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S Manzi

Publications and source records attributed to S Manzi.

45 records · Page 3Linked to original sources

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↗

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↗

Antiphospholipid antibodies in pediatric systemic lupus erythematosus.

OBJECTIVE: Antiphospholipid antibodies (aPLs) have been extensively studied in adults with systemic lupus erythematosus (SLE) and have been associated with arterial and venous thrombosis, thrombocytopenia, neurologic disorders, and recurrent fetal loss. In contrast, very little is known about the frequency and clinical significance of aPLs in pediatric SLE. This study was designed to determine the frequency of aPLs in pediatric SLE and the temporally associated clinical manifestations. DESIGN: We studied 29 consecutive patients with onset of SLE in childhood seen in the Pediatric Rheumatology Clinic at the University of Pittsburgh, Children's Hospital, between 1985 and 1992. We defined aPL as the presence of a lupus anticoagulant (LAC), immunoglobulin G or immunoglobulin M anticardiolipin antibodies (aCLs), or a biologic false-positive serologic test for syphilis determined by a VDRL test. Clinical manifestations were temporally correlated to the presence of aPLs if they occurred within 6 months. RESULTS: Overall, 19 (65%) of 29 children with SLE had one of the three laboratory abnormalities defining aPL. LAC was detected in 16 (62%) of 26, aCL in 18 (66%) of 27, and false-positive VDRL test results in 11 (39%) of 28. Twenty-five of the 29 patients had all three tests performed. In 10 patients, all three tests were abnormal. The presence of thrombosis in 7 patients (4 venous, 2 arterial, and 1 both) was associated with a positive aPL, specifically aCL. The presence of an aPL was significantly associated with anti-double-stranded DNA antibodies, but not with neuropsychiatric manifestations or with thrombocytopenia. The presence of an aCL was significantly associated with hemolytic anemia. A prolonged prothrombin time, in the setting of an LAC (all with a prolonged activated partial thromboplastin time), was associated with life-threatening disease in 6 of 15 patients. CONCLUSION: Sixty-five percent of 29 consecutive pediatric patients with SLE had evidence of aPL. The presence of aPL, specifically aCL, was significantly associated with thrombotic events. The presence of a prolonged prothrombin time in the setting of an LAC may be a marker of more serious disease in pediatric SLE.

Adolescent↗

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↗

Complement C3 fragments in urine: detection in systemic lupus erythematosus patients by western blotting.

We characterized urinary excretion of C3 fragments among patients with systemic lupus erythematosus (SLE) as a possible indicator of renal involvement. 28 patients, representing a broad range of disease activity were admitted to our study. Urinary proteins were separated on 4-20% gradient SDS-PAGE gels, under reducing conditions, and transblotted to nitrocellulose. Western blots were developed with a polyvalent goat-anti-human C3d antiserum, and an alkaline phosphatase-conjugated rabbit anti-goat IgG. Three patterns were obtained: 1) no bands detected; 2) bands suggesting the presence of intact C3; and 3) samples with additional low molecular (< 4 x 10(4)) bands. The 12 patients with no C3 bands had minimal disease activity (e.g. fatigue, arthralgia, arthritis, rash, oral ulcers). The seven patients with intact C3 patterns also had minimally active disease. Their primary clinical findings included fatigue, pleurisy, renal disease which had been treated, hemolytic anemia, and arthritis. Patients with low molecular weight C3 fragments in their urine formed two sub-sets, based upon their presenting features. The first group had severe disease and contained all patients with active lupus nephritis (n = 4), while the second consisted of non-renal patients with primary clinical findings of moderate disease activity (e.g. thrombocytopenia, pneumonitis, arthritis). Our results suggest urinary excretion of low molecular weight C3 fragments correlates with active renal disease, but is a variable finding among SLE patients with non-renal manifestations of disease activity.

Blotting, Western↗

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↗

Outcome of the in vitro studies on drug absorption employing the isolated rat small intestine.

The in vitro methods for studying the intestinal absorption of drugs involving the use of the isolated rat small intestine are reviewed in order to provide indications on their actual usefulness and limitations. The most significant works dealing with the structural integrity of the intestinal mucosa, the functional integrity of the isolated intestine, the factors influencing passive transport such as pH, sugars, ions, physiological and synthetic surface-active agents, and complexation, are reviewed.

Animals↗