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Biomedical subjects

R Ramsey-Goldman

Publications and source records attributed to R Ramsey-Goldman.

At least 55 records · Page 3Linked to original sources
PubMed1996

Sensitivity and specificity of plasma and urine complement split products as indicators of lupus disease activity.

OBJECTIVE: To determine if measurement of serum complement split products (C4d, Bb, C5b-9) is better than conventional C3 and C4 measurements in distinguishing patients with varying degrees of lupus disease activity, and to determine if the presence of C3d in urine is helpful in distinguishing lupus patients with from those without early lupus nephritis. METHODS: Lupus disease activity was prospectively determined at 3 consecutive visits an average of 4 months apart, using the Systemic Lupus Activity Measure (SLAM), the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), and physician global assessment (PGA). Blood samples were evaluated for the presence of C4d, Bb, and C5b-9 by quantitative microassay plate enzyme immunoassay at each patient visit. We characterized urinary excretion of C3 fragments (with attention to C3d) by sodium dodecyl sulfate-polyacrylamide gel electrophoresis with Western blotting. RESULTS: Thirty-one SLE patients were enrolled in the study. The mean SLAM score and the mean SLEDAI score each correlated well with the PGA at all 3 visits. A SLAM score of 6 and a SLEDAI score of 4 had the best overall sensitivity and specificity for predicting moderate-to-severe disease activity by PGA (100% and 73%, respectively, for the SLAM and 86% and 94%, respectively, for the SLEDAI). Serum C4d and Bb were more sensitive indicators of current moderate-to-severe lupus disease activity at all 3 visits than were serum C5b-9, C3, and C4. C3 and C4 were more specific indicators of moderate-to-severe disease activity. Serum C4d and Bb were more sensitive at predicting moderate-to-severe disease activity at subsequent visits than were C5b-9, C3, and C4. Urine C3d was better than C3, plasma C4d, Bb, C5b-9 and anti-double-stranded DNA antibody in distinguishing patients with from those without acute lupus nephritis (P = 0.02). CONCLUSION: C4d and Bb are sensitive indicators of moderate-to-severe lupus disease activity and may be most helpful in situations where conventional measurements are not, such as in lupus patients whose C3 and C4 levels remain normal despite evidence of clinical disease activity. It appears from this study that detection of urine C3d may be a simple way of measuring complement activation in the setting of lupus renal disease. The availability of instruments for clinical disease activity measurement such as the SLAM and the SLEDAI may enable more consistent definition of lupus disease activity and may thus provide a means for better examining the role of complement activation products in predicting lupus disease activity in larger patient populations.

Adult↗
PubMed1996

Ethnicity and access to care in systemic lupus erythematosus.

OBJECTIVE: To define access to care and to examine the relationship between ethnicity and access to care in systemic lupus erythematosus (SLE). METHODS: A review of published literature was supplemented with preliminary data from a pilot study. Data from patient interviews, chart reviews, and insurer surveys were collected at 2 sites and used to develop several measures of access. The relationship between ethnicity and access was examined through chi-square analyses, difference of means testing, and multivariate regression. RESULTS: Although African-American SLE patients appear less likely to be privately insured and more likely to be uninsured, no significant differences in utilization rates were detected between ethnic groups. Uninsured patients, however, had significantly fewer physician visits than both the Medicaid and the privately insured patients. Multivariate regression confirm a strong and negative relationship between physician visits and patient coinsurance rates. CONCLUSION: Careful examination of multiple dimensions of access may highlight differences between ethnic groups. Further research is necessary to document these differences and explore their relationships to outcomes.

Adolescent↗
PubMed1996

Retroviral risk factors in patients with autoimmune disease.

