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Biomedical subjects

F Hiepe

Publications and source records attributed to F Hiepe.

At least 91 records · Page 5Linked to original sources
PubMed1993

Widespread vasculopathy with hemolytic uremic syndrome, perimyocarditis and cystic pancreatitis in a young woman with mixed connective tissue disease. Case report and review of the literature.

A 15-year-old girl had severe Raynaud's phenomenon and arthralgias. A high ANA-IF titer was found and undifferentiated connective tissue disease was diagnosed. After 7 years of moderately flaring disease the patient deteriorated and presented with congestive heart failure, pleuropericardial effusion, hemolytic uremic syndrome, proteinuria and moderate hypertension. Autoantibodies against DNA, Sm-protein, and very high titers against U1RNP were detected. Therapy with high steroid doses, a cyclophosphamide pulse and 4 weeks of plasmaphresis with plasma exchange improved the heart, but not the renal condition. Symptomatic pancreatitis became the dominant problem of a progressively consuming process that resulted in the death of the patient. Post-mortem examination revealed widespread vasculopathy with intima proliferation and only minimal fibrosis involving the kidneys, heart and other main organs, including the pancreas. Taken together, the clinical picture was of an overlap between scleroderma and systemic lupus erythemathosus; the serologic and histopathologic findings suggest a diagnosis of a severe form of mixed connective tissue disease (MCTD).

Adolescent↗
PubMed1992

[Autoimmune phenomena during Trypanosoma equiperdum inoculation of the laboratory mouse].

In the course of the Trypanosoma equiperdum-infection of mice an increase of IgM antibodies against the autoantigens dsDNA, keratin and collagen as well as against a protozoan foreign antigen consisting of Sarcocystis gigantea-extract could be observed with a maximum level between 4th and 8th day p. i. The IgG-antibodies did not significantly change during the investigation time. A splenomegaly appeared after the infection. The weight of spleen was four times higher than normal. It was suggested that splenomegaly as well as induction of antibodies against several autoantigens and a foreign antigen were due to a polyclonal activation of lymphocytes.

Animals↗
PubMed1992

[Multiple myeloma with antinuclear antibodies. Its course during cytostatic therapy].

A 56-year-old man complained of general malaise, diffuse bone pain, nocturnal sweating, lack of appetite and weight loss (7 kg within one year). Pain in the pelvic and lumbar regions markedly impaired movement. Roentgenologically, multiple lucencies were visualised in many skeletal areas. Examination of the bone marrow revealed an increase in the proportion of atypical plasma cells to 40%. The IgG concentration was 29.8 milligrams, mainly monoclonal immunoglobulins of the IgG-kappa type, which reacted against nuclear antigens (68-kD-U1-RNP nuclear protein) with a 1:2,000 titre. The symptoms markedly regressed under cyclical combined therapy with three times 5 mg melphalan daily for 4 days and 100 mg prednisolone daily for 7 days (a total of 24 cycles). At the same time the IgG concentration and the autoantibody titre decreased markedly and the patient became symptom-free.

Antibodies, Antinuclear↗
PubMed1992

Investigations into Ro-specific antibody-associated congenital cardiac conduction defects.

Forty-two babies with different congenital cardiac conduction defects, and in 12 cases the mothers, were tested for autoantibodies to Ro, La, U1RNP and Sm. Ro-specific antibodies were detected most frequently. They were to be found in 16 sera from infants and in 8 maternal serum samples. The occurrence of anti-Ro was associated preferentially with several atrioventricular conduction blocks. The sex relation of anti-Ro associated congenital heart block did not show a typical preference (6 male/10 female). At the time of giving birth, 5 anti-Ro-positive mothers did not have any clinical symptoms of rheumatic autoimmune diseases. Three of them had a first degree atrioventricular block. Our findings indicate that all pregnant women at risk for anti-Ro like connective tissue disease or cardiac conduction defects should be tested for these autoantibodies because of the suspicion of cardiac conduction abnormalities in the offspring. Anti-Ro-positive infants should be examined for structural heart disease by echocardiography.

Antibodies, Antinuclear↗
PubMed1992

[Relation between 67-gallium scintigraphy and bronchoalveolar lavage--differential cell count and superoxide anion liberation--in patients with systemic scleroderma and systemic lupus erythematosus].

The aim of the study was to determine the pulmonary 67-gallium uptake, bronchoalveolar lavage (BAL) cell differentiation and the activity of BAL cells, measured as release of superoxide anion (O2-), and to investigate the results whether there are relations. In 11 nonsmoking systemic scleroderma (SS) patients and 11 systemic lupus erythematosus (SLE) patients with lung involvement double-sided BAL were performed, mainly in regions with increased 67-gallium uptake. Release of O2- was measured by INT-assey. BAL cell differentiation was in SS and SLE pathological in 68.2% without side-difference. In contrast to SS, O2(-)-release in SLE depends on BAL cell differentiation and is most increased in normal BAL cell differentiation. There is no correlation between 67-gallium uptake and both BAL cell differentiation and O2(-)-release. The results suggest, that in contrast to SS, BAL cells in SLE with pathological differentiation are less activated than BAL cells with normal differentiation probable due to autoimmunological factors. Pulmonary 67-gallium scan and BAL seem to be independent from each other.

