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Biomedical subjects

G Sturfelt

Publications and source records attributed to G Sturfelt.

At least 109 records · Page 6Linked to original sources

Influence of factor D concentrations on fluid phase C3 activation, lysis of rabbit erythrocytes and solubilization of immune complexes.

Factor D depleted serum did not support hemolysis of rabbit erythrocytes and solubilization of performed immune complexes. Fluid phase C3 cleavage increased in a dose dependent manner when D protein was added to normal or to factor D depleted serum. During short incubation factor D concentrations were correlated with the capacity of serum to solubilize immune complexes and to lyse rabbit erythrocytes. With prolonged incubation, the hemolytic activity decreased in a factor D dose dependent manner. This was probably due to fluid phase breakdown of C3 and factor B in the presence of high factor D concentrations. Hypocomplementemic sera from patients with active systemic lupus erythematosus (SLE) did not support solubilization of bovine serum albumin (BSA) anti-BSA complexes when factor D was added in excess. Patients with polycystic kidney disease with reduced renal function and high factor D concentrations showed increased concentrations of circulating C3d/dg fragments. The possibility was considered that high factor D concentrations in uremia might promote fluid phase C3 degradation and thereby limit the in vivo efficiency of alternative pathway activation on target surfaces.

Animals↗

Lung function in patients with systemic lupus erythematosus and persistent chest symptoms.

Twelve consecutive patients with systemic lupus erythematosus (SLE) and chest symptoms of at least 3 months' duration were investigated with spirometry, lung mechanics at rest and exercise, diffusion capacity and right heart catheterization. Vital capacity (88% of predicted, p less than 0.05), and FEV1 (84%, p less than 0.01) were decreased in the study group, but spirometric and diffusion capacity abnormalities were moderate compared with previous studies. The single breath CO2 test showed, in six patients, ventilation-perfusion mismatch with patterns typical for either bronchial obstruction or vascular disease. Non-respiratory factors were responsible for reduction of working capacity (on average 68% of predicted normal values (p less than 0.001]. Two patients with pulmonary hypertension were identified by right heart catheterization. One of them had overlap features with the CREST syndrome. Both these patients had abnormal SBT-CO2 test and diffusion capacity, along with diffuse perfusion defects on perfusion scintigraphy. The low frequency of pulmonary function abnormalities in this study suggests that irreversible pulmonary damage is uncommon in SLE.

Adult↗

Outcome in systemic lupus erythematosus: a prospective study of patients from a defined population.

All adult patients with systemic lupus erythematosus (SLE) (greater than or equal to 15 years old, n = 86) from a defined population (approximately 160,000 population at risk) were followed prospectively over 6 years. The study area comprised 2 health care districts served by only 1 hospital. Retrieval was based on clinical case finding and computerized diagnosis and laboratory registers. The incidence of the disease was 4.0 cases/100,000 adults/year and was stable during the 6 years, suggesting that completeness of retrieval was high. Point prevalence by the end of 1986 was 42 cases/100,000 population at risk and 5-year survival in the prospective group 97%. Immunologically the group was characterized by a high frequency of positive anti-dsDNA (73%), and a low frequency of rheumatoid factor positivity (10%). The frequency of ARA criteria (median, 6) was comparable with previous larger series of selected patients. Sixteen percent of the patients were males, and they had more serositis and renal manifestations than females. In view of the low mortality we studied the more sensitive outcome measures: disease activity, irreversible organ damage, and functional impairment. After the diagnosis year, disease flares occurred with a constant frequency of 0.2 flares/year/patient, even after long duration of disease. Patients with neuropsychiatric disease, history of drug reactions, and immunological abnormalities such as persistent hypocomplementemia, antibodies to dsDNA, and cardiolipin had a high frequency of relapse. In contrast, elderly patients with serositis during their first flare seldom relapsed. The number of gainfully employed individuals was normal. Neuropsychiatric disease and joint involvement were principal causes of long-standing functional impairment. Notably, patients with renal disease usually fared well, as reflected by preserved renal function and little functional by preserved renal function and little functional impairment. Disease duration and glucocorticoid treatment were major denominators for morbidity due to infections and vascular disease with the incidence of myocardial infarctions being 9 times more common than that in a Swedish control population. Prolonged glucocorticoid treatment was also related to mortality, which was predominantly due to cardiovascular or central nervous system disease. The present prospective and epidemiologically based study of outcome in SLE was made possible by a uniquely coordinated health care system, enabling complete identification of the patients within the study area.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗

Palmoplantar pustulosis and sternocostoclavicular arthro-osteitis.

