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Biomedical subjects

E Hedfors

Publications and source records attributed to E Hedfors.

At least 19 recordsLinked to original sources
PubMed1998

Ro/SS-A and La/SS-B antibody level variation in patients with Sjögren's syndrome and systemic lupus erythematosus.

To examine both possible correlations between anti-Ro/SS-A and anti-La/SS-B levels and their correlation with clinical disease activity in patients with Sjögren's syndrome (SS) or systemic lupus erythematosus (SLE), an ELISA was developed using purified recombinant Ro 60 kDa, Ro 52 kDa and La antigens. The ELISA was used for testing sequential serum samples from 16 patients with either SS or SLE. The patients were followed for periods between 15 and 128 months, and 3-15 serum samples per patient were analysed and compared with clinically apparent disease activity at the time of sampling in 14 patients. A temporal correlation of antibody levels to Ro and La antigens was found, and antibodies to different epitopes of the Ro 60 kDa protein showed parallel variation in seven of eight patients tested. Co-variation of autoantibody levels and disease activity was found in 11 of 14 patients. In seven of these 11 patients the anti-Ro and anti-La levels were stable and changes in disease activity were minimal during the observation period. In the other four of these 11, changes in disease activity were noted, with an associated change in autoantibody levels. The results suggest that the serological response to Ro and La antigens, as well as to different epitopes of the Ro 60 kDa protein, is antigen driven and regulated by common mechanisms, and indicate a correlation of Ro and La antibodies with pathogenic events.

Adult↗
PubMed1993

Behçet's disease: a review and a report of 12 cases from Sweden.

In a retrospective study of 12 patients with Behçet's disease, more than half were found to originate from the Near East, where the prevalence of the disease is known to be high. The immigrant patients were all males, whereas 3 of the 5 patients with Swedish ancestry were females. Certain differences emerged between the two groups, including different sex ratio and absence of HLA B5 association and pathergy skin reaction among the Swedish patients. Moreover, serious neurological and ocular symptoms showing no tendency to recede with age afflicted all the Swedish female patients. Urogenital symptoms were, besides ulcers, common in both groups, including prostatitis, urethritis, orchitis, chronic sterile cystitis and relapsing salpingitis. Although the maternal does not allow statistical inferences, the estimated prevalence was higher than expected among both Swedish and immigrant patients. Recent studies, including the diagnostic criteria proposed by the "International Study Group for Behçet's disease", are discussed in relation to previously used criteria as well as present findings. The sensitivity and specificity of the first mentioned criteria and the ones proposed by Mason & Barnes seemed equal.

Adult↗
PubMed1992

A clinical, serological, and histopathological study of myositis patients with and without anti-RNP antibodies.

Twenty-nine patients with myositis, including 10 with polymyositis (PM), 6 with dermatomyositis (DM), and 13 with myositis associated with a connective tissue disease (CTD), were followed up for a mean observation time of 49 months. The 13 patients with CTD-associated myositis were further separated by the presence or absence of anti-RNP antibodies. The functional disability at diagnosis was pronounced without differences between the groups. The patients with anti-RNP antibodies did not differ from the other patients regarding initial muscle weakness, erythrocyte sedimentation rate, or creatinine phosphokinase values, but the histopathological muscle changes were generally milder. Rapid improvement of muscle strength on moderate doses of corticosteroids was seen in most patients. At the end of study, corticosteroid treatment had been withdrawn from 17 patients because of remission, including 6 of the 7 patients with anti-RNP antibodies. The presence of electromyographic changes compatible with myositis, pronounced muscle weakness before treatment, and a low erythrocyte sedimentation rate seemed to indicate a less favorable outcome unrelated to diagnostic subgroupings. However, the combination was rarely found among the anti-RNP-positive patients.

Adolescent↗
PubMed1992

Clinical manifestations and anti-(U1)snRNP antibodies: a prospective study of 29 anti-RNP antibody positive patients.

