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Biomedical subjects

J C Nossent

Publications and source records attributed to J C Nossent.

At least 37 records · Page 2Linked to original sources
PubMed1992

Systemic lupus erythematosus.

The reported change in the incidence of systemic lupus erythematosus (SLE) is mainly due to a greater awareness of the disease, and to the introduction of serological detection methods such as the LE cell assay and subsequently a variety of other antinuclear antibody assays. SLE is seldom preceded by rheumatoid arthritis, nor does SLE often develop in patients with rheumatoid arthritis. In a substantial proportion of our SLE patients, discoid LE occurred first. On analyzing the literature no evidence could be found that nowadays less severe SLE is diagnosed. Also, in the last three decades no change has been observed in the prevalence of clinical features in large groups of patients with SLE. These data indicate that there has been no change in the expression or prognosis of SLE in recent decades.

Arthritis, Rheumatoid↗
PubMed1992

Systemic lupus erythematosus on the Caribbean island of Curaçao: an epidemiological investigation.

To determine the incidence, prevalence, and outcome of systemic lupus erythematosus (SLE) in a well delineated black population in the Caribbean basin data were collected on the disease course of all patients with definite SLE seen during a 10 year period (1980-9) using three different sources of information (hospital records, private practice records, and death certificates). Ninety four patients were identified giving an average annual incidence rate of 4.6/100,000 (95% confidence interval (CI) 0.4 to 8.8), which showed little variation during the study period. Twenty five patients (27%) died during the study period, giving a point prevalence of 47/100,000 (CI 34.1 to 51.1) in 1990. In women aged 15-44 years the annual incidence (12/100,000; CI 5.3 to 18.9) was highest, whereas in women aged 44-65 years the 1990 point prevalence rate (one in 526; CI 469 to 625) was highest. Annual mortality was 1.7/100,000 (CI -0.8 to 4.2) with a female to male ratio of 5.3. Renal disease was the most common complication, occurring in 73 (78%) patients. Thus the transatlantic movement from an area with a (presumably) low prevalence of SLE (Central Africa) has been accompanied by an increase in the prevalence of SLE in the black population of Curaçao, indicating that environmental factors may prevail over genetic factors in the expression of this disease.

Adolescent↗
PubMed1992

Pneumatosis intestinalis in mixed connective tissue disease.

Two young female patients with established mixed connective tissue disease (MCTD) are described, in whom the disease course was complicated by intestinal pseudo-obstruction and pneumoperitoneum due to pneumatosis intestinalis. Conservative management of this rare complication in MCTD, which included the use of high-flow normobaric oxygen by mask, led to a complete resolution of the abdominal symptoms in both patients.

Adult↗
PubMed1991

Single-photon-emission computed tomography of the brain in the evaluation of cerebral lupus.

We sought to determine whether single-photon-emission computed tomography (SPECT) of the brain is useful for detecting abnormalities of regional cerebral blood flow in patients with cerebral lupus. Twenty lupus patients with clinical evidence of cerebral involvement underwent SPECT and CT scanning of the brain, as well as clinical, expert neurologic, and serologic evaluation. Fifteen patients (75%) had a clear regional cerebral hypoperfusion. Seven of 8 patients (88%) who were ultimately thought to have active cerebral lupus had abnormal SPECT scan findings, while 8 of 12 patients (67%) who were ultimately thought not to have active cerebral lupus had abnormal SPECT scan findings. There was no correlation of SPECT findings with CT scan results, overall disease activity, or serologic findings. Regional cerebral blood flow measured by SPECT is often abnormal in patients with active cerebral lupus, but is also frequently abnormal in lupus patients with neuropsychiatric symptoms not attributable to cerebral lupus activity.

Adult↗
PubMed1991

Relation between serological data at the time of biopsy and renal histology in lupus nephritis.