Seventeen patients with shoulder pain and radiographic involvement of the sternoclavicular or sternocostal joints, or both, are described. Eleven of these patients also had palmoplantar pustulosis. Histological examination of the joints showed chronic and subacute inflammation, increased osteoblastic activity, and cartilage degeneration. Propionibacterium acnes was cultured in tissue samples from seven of the 15 biopsied patients, a finding at variance with those of previous reports. The possibility that sternoclavicular arthro-osteitis is of infectious origin should be the subject of further investigation. Non-steroidal anti-inflammatory drugs (NSAIDs) may provide pain relief, possibly owing to their inhibitory action on osteoblasts. In cases of severely restricted movement or severe pain resection of the medial clavicle may be considered.

Adult↗

The effect of age on clinical and serological manifestations in unselected patients with systemic lupus erythematosus.

In a unique series of 80 patients with systemic lupus erythematosus constituting all identified cases in a defined population, the effect of age, at onset and at diagnosis, on disease expression was examined. Contrary to current views, the incidence of the disease was similar among all age groups. The proportion of patients with serositis, secondary Sjögren's syndrome, and anti-SSA antibodies gradually increased with age, while that of cutaneous symptoms, antibodies to native DNA, and hypocomplementemia decreased. Serositis was common as the initial symptom in the elderly and serositis or musculoskeletal manifestations were the initial symptom in 88% of patients over 50 at onset of disease.

Adult↗

Complement fixation by solid phase immune complexes. Reduced capacity in SLE sera.

We describe an ELISA for assessment of complement function based on the capacity of serum to support fixation of complement components to solid phase immune complexes (IC). Microplates were coated with aggregated bovine serum albumin (BSA) followed by rabbit anti-BSA IgG. The solid phase IC were reacted with human serum. The uptake of C3b, C4b and properdin was measured using biotinylated F(ab)2 antibodies to each of the proteins, avidin alkaline phosphatase, and paranitrophenyl phosphate. Serial samples obtained from 15 patients with systemic lupus erythematosus were investigated. Out of 72 sera, 24 showed a reduced capacity to support incorporation of C4b into solid phase IC. Thirty-one of the sera showed low C3b binding and 59 of the sera a reduced uptake of properdin. The incorporation into solid phase IC of C3b and C4b as well as of C3b and properdin were closely correlated at high disease activity. In general, patients with severe disease manifestations showed low values in the uptake assays. Judging from the results obtained by analysis of serial samples, the uptake of C3b, C4b and properdin, complement mediated solubilization of fluid phase IC and the concentrations of C1q binding IC were useful indicators of disease activity in the patients. The concentrations of circulating C4, C3 and properdin varied less consistently according to disease activity. The concentrations of serum properdin were never found to be low, which was in contrast to the finding of reduced properdin uptake by solid phase IC in most of the samples.

Adult↗

Anticardiolipin antibodies in patients with systemic lupus erythematosus.

We studied a group of 59 unselected patients with systemic lupus erythematosus (SLE); these patients were from a defined population who lived in southern Sweden. We found that serum concentrations of anticardiolipin antibodies were increased in 32 SLE patients (54.2%). No significant correlation between increased amounts of anticardiolipin antibodies and clinical symptoms, such as thrombocytopenia or thrombosis, was found. Serial serum samples from 28 patients (12 patients were from the epidemiologic cohort) were analyzed. Sixteen of these 28 patients (57.1%) had increased levels of anticardiolipin antibodies; in most cases, there was no variation in these values with regard to clinical disease flares or treatment. Increased concentrations of anticardiolipin were observed in 4 patients with cerebral infarction. However, very high concentrations of anticardiolipin antibodies were observed in several patients with inactive SLE who had no history of thrombosis or thrombocytopenia. Our results underscore the importance of studying unselected patient groups when correlating laboratory data with clinical manifestations of disease.

Adult↗

Clinical and serological features of severe vasculitis in rheumatoid arthritis: prognostic implications.

Sixteen patients with classic rheumatoid arthritis (RA) complicated by severe vasculitis were studied and compared with a matched control group of 16 RA patients without vasculitis. Seven of the patients with vasculitis died within 4 to 120 months (median 32 months) after developing vasculitic symptoms. Gangrene of digits and extremities, bowel ulcers or bowel perforation, or both, and cardiac involvement were more common among the patients who died than among those with a more favourable course. The present data suggest that large vessel vasculitis in RA is associated with high frequency of arteriosclerotic vascular disease. The serum concentrations of complement components C3 and C4 were lower, and concentrations of IgM rheumatoid factor, complement activating rheumatoid factor, and C1q binding immune complexes (C1q solid and C1q fluid phase assay) were significantly higher among vasculitic patients than in the control group. Laboratory data provided little prognostic information with regard to rheumatoid vasculitis, with the exception that IgM and IgG rheumatoid factors were significantly higher among patients with fatal course of disease than in those who achieved remission.

Aged↗

Anticardiolipin and complement activation: relation to clinical symptoms.