Twenty-nine anti-RNP positive patients were followed prospectively with a mean observation time of 65 months (29-120 months). The clinical course was correlated to the presence of IgM and IgG anti-(U1)snRNP antibodies as revealed by immunoblotting from sequentially obtained sera. There was a striking dissociation between the fluctuating course, with the appearance of new manifestations followed by remissions, and the stability of the anti-snRNP antibody specificities where an appearance or a disappearance of anti-snRNP specificities was a rare phenomenon. The main epitope recognized by the IgG antibodies was the 70 kDa protein and of the IgM antibodies the B/B' proteins. No shift from the IgM to the IgG isotype was observed. The presence of IgG anti-70 kDa and IgM anti-B/B' antibodies was highly associated with presence of arthralgias, Raynaud's phenomenon and arthritis. Further, an association was noted between the combined presence of IgG anti-70 kDa, anti-A and anti-C antibodies and IgM anti-B/B' and puffy hands, myositis, pulmonary fibrosis and sclerodactyly, i.e. all manifestations of mixed connective tissue disease (MCTD). On the contrary, serositis as often seen in SLE was correlated to the presence of IgG anti-B/B' antibodies. Thus the longitudinal analysis of the correlation between anti-snRNP antibody specificities and clinical manifestations support the concept of MCTD as a distinct entity.

Adolescent↗
PubMed1992

IgG and IgM anti-snRNP reactivity in sequentially obtained serum samples from patients with connective tissue diseases.

Sequentially obtained serum samples from 30 patients with connective tissue disease positive for antibody to ribonucleoprotein (RNP) were examined to determine the specificities of IgG and IgM antibodies to snRNP during the disease course using immunoblotting of nuclear extracts. The antibody patterns were correlated with disease activity. The patterns of antibody to snRNP of individual patients were mainly stable during the study but changes in levels of antibody to snRNP were seen corresponding to changes in clinical activity. These results indicate that increased reactivity of serum IgM antibodies against the B/B' proteins seems to precede a clinically evident exacerbation of disease whereas IgG antibody reactivity to the 70 K protein peaks at the time of a disease flare.

Autoantibodies↗
PubMed1991

Pregnancy outcome in patients with high titer anti-RNP antibodies. A retrospective study of 40 pregnancies.

In a retrospective study the outcome of 40 pregnancies in 20 women with a high titer of anti-RNP antibodies was evaluated. In the 18 pregnancies that occurred after disease onset, transient proteinuria was noted in 3 and transient thrombocytopenia in 2. Deep venous thrombosis was observed in one patient. Preeclampsia in another woman necessitated cesarean sections in 2 pregnancies with successful outcome. The observed complications may all be seen in normal pregnancies. There was no evidence of exacerbation of maternal disease during pregnancy or in the postpartum period. Our study indicates that in women with high anti-RNP titer the risk of fetal loss or maternal worsening of disease seems slight.

Adolescent↗
PubMed1991

Clinical course of patients with anti-RNP antibodies. A prospective study of 32 patients.

Thirty-two patients with high and low anti-RNP antibody titers were followed prospectively during a mean observation of 65 months. The following 4 titer patterns were observed: persistently high, low increasing to high, high decreasing to low and persistently low titers. At first admission, 17 of the 23 patients with high anti-RNP titers did not fulfill the criteria of defined connective tissue diseases (CTD). The clinical courses were characterized by the appearance of new organ manifestations and at the end of the study 17/23 fulfilled the criteria for mixed CTD (MCTD). A development towards systemic lupus erythematosus (SLE) was seen in one, concomitant with decreasing anti-RNP titer. None developed symptoms compatible with progressive systemic sclerosis. The 9 patients with a low anti-RNP titer were characterized by a stable clinical course, including 4 with SLE, 2 with Raynaud's phenomenon, and one each with Sjögren's syndrome, discoid lupus erythematosus and rheumatoid arthritis + Sjögren's syndrome. The most frequently occurring clinical manifestations among the patients with MCTD were Raynaud's phenomenon, puffy hands, arthritis, myalgias, and sicca symptoms. Myositis and impaired pulmonary function were also seen, but glomerulonephritis was not. The symptoms were fluctuating and the manifestations clinically different from other well defined CTD. The morbidity was moderate and the mortality low. High anti-RNP titer observed at any time seems associated with a clinical syndrome phenotypically different from other CTD favoring the notion of MCTD being a distinctive clinical syndrome.

