved to Tucson, Ariz. At this time, there was a relapse of the SLE. By Sept 30, pulmonary coccidioidomycosis developed, and by February 1964 the patient manifested the disseminated form of this disease. By Dec 15, a total of 2,285 mg of amphotericin B had been administered. Both diseases have remained in remission with residuals of nephrotic syndrome, persistent anemia, and leukopenia.