OBJECTIVE: Retroviruses can cause immunoregulatory disturbances and may play a role in the pathogenesis of autoimmune disorders. Little is known about the frequency of behavioral risk factors for exogenous retroviral infections in patients with autoimmune diseases. We compare the frequency of recognized risk factors for retroviral infections among patients with systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and controls. METHODS: Patients with SLE and RA from a university rheumatology clinic and control patients were enrolled in this study. The presence of retroviral risk factors (intravenous drug use, prostitution, increased number of sex partners, sexually transmitted diseases, high risk sex partners, blood transfusion) was determined by a self-administered questionnaire. RESULTS: We surveyed 81 patients with SLE and 117 with RA and 100 healthy controls. Patients in all groups reported similar exposure to all risk factors surveyed for retroviral infection except sexually transmitted disease, which was reported more often in patients with SLE (25% of SLE versus 11% of RA and 11% of controls, p = 0.013). CONCLUSION: Self-reported retroviral risk factors were generally not increased in patients with autoimmune disease compared to healthy controls; the role of exogenous retroviruses in the pathogenesis of SLE and RA remains unclear.

Adolescent↗
PubMed1995

Incidence of systemic lupus erythematosus. Race and gender differences.

OBJECTIVE: To examine racial differences in the incidence of systemic lupus erythematosus (SLE). METHODS: A population-based registry of SLE patients in Allegheny County, Pennsylvania, was used to identify incident cases of SLE diagnosed between January 1, 1985 and December 31, 1990, from 3 sources, by medical record review (University of Pittsburgh Lupus Databank, rheumatologists, and hospitals). Capture-recapture methods using log-linear models were used to estimate the level of case-finding and to calculate 95% confidence intervals (CI). Incidence rates were calculated per 100,000 population. RESULTS: A total of 191 definite and 78 probable incident cases of SLE were identified, and the overall annual incidence rates were 2.4 (95% CI 2.1-2.8) and 1.0 (95% CI 0.8-1.3), respectively. The crude incidence rates of definite SLE were 0.4 for white males, 3.5 for white females, 0.7 for African-American males, and 9.2 for African-American females. The annual incidence rates of definite SLE remained fairly constant over the study interval. African-American females with definite SLE had a younger mean age at diagnosis compared with white females (P < 0.05). Since the overall ascertainment rate was high (85%; 95% CI 78-92%), the ascertainment-corrected incidence rate for definite SLE, 2.8 (95% CI 2.6-3.2), was similar to the crude rate. CONCLUSION: Our rates clearly confirm previous reports of an excess incidence of SLE among females compared with males and among African-Americans compared with whites. We have used capture-recapture methods to improve the accuracy of SLE incidence rates, and we advocate their use to facilitate comparisons across studies.

Adolescent↗
PubMed1995

Risk of malignancy in women with systemic lupus erythematosus.

OBJECTIVE: To estimate the risk of malignancy in a cohort of patients with systemic lupus erythematosus (SLE) from a university medical center. METHODS: The cohort consisted of patients with lupus who were residents of Allegheny County and who were seen at the University of Pittsburgh, 1981-91. Cases of cancer were identified by a postal survey and review of medical records. The expected number of malignancies in the lupus cohort were estimated from age, sex, and race adjusted census and cancer incidence data from Allegheny County. Standardized incidence ratios and 95% confidence intervals for all cancers were calculated. RESULTS: Six (2.7%) malignancies were observed in the 219 lupus patients from Allegheny County during the observation interval (1981-91) and after the first visit at the University of Pittsburgh. The expected number of malignancies was 4.42. The standardized incidence ratio for cancer in the lupus cohort from Allegheny County was 1.36 (95% confidence interval 0.50-2.96). The frequency of malignancy was not increased in the small number of patients who had received immunosuppressive drugs before the diagnosis of cancer. Non-Hodgkin's lymphoma occurred in one patient, who also had Sjögren's syndrome, and in one patient before entry into this study who was treated with FK-506 following renal transplant. No bladder cancers were observed during the study interval. CONCLUSION: The overall frequency of malignancy was not increased in this medical center cohort of patients with lupus during a mean followup interval of 5.2 yrs. Longterm followup and a multicenter effort is needed to refine risk estimates of cancer in patients with lupus.