As autoantibodies are thought to participate in the pathogenesis of renal inflammation in systemic lupus erythematosis (SLE) we investigated associations between serological markers of disease activity in SLE and the activity of renal histopathological lesions in thirty-five patients with lupus nephritis (LN). We found the following prevalence of serum auto-antibodies in LN: IgG antinuclear antibodies (ANA) 100%, IgM ANA 69%, IgA ANA 60%, IgG anti-dsDNA 60%, IgM anti-dsDNA 71%, IgA anti-dsDNA 60%, anti-RNP 20%, anti-Sm 14%, anti-SSA 31%, anti-SSB 14%, anti-histone 37%, anti-cardiolipin 80% and antibody to ribosomal protein (anti-P) 6%. No correlation was found between serological parameters and the WHO-classification of biopsies. The activity-index of histological lesion, assessed according to the NIH-renal histology scoring system, correlated with IgM ANA and IgM anti-dsDNA titers. Of all the specific features of histological renal inflammation, glomerular proliferation showed the best overall correlation with serological parameters of disease activity. Anticardiolipin antibodies were correlated with overall disease activity, but not with renal histological activity. Thus, serological markers of disease activity did not adequately reflect the amount of renal inflammation in LN and cannot replace renal biopsy as a diagnostic tool.

Adolescent↗
PubMed1991

Prognostic factors in systemic lupus erythematosus.

In the past 40 years an impressive improvement in the prognosis of SLE patients has occurred. Factors which might be responsible for this improvement are discussed. Two of the factors most frequently cited are the advances in disease recognition and treatment. However, as already noted by Albert (1979) this is questionable, as average disease duration and survival have increased in a linear fashion related to the number of publications devoted to this subject from 1950 on. Further evaluation of the literature shows that the most prominent factors which have an impact on the survival rate are specific disease manifestations (lupus nephritis) and the overall disease course (number of exacerbations). This effect of morbidity on the survival rate is greater than that of factors such as sex and race. Socio-economic factors or age at onset have no effect on the outcome.

Adrenal Cortex Hormones↗
PubMed1991

Systemic lupus erythematosus--changing disease patterns in the disease course. Dutch experience with 110 patients studied prospectively.

The aim of this study was to investigate which patients with systemic lupus erythematosus (SLE) are prone to develop more than one exacerbation, and to establish the variability in the clinical symptoms during exacerbations as compared with the initial symptoms of the disease. At disease origin, photosensitivity, pleuritis and Raynaud's phenomenon were slightly increased in the patients with a stable disease, while pericarditis was rarely seen in patients with a remitting disease course. In this prospective study it was clearly shown that during the disease course, depending on the exacerbation frequency, an increasing number of organs were involved. Striking features were the increase in haematological abnormalities in the third exacerbation, and the fact that psychosis and seizures did not recur in the second exacerbation when they were diagnosed in the preceding period. We also showed that symptoms seen in an exacerbation may be quite different from those seen in a previous exacerbation.

Adult↗
PubMed1991

Daily stressors and systemic lupus erythematosus: a longitudinal analysis--first findings.

Patients with systemic lupus erythematosus (SLE) and a comparison group of patients with rheumatoid arthritis (RA) completed the Dutch Everyday Problem Checklist and the Dutch Arthritis Impact Measurement Scale measuring physical and psychosocial aspects of illness. Both scales were completed 9 times at 6-week intervals. In addition several hematological and serological parameters were assayed. Analysis over several time points resulted in only weak correlations between the variables under study. The most remarkable finding of this study was that in SLE patients number and intensity of daily stressors are more strongly related to physical and psychosocial status than in RA patients.

Activities of Daily Living↗
PubMed1991

Cognitive and emotional disturbances in systemic lupus erythematosus.

Do patients with central nervous system systemic lupus erythematosus (CNS-SLE) demonstrate more cognitive and emotional disturbances than SLE patients without cerebral involvement (non-CNS-SLE) and patients with rheumatoid arthritis (RA)? Test results indicated that CNS-SLE patients performed less well on two concentration subtests in comparison with both non-CNS-SLE and RA patients and scored higher on three psychopathology scales (borderline personality symptoms, dysthymic symptoms and psychotic depression). The representativity and reliability of the present results will be discussed.

Affective Symptoms↗
PubMed1991

Prevalence and significance of haematological abnormalities in patients with systemic lupus erythematosus.