Adult↗
PubMed1992

[Treatment of autoimmune diseases and graft rejection with anti-CD4 antibodies].

Based on the experience that T helper lymphocytes play an important part in the initiation and maintenance of various autoimmune diseases and also in graft rejection, novel therapeutic approaches have been developed and are under investigation. They are aimed at selective inhibition of T cells whose activation is unwanted. Useful tools for this purpose are monoclonal antibodies to cell surface molecules which are restricted to certain cell populations. In this review the concept of treatment with antibodies to CD4-a surface molecule characteristic of T helper lymphocytes-is discussed. Encouraged by experimental experiences obtained during the past years, a series of case reports were published and clinical pilot studies have been performed, the preliminary results of which are now becoming available. Anti-CD4 therapy appears to be a promising approach. Short-lasting effects can be separated from long-lived effects. The latter are not easy to explain, although hypotheses have been developed still requiring more detailed experimental confirmation.

Animals↗
PubMed1991

Development of a solid phase enzyme immunoassay for the detection of anti-Ro autoantibodies.

An enzyme immunoassay was developed to detect anti-Ro(SS-A) autoantibodies. Both Ro-antigen components (52 and 60 kD) were purified from a pig spleen extract, using fast protein liquid chromatography (FPLC). Anti-La, anti-RNP, anti-DNA and anti-Sm antibodies do not react to the purified antigen. There was a strong correlation between anti-Ro activity in EIA and the titers in counter immunoelectrophoresis (rs = 0.893). Anti-Ro antibodies were found in 54 (69.2%) of 78 SLE sera by the developed EIA.

Animals↗
PubMed1991

[Bronchoalveolar lavage in systemic scleroderma and systemic lupus erythematosus--differential cell values and enzyme cytochemistry].

The aim of the study was to determine the bronchoalveolar lavage (BAL) cell differentiation and the activity of beta-glucuronidase and N-acetyl-beta-D-glucosaminidase in alveolar macrophages. In 12 patients with systemic sclerosis (SS), 4 with systemic lupus erythematosus and 4 healthy controls BAL was performed. The activity of beta-glucuronidase and N-acetyl-beta-D-glucosaminidase was measured semiquantitative by means of cytochemical methods. Lymphocytes and neutrophils in BAL cell differentiation are increased, also the activity of beta-glucuronidase. The activity of N-acetyl-beta-D-glucosaminidase is decreased in SS and SLE in comparison with controls. The activity of beta-glucuronidase seems to be a marker of activity of alveolar macrophages in SS and SLE.

Acetylglucosaminidase↗
PubMed1991

[Bronchoalveolar lavage in patients with systemic scleroderma and systemic lupus erythematosus: characterization of cell activity by cytofluorometry, chemiluminescence and differential cell count].

The purpose of the study was to determine the role of lymphocyte subsets (Ly) and reactive oxygen metabolites RSM, concerning the activity of BAL cells, in the pathogenesis of lung involvement in 12 patients with systemic sclerosis (SS) and 4 with systemic lupus erythematodes (SLE) in comparison with 10 control subjects. The cellular activity was measured by means of cytofluorometry (CFM) and chemiluminescence (CL). In SS/SLEY CD3+, CD4+, CD4/CD8-ratio, CD25 + T-Ly and luminol-dependent CL are increased (p less than 0.05). Correlations exist between CD3+, CD4+, CD8+ and CD25 + T-Ly and both luminol-dependent CL and neutrophils (p less than 0.01). The results suggest, that increased secretion of RSM by BAL-cells may be caused by local release of lymphokines by these activated T-Ly. Therefore CFM and CL seem to be useful in addition to BAL cell differentiation in characterizing the BAL cell activity in the diagnostic of lung involvement in SS and SLE.

Adult↗
PubMed1990

Heterogeneity of RNP and Sm autoantigens in relation to the cell sources and the activated state of the cells.

The extracts of rabbit thymus (RTE), HeLa cells, human histiocytic lymphoma cell line U-937, human promyelocytic cell line HL-60, Ehrlich ascites tumor cells (EACs), and peripheral white blood cells (WBCs) were tested for their composition, molecular weight, and amount of Sm and RNP autoantigens on immunoblotting. The molecular weight of the so-called 68 kDa U1 RNP antigen, which is associated with mixed connective tissue disease (MCTD), was 64.5 kDa in RTE, 62.5 kDa in HeLa cells and HL-60 cells, and 59 kDa in WBCs. Surprisingly, in both WBCs and U-937 cells, the main protein band bearing the 68 kDa U1 RNP antigenic determinants was 30.5 kDa in molecular weight, which was confirmed using antibodies purified by affinity chromatography. After stimulation with phytohemagglutinin (PHA), there was in the human lymphocytes a diminished amount of the 30.5 kDa protein and simultaneously an increased synthesis of several proteins of higher molecular weight, especially the 57 kDa protein bearing the 68 kDa antigenic determinants. The concentrations of the A, B/B', C, D, and E proteins also increased with PHA stimulation. Our results indicate that the expression of Sm and RNP autoantigens may depend on the cell source as well as the activated state of cells. These differences should be taken into consideration in the detection of anti-RNP and anti-Sm antibodies by immunoblotting.

Animals↗