Adolescent↗
PubMed1990

Recombinant 70-kD protein used for determination of autoantigenic epitopes recognized by anti-RNP sera.

A panel of high- and low-titre anti-RNP-positive patient sera was tested for reactivity with human snRNP proteins. The U1 snRNP-specific 70-kD peptide was found to be the most prominent RNP antigen recognized by high-titre anti-RNP sera, mainly found in patients with mixed connective tissue disease (MCTD). The reactivity with the 70-kD protein was further analysed with recombinant fusion proteins containing different segments of the protein. One major and two minor antigenic regions were found. Most patient sera, both with high- and low-titre anti-RNP antibodies, only recognized the major region. The most aminoterminal region, showing partial sequence homology with a mouse retroviral p30 gag protein, contained an epitope that was recognized by one serum only.

Adult↗
PubMed1990

Variation of MHC class I and II antigen expression in relation to lymphocytic infiltrates and interferon-gamma positive cells.

The occurrence of MHC antigens on epithelial cells in lip salivary glands obtained from patients with various connective tissue diseases and from bone marrow recipients was studied. The amount of infiltrating lymphocytes correlated to an increase in MHC class I and II antigen expression, but not to diagnosis or glandular function. Interferon-gamma + infiltrating cells were scanty. The role of interferon-gamma as the main inducer of MHC antigens and the notion "aberrant" HLA-DR thought to perpetuate chronic autoimmune disease are questioned.

Adolescent↗
PubMed1990

Restricted dose and duration of corticosteroid treatment in patients with polymyalgia rheumatica and temporal arteritis.

To analyze whether corticosteroids in low doses during limited time periods could be safely used in the treatment of patients with polymyalgia rheumatica (PMR) or temporal arteritis (TA) the records of 91 patients diagnosed between 1980 and 1987 were reviewed. The mean initial prednisolone dose was in patients with PMR 18 mg/day and the mean duration of treatment was 17 months. In patients with TA the mean initial dose was 31 mg/day and the mean duration of treatment 16 months, and in all the corticosteroid treatment was terminated within 24 months. Patients with coexisting TA and PMR demanded longer treatment compared to patients with either TA or PMR and thus 18/91 patients were treated for more than 2 years. No visual or neurological complications occurred after treatment with corticosteroids had been initiated. Our study indicates that most patients with PMR or TA can be treated safely with an initial prednisolone dose of 10 mg given twice daily. With few exceptions corticosteroid treatment can be terminated within 24 months.

Adrenal Cortex Hormones↗
PubMed1989

Lymphocytic infiltrations of lip salivary glands in bone marrow recipients. A model for the development of the histopathological changes in Sjögren's syndrome?

By use of a PAP staining technique, the immunohistopathology in repeated biopsies from the lip salivary glands (LSGs) from patients undergoing bone marrow transplantation (BMT) was studied. In the previously normal LSGs, focally arranged lymphocytes and adjacent epithelial DR-expression appeared within 12 weeks post BMT, reaching a maximum between 26 and 52 weeks post BMT. Two years post BMT, lymphocytic infiltrates and epithelial DR-expression were still present in half of the specimens but were not seen in the remaining ones. The immunohistopathological changes seen in LSGs post BMT were indistinguishable from what has been found in Sjögren's syndrome. The appearance, and in some patients the subsequent disappearance, of the lymphocytic infiltrates and the epithelial DR-expression, without correlation to clinical symptoms of chronic graft-versus-host disease or immunosuppressive treatment, emphasizes the dynamic nature of lymphocytic infiltration of LSGs in BMT patients. Whether this also pertains to naturally occurring Sjögren's syndrome remains to be elucidated.

Bone Marrow Transplantation↗