Adult↗
PubMed1995

Herpes zoster in systemic lupus erythematosus.

OBJECTIVE: To define the clinical spectrum and disease sequelae of herpes zoster and to determine the risk factors associated with the development of herpes zoster in patients with systemic lupus erythematosus (SLE). METHODS: Retrospective matched case control study in a consecutive series of patients with SLE first evaluated between 1979 and 1989. Patients were classified as cases if their first episode of zoster occurred after lupus diagnosis. Lupus patients who never had zoster were eligible as controls and were matched 2:1 to cases for age, race, sex, and survival status. Clinical features of the cases from the time of lupus diagnosis to the time of zoster were compared to their respective controls over similar time periods. RESULTS: Forty eight (15%) of 321 patients were classified as cases. Cases were more likely to have received cyclophosphamide (p = 0.03), and azathioprine (p = 0.006). More cases had lupus nephritis (p = 0.02), and a concurrent or previous malignancy (p = 0.01) than their controls. Seven cases had cutaneous dissemination. Seven patients had postherpetic neuralgia > 2 months and in only 2 patients symptoms persisted for > 12 months' duration. Only 3 of 36 patients had immunosuppressive medication discontinued at the time of diagnosis of zoster, and 10 cases received acyclovir for the zoster infection. There were no permanent neurologic deficits or death. CONCLUSION: Immunosuppressive therapy, specifically cyclophosphamide and azathioprine, lupus nephritis, and a concurrent or previous malignancy may be risk factors for the development of herpes zoster infections in patients with SLE. Our study suggests that although herpes zoster occurs frequently in patients with SLE, it has a relatively benign course. Discontinuing needed immunosuppressive therapy in patients with SLE may be unnecessary in the setting of a zoster infection. With the current emphasis on reduction in medical costs, both by limiting inpatient admissions and eliminating unneeded medications, it is necessary to identify which patients require more intensive therapy with antiviral medications and/or hospitalization and which are likely to have a benign, self-limited course without intervention.

Adolescent↗
PubMed1993

Pregnancy outcome and anti-cardiolipin antibody in women with systemic lupus erythematosus.

Pregnancies in women with systemic lupus erythematosus are recognized to result in excessive fetal morbidity and mortality. Maternal autoantibody status may explain some of these problems. Anti-cardiolipin antibody has been associated with recurrent pregnancy losses in some women with lupus, but the risk of these losses has not been defined. At the University of Pittsburgh between January 1, 1979, and December 31, 1989, an unmatched case-control study design was used to determine whether patients with lupus and anti-cardiolipin antibody (81 cases) were at increased risk for adverse pregnancy outcomes in comparison with lupus patients without the antibody (174 controls). Cases had 98 of 192 (51%) pregnancies with an adverse outcome, while controls had 212 of 494 (43%). The odds ratio for having any adverse pregnancy outcome was 1.40 (95% confidence interval (CI) 0.98-1.98). When pregnancies were classified according to specific adverse outcome types, the frequency of late miscarriages (14-20 weeks gestation) in cases and controls was 8% and 3%, respectively. The odds ratio for late miscarriage was 2.94 (95% CI 1.31-6.60). When pregnancies were stratified by birth number and by occurrence of pregnancy before or after diagnosis, the increased frequency of late miscarriages in cases was noted only in the first pregnancy when the pregnancy occurred before recognized disease. Preterm births (before 38 weeks gestation) were increased in cases compared with controls in pregnancies that occurred after diagnosis for second and third pregnancies. If a case had one previous adverse outcome, the odds ratio for another adverse outcome was 3.00 (95% CI 1.62-5.57). If a case had two previous adverse outcomes, the odds ratio for a third adverse pregnancy outcome was 4.14 (95% CI 1.62-10.58). Thus, a previous adverse pregnancy outcome was the most important risk factor for an adverse outcome in a subsequent pregnancy.