Anticardiolipin antibodies which seldom occur in healthy persons are frequently found in systemic lupus erythematosus (SLE). Their presence, especially at high levels (greater than 10 units) are often accompanied by thromboembolic manifestations as well as thrombocytopenia and recurrent abortions. The incidence of cardiolipin antibodies was as great in SLE as in women with clinically unexplained recurrent abortions. The anticardiolipin levels remained unchanged in most patients for long periods and were remarkably unaffected by disease activity and therapy. On the other hand, in several cases the activated partial thromboplastin time which was prolonged in most patients with persistently high cardiolipin antibody levels, approached normal during treatment with prednisolone and salicylic acid. This seemed to facilitate a successful outcome of pregnancy. Absorption of sera with cardiolipin and other negatively charged phospholipids but not with uncharged phospholipids abolished the anticardiolipin activity in enzyme linked immunosorbent assay. In most sera with cardiolipin antibody levels greater than 10 units complement activation by the classical pathway could be demonstrated. Whether the anticardiolipin antibodies contributed to complement activation could not be determined.

Abortion, Habitual↗

C1 activation, with C1q in excess of functional C1 in synovial fluid from patients with rheumatoid arthritis.

Free Clq, in functionally active form was present in increased amounts in the synovial fluid of patients with rheumatoid arthritis. The presence of free Clq was associated with low concentrations of hemolytic C1, low C4 and raised amounts of C3dg/d fragments in the synovial fluid. The findings suggested intra-articular C1 activation with dissociation of C1 into free C1q and complexes containing C1r, C1s, and C1 inactivator. However, the immunochemical properties of synovial fluid C1r-C1s-C1 inactivator complexes appeared to differ from those of the complexes formed in serum, which hampered quantification with the assay used. Control patients with osteoarthritis or spondylarthritic syndromes did not show evidence of intra-articular complement activation, even though 1 patient with Reiter's disease had unexplained low concentrations of synovial fluid C4 and C3. The concentrations of circulating complement components were largely normal in the patients. Slightly increased concentrations of free C1q and C1r-C1s-C1 inactivator complexes in serum and C3dg/d fragments in EDTA plasma were observed, particularly in the patients with rheumatoid arthritis.

Adult↗

Symptomatic secondary Sjögren's syndrome in patients with systemic lupus erythematosus (SLE). Relation to anti-SS-A and anti-SS-B autoantibodies.

The prevalence of symptomatic secondary Sjögren's syndrome in 66 patients with SLE recruited from a defined population in southern Sweden was 59%. In 26 patients (39%) the symptoms were mild and variable, related to disease activity, while in a smaller group (20%) the symptoms appeared to be constant. The latter patients had an increased frequency of thyroid disease and autoantibodies to SS-A. The patients in this group were older, but did not seem to differ in regard to other disease manifestations.

Antibodies, Anti-Idiotypic↗

Systemic lupus erythematosus in an adult population in southern Sweden: incidence, prevalence and validity of ARA revised classification criteria.

In a defined Swedish population an annual incidence of 4.8 cases, a mortality of 1.3 cases and a prevalence of 39 cases per 100 000 individuals was found for systemic lupus erythematosus. Sensitivities were comparable (94%, cf. 92%) for ARA criteria of 1982 and those of 1971. Males had more internal organ criteria and showed a tendency to fewer cutaneous criteria than females. Spontaneous abortion was rare and hospitalization infrequent among the 65 patients studied.

Adolescent↗

Serum concentration of cystatin C, factor D and beta 2-microglobulin as a measure of glomerular filtration rate.

Serum concentrations of creatinine and of the three low molecular weight (LMW) proteins cystatin C, factor D of the complement system and beta 2-microglobulin were measured in 135 consecutive patients, whose glomerular filtration rates (GFR) were determined by Cr-EDTA. In the total patient series, the reciprocals of S-creatinine and S-cystatin C were numerically and, in males, significantly more closely correlated to GFR than the reciprocals of S-factor D. The reciprocals of beta 2-microglobulin showed a weaker correlation to GFR than those of the other three substances. The calculated glomerular elimination rates of creatinine, cystatin C and factor D were normally distributed, in contrast to those of beta 2-microglobulin. According to data presented so far, cystatin C seems to be the LMW protein of first choice when GFR is to be estimated by measuring the plasma concentration of a LMW protein.

Adolescent↗

Lyme disease in a 12-year-old girl.

We report the case of a 12-year-old girl with erythema chronicum migrans, aseptic meningitis and knee arthralgia. Rise of specific antibody titre against an Ixodes ricinus spirochaete was demonstrated. Circulating immune complexes and high levels of C1r-C1s-C1IA complexes indicating activation of the complement system via the classical pathway were found. The clinical features and the laboratory findings warranted a diagnosis of Lyme disease.

Antigen-Antibody Complex↗