The occurrence and significance of haematological abnormalities were analysed in 126 prospectively studied patients with systemic lupus erythematosus. Haemolytic anaemia occurred in 13 per cent, neutropenia in 47 per cent, lymphocytopenia in 20 per cent and thrombocytopenia in 27 per cent of all patients. Patients with haemolytic anaemia were less likely to have serositis, but no differences in the incidence of renal or cerebral manifestations were found between the various groups. Infections were mainly associated with the use of corticosteroid therapy. Life-table analysis showed no adverse influence on survival of haemolytic anaemia, neutropenia or lymphocytopenia, but late onset thrombocytopenia was associated with a decreased survival. No relationship was found between thrombocytopenia and the presence of antiphospholipid antibodies or the occurrence of thromboembolic events. We conclude that late onset thrombocytopenia in patients with systemic lupus erythematosus is associated with a decreased probability of survival but this could not be attributed to thromboembolic events.

Adolescent↗
PubMed1990

Antinuclear antibody profiles in relation to specific disease manifestations of systemic lupus erythematosus.

UNLABELLED: The aim of the study was to investigate the prevalence of antinuclear and associated antibodies (anti-dsDNA, anticardiolipin, anti-RNP, anti-Sm, anti-SSA and anti-SSB) and/or combinations thereof in systemic lupus erythematosus (SLE) patients with respect to their diagnostic and pathogenetic significance. The prevalence of anti-dsDNA antibodies was strongly influenced by the selection criteria of the patient; the lowest prevalence was found in SLE patients with central nervous system (CNS) involvement; the highest prevalence in patients with nephritis. The results were also influenced by the different assays. Combinding different assays (Farr/PEG ratio) quantitative as well as qualitative differences could be shown between patients with nephritis (Farr/PEG ratio greater than or equal to 5) and with CNS involvement (Farr/PEG ratio less than 5). No difference in anticardiolipin antibody prevalence between the different SLE patient groups could be demonstrated. Regarding antibodies against RNP, Sm, SSA and SSB, the prevalence was found to be strongly influenced by the criteria used for patient selection. Only in CNS patients and association with anti-RNP and anti-SSB antibodies alone or in combination was found. In pleuropericarditis a weak association with RNP antibodies existed. IN CONCLUSION: studying the prevalence and possible pathogenetic significance of antibodies one should always consider patient selection criteria and the effect of the different assays used when analysing the results.

Antibodies, Antinuclear↗
PubMed1990

Anti-heparan sulphate reactivity in sera from patients with systemic lupus erythematosus with renal or non-renal manifestations.

Previously, we have shown that anti-DNA can bind to heparan sulphate (HS), a constituent of the glomerular basement membrane (GBM). We hypothesized that binding of anti-DNA to HS in the GBM plays a role in the onset of systemic lupus erythematosus (SLE) nephritis. To test this hypothesis we measured the anti-HS reactivity in cross-sectional and longitudinal studies of SLE patients with or without nephritis. In the transverse serum study single serum samples from 26 SLE patients were studied. We found no correlation between anti-HS reactivity and previously development of nephritis (anti-HS positive: seven out of 16 with history of nephritis, two out of 10 without nephritis). However, six of the seven anti-HS positive sera in the nephritis group were obtained within 1 month of the onset of nephritis, suggesting a temporal relationship between anti-HS reactivity and onset of nephritis. In the longitudinal serum study between six and 16 serum samples were studied from each of 10 SLE-patients. In five out of five episodes of nephritis we found anti-HS reactivity before the onset or exacerbation of the nephritis. In four non-renal manifestations anti-HS reactivity was found in only one episode; in none of the three patients who remained clinically stable did serum samples show anti-HS reactivity. Anti-HS reactivity was only found in sera positive for anti-DNA by Farr assay but the anti-HS titre was not a mere reflection of the reactivity measured in the Farr assay. This indicates that only a subpopulation of anti-DNA can bind to HS. We found a high correlation (r = 0.99) between anti-HS reactivities in plasma and serum and we conclude that anti-HS reactivity in serum samples from SLE patients is not due to in vitro complex formation during clotting. Although further prospective analysis is necessary, our data suggest that measurement of anti-HS reactivity in SLE patients might identify patients at risk for the development of nephritis.

Adult↗
PubMed1990

Pancytopenia in systemic lupus erythematosus related to azathioprine.

A patient with systemic lupus erythematosus (SLE) developed pancytopenia when azathioprine (first 2.0 mg kg-1 day-1, then 2.5 mg kg-1 day-1) was added to prednisone for active SLE. This unusual toxic effect of azathioprine occurred during the reduction of corticosteroid dosage. After discontinuation of azathioprine, clinical and haematological recovery occurred.