Adult↗
PubMed1993

Pregnancy outcome in women with systemic lupus erythematosus treated with immunosuppressive drugs.

OBJECTIVE: The aim of this retrospective study was to assess the impact of immunosuppressive drugs (ISD) on pregnancy outcome in women with systemic lupus erythematosus (SLE). METHODS: Pregnancy outcome, disease manifestations, and medication use were ascertained by interview and review of the medical record. Three hundred thirty-four (88%) of 380 consecutive women with lupus seen during 1979-1989 at the Lupus Diagnostic and Treatment Center at the University of Pittsburgh participated in this study. RESULTS: Adverse pregnancy outcomes were noted in 59 of 113 (52%) pregnancies in patients with pregnancies after diagnosis of SLE and no exposure to ISD (Group 1) and in 10 of 23 (43%) pregnancies in patients with pregnancies after diagnosis of SLE and with ISD use prior to or during pregnancy (Group 2). Only 3 of 23 or 13% pregnancies in Group 2 resulted in a fetal or neonatal loss compared to 27% in Group 1 (NS). No history of malignancy has been noted in the longterm followup of the surviving children in Group 2 (current mean age is 6.1 years, range 1.5-13 years). CONCLUSIONS: Although overall survival was encouraging in Group 2, questions remain about the safety and longterm mutagenic effects of ISD in women with SLE. Judicious use of these medications prior to or during pregnancy is warranted.

Abortion, Spontaneous↗
PubMed1991

Systemic lupus erythematosus in a boy with chronic granulomatous disease: case report and review of the literature.

We describe a patient with X-linked chronic granulomatous disease (CGD) who developed systemic lupus erythematosus, which was characterized by photosensitivity, malar rash, glomerulonephritis, leukopenia, hypocomplementemia, antinuclear antibodies, and anti-double-stranded DNA antibodies, at age 3. The patient's mother is an asymptomatic carrier of CGD, and her other son (the patient's half-brother) also has CGD. Neither the mother nor the brother has clinical or serologic evidence of systemic lupus erythematosus. Previous cases of discoid lupus-like skin lesions have been reported both in carriers and in patients with CGD. Our patient represents the first reported case of an individual with convincing clinical, serologic, and pathologic evidence of systemic lupus erythematosus. The association between defective host defense mechanisms and autoimmune phenomena has been described previously in patients with Job's syndrome and in patients with B cell and T cell deficiency disorders, including the acquired immunodeficiency syndrome. The relationship between the known leukocyte defects in CGD and the pathogenesis of a lupus-like illness is unclear.

Child, Preschool↗
PubMed1990

Pregnancy outcomes in women with primary Raynaud's phenomenon.

Pregnancy outcomes were assessed in 67 women who had well-defined primary Raynaud's phenomenon and in 30 controls, each of whom had been pregnant at least once. Twenty-seven patients had onset of Raynaud's phenomenon prior to any pregnancy, and 37 patients had onset of Raynaud's phenomenon after their last pregnancy. The frequency of miscarriage, stillbirth, and small full-term infants was similar in all 3 study groups. Among the 4 Raynaud's phenomenon patients who had second-trimester miscarriages, 2 of 3 who were tested had moderately positive levels of IgG or IgM anticardiolipin antibody. Premature births were significantly more common in pregnancies that occurred after the onset of Raynaud's phenomenon (24%) than in pregnancies that occurred prior to the onset of Raynaud's phenomenon or in pregnancies among control subjects (9% and 1%, respectively). In addition, the mean weight of the full-term babies of women in both Raynaud's phenomenon groups was significantly less than that of babies born to controls. There were no neonatal deaths or other serious adverse outcomes. These findings may represent yet another manifestation of systemic vasospasm in patients with primary Raynaud's phenomenon.

Abortion, Spontaneous↗
PubMed1990

Hydralazine induced lupus and Sweet's syndrome. Report and review of the literature.