Adult↗
PubMed1989

Systemic lupus erythematosus. III. Observations on clinical renal involvement and follow up of renal function: Dutch experience with 110 patients studied prospectively.

A prospective study of 110 patients with systemic lupus erythematosus (SLE) was undertaken to evaluate the reliability of clinical signs of lupus nephritis, which developed in 39 (35%) patients. Those patients with SLE who showed no clinical signs of lupus nephritis had an excellent survival rate (10 year survival 93%) and retained normal renal function (serum creatinine less than 130 mumols/l); clinical lupus nephritis developed mainly in the first three years after diagnosis of SLE and was associated with a decreased survival rate (10 year survival 62%). Increased mortality was found in male patients with lupus nephritis over 25 years of age and in female patients with lupus nephritis under 25 years of age, while renal failure rates did not differ between these groups. Treatment of lupus nephritis with high dose prednisone alone or in combination with immunosuppressants did not result in differences in patient survival or renal function preservation. It was concluded that clinical variables are a reliable guide in the management of patients with SLE, and routine use of renal biopsy in these patients is rejected.

Adult↗
PubMed1989

Systemic lupus erythematosus. I. Outcome and survival: Dutch experience with 110 patients studied prospectively.

This report presents an analysis of the cumulative survival in 110 well defined patients with systemic lupus erythematosus (SLE) who were followed up over a prolonged period of time. Special attention was paid to possible differences between patients who died and those who were still alive at the end of the study. Of the 110 patients with SLE, 96 (87%) were still alive after 10 years; the cumulative survival for men was 69% (11/16) and for women 90% (85/94). Patients who never developed a new exacerbation after the diagnosis for SLE had been established had a 10 year survival of 100%; for patients with one, two, or three exacerbations the 10 year survival was 91%, 69%, and 33% respectively. From these prospective studies it was found that the exacerbation frequency is most closely related to survival. Disease symptoms of renal involvement or neurological involvement, or both, present at the onset or at the moment the SLE diagnosis was established, were predominantly seen in patients who died during the follow up.

Adult↗
PubMed1989

Systemic lupus erythematosus. II. Observations on the occurrence of exacerbations in the disease course: Dutch experience with 110 patients studied prospectively.

The incidence of exacerbations in the disease course was investigated in 110 patients with systemic lupus erythematosus (SLE) who were studied prospectively at our institute for lupus research. At the time of disease onset and diagnosis the male patients were much older than the female patients (about 10 years); exacerbation frequency during follow up was increased in the male patients. The follow up data showed that if a patient with SLE was prone to develop an exacerbation this mostly took place within the first five years of follow up. It could be calculated that after fulfilling the American Rheumatism Association criteria only 56% (62/110) of the patients developed a subsequent exacerbation. Features at the time of diagnosis, distinguishing those patients who developed a subsequent exacerbation from those who did not, were haemolytic anaemia, the presence of anti-Sm antibodies, and a falsely positive serological test for syphilis. At the time of diagnosis, however, the prevalences of these features were low; for haemolytic anaemia, anti-Sm antibodies, and a falsely positive serological test for syphilis they amounted to 40%, 5%, and 12% respectively.

Adult↗
PubMed1989

Low avidity antibodies to double stranded DNA in systemic lupus erythematosus: a longitudinal study of their clinical significance.

In a longitudinal study the relevance of the detection of low avidity antibodies to double stranded DNA (dsDNA) as measured by the polyethylene glycol (PEG) assay in patients with systemic lupus erythematosus (SLE) was evaluated. It was found that 35 patients positive in the PEG assay only--that is, having only low avidity anti-dsDNA in their circulation, had a mild course of their SLE with absence of renal involvement. The PEG assay had little predictive value but a high specificity (90.2%) for clinical exacerbations; furthermore, a change in avidity of anti-dsDNA in such patients was seldom observed. In 14 patients positive in both Farr and PEG assays--that is, with a relative preponderance of high avidity anti-dsDNA, there was a clear correlation between rises in Farr assay and major exacerbations, while the PEG assay on its own was not helpful in predicting disease manifestations; disease manifestations were often heralded by a change in the Farr/PEG ratio, with renal and cerebral exacerbations associated with the greatest increase in the Farr/PEG ratio (more than 10 times the previous value).

Adult↗