This is the second case report of hydralazine induced Sweet's syndrome and lupus erythematosus. Our patient had the characteristic rash of Sweet's syndrome, confirmed histopathologically, as well as laboratory features consistent with drug induced lupus, including positive serum antinuclear and antihistone antibody tests. The association between these 2 clinical entities is discussed and a review of the literature is presented.

Acute Disease↗
PubMed1989

Pregnancy in women with systemic sclerosis.

We assessed fetal morbidity and mortality in women with systemic sclerosis (SSc). Women with a history of SSc and a concomitant pregnancy completed a detailed questionnaire about the pregnancy. These 48 subjects were age-matched and race-matched to 2 other groups of women (a rheumatoid arthritis group and a control group from the same neighborhood), all of whom had been pregnant at least once. There were no differences in the frequencies of miscarriage or perinatal death in the SSc group compared with the 2 control groups. Preterm births occurred slightly more frequently in both SSc patients and rheumatoid arthritis patients compared with the neighborhood control subjects. There were significantly more small full-term infants born to women with SSc. Interestingly, the increase in preterm births and small full-term babies occurred with equal frequency prior to and after the onset of disease. Although close monitoring for premature birth and intrauterine growth retardation is necessary, we conclude that an uneventful, healthy pregnancy is possible for women with SSc. Those with early, rapidly progressive, diffuse skin thickening should avoid becoming pregnant since, intrinsically, they are at higher risk of developing renal crisis.

Abortion, Spontaneous↗
PubMed1988

Phenothiazine-induced systemic lupus erythematosus with superior vena cava syndrome: case report and review of the literature.

Phenothiazine-induced lupus with circulating anticoagulant (CAC) has been reported infrequently, and is rarely associated with significant symptoms. We describe a patient who we suspect had perphenazine-induced lupus erythematosus with CAC. She had recurrent episodes of venous thrombosis and ultimately developed superior vena cava syndrome. The relationships among phenothiazine-induced lupus, CAC, and thrombosis are discussed.

Adult↗
PubMed1988

Syndrome of diffuse abnormal insertional activity: case report and family study.

In 1979 Wiechers and Johnson described ten patients with diffuse abnormal insertional activity on EMG examination in the absence of neuromuscular disease. We present a family group with identical findings. The propositus is a 53-year-old woman who presented with back pain. EMG studies revealed trains of positive sharp waves with needle movement in all muscles studied. Nerve conduction studies, radiographs, and laboratory studies were all unremarkable. We recruited eight additional family members who underwent a screening EMG of five muscles. Four patients had trains of positive sharp waves present in all five muscles. To our knowledge, this is the first report confirming the findings of Wiechers and Johnson. We concur with them that the abnormality appears to be genetically transmitted in an autosomal dominant pattern. Although without clinical significance, it is important for electromyographers to be aware of this entity so as not to mistakenly ascribe serious neuromuscular disease to these patients.

Adolescent↗
PubMed1988

Pregnancy in systemic lupus erythematosus.

Issues concerning contraception, fertility, and pregnancy usually arise during a typical lupus patient's disease course. Pregnancy superimposed on established lupus may alter the course of the disease, and, conversely, lupus may affect the natural history of pregnancy. Two recently described autoantibody markers, anti-SSA (Ro) and anticardiolipin, have provided new insights concerning fetal risks in these patients. Furthermore, they should lead to improved understanding of mechanisms of tissue injury and to new ideas about therapeutic interventions and/or prevention of pregnancy complications.

Adrenal Cortex Hormones↗
PubMed1987

Localization of Ro (SS-A) antigen in the cardiac conduction system.

The presence of anti-Ro (SS-A) antibody in maternal serum has been associated with congenital heart block. Using human sera monospecific for anti-Ro (SS-A) antibodies in an indirect immunofluorescence assay, Ro (SS-A) antigen was found to be present in the nuclei of myocardial cells and cells of the cardiac conduction system. The possible relationship between the location of Ro (SS-A) antigen, anti-Ro (SS-A) antibody, and heart block is discussed.

Autoantigens↗