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Biomedical subjects

A Gibofsky

Publications and source records attributed to A Gibofsky.

At least 55 records · Page 3Linked to original sources
PubMed1983

Familial systemic lupus erythematosus in males.

Several families in which systemic lupus erythematosus predominates in males are presented. The disease primarily manifested itself in the sons and in the male parents. Females in some of the families had other autoimmune diseases such as idiopathic thrombocytopenic purpura or symptoms suggestive of a lupus diathesis. It is suggested that the sons inherited the disease from their fathers in these families. The disease in the patients had some similarity to the disease in BXSB mice.

Adolescent↗
PubMed1983

Pac-Man phalanx.

Explore the source record for details and available documents.

Adult↗
PubMed1983

Immunogenetic and clinical findings in idiopathic pulmonary fibrosis. Association with the B-cell alloantigen HLA-DR2.

In order to assess the role of immunogenetic factors in the pathogenesis of idiopathic pulmonary fibrosis (IPF), HLA-A, B, C, and DR typing was performed on 20 Caucasian patients with IPF seen from 1978 to 1980 and in 200 healthy blood donors. An increase in the frequency of the B-cell alloantigen, HLA-DR2, was observed; HLA-DR2 was found in 65% of patients with IPF compared with 26% of the control subjects (corrected p value = 0.005, chi-square = 11.476, relative risk = 5.286). Although not significant, increases were observed in HLA-B7 and HLA-A3, antigens known to be in linkage disequilibrium with HLA-DR2. The HLA-DR2 was observed in 12 of 14 patients with "ground glass linear" and "cystic honeycombing" radiographic patterns, whereas it was observed in only 1 of 6 patients with "ascinar nodular" radiographic patterns (p = 0.01). No other correlation was observed between HLA type and clinical, laboratory, or pathologic parameters. We conclude that immunogenetic factors, perhaps genes within the major histocompatibility complex, are relevant to the pathogenesis of IPF.

Adult↗
PubMed1982

Defective Fc receptor-mediated function of the mononuclear phagocyte system in lupus nephritis.

To determine whether patients with systemic lupus erythematosus and nephritis have more profound defects in mononuclear phagocyte system clearance than their counterparts without renal disease, we studed Fc receptor-mediated splenic clearance function in 32 patients. Clearance half-times were prolonged in patients with lupus erythematosus compared with those in normal controls (p less than 0.0001) and longer in patients with renal disease than in those without (p less than 0.025). Both renal (tau = 0.45, p less than 0.0002) and nonrenal (tau = 0.35, p less than 0.003) disease activity were significantly but independently associated with clearance half-times. When matched for nonrenal activity, patients with nephritis had greater clearance dysfunction than their counterparts without renal disease. Circulating immune complexes did not correlate with clearance for all patients. Neither B8 nor DR3 histocompatibility antigen markers differentiated the renal and nonrenal disease subgroups. The greater Fc receptor-mediated clearance dysfunction, which occurs in patients with lupus erythematosus and nephritis, could lead to enhanced glomerular deposition of immune complexes as a primary event, or as a secondary event amplifying previously established lesions.

Adult↗
PubMed1981

Three siblings with scleroderma (systemic sclerosis) and two with Raynaud's phenomenon from a single kindred.

A kindred is reported which contains 3 siblings with scleroderma, 2 siblings with Raynaud's phenomenon, and 2 first-degree relatives with histories suggestive of connective tissue syndromes. Studies of microvascular morphology and flow, serum endothelial cytotoxic activity, antinuclear antibodies, and HLA haplotypes in 18 relatives and 6 spouses revealed that 4 of 5 affected siblings expressed the HLA-DRw4 antigen, which was also present in 2 of 3 asymptomatic relatives whose serum contained endothelial cytotoxic activity. The evidence for an inherited susceptibility to scleroderma is reviewed.

Adult↗
PubMed1980

Peripheral blood Ia-positive T cells. Increases in certain diseases and after immunization.

The Ia antigens, usually expressed primarily on B lymphocytes, are found on a small percentage of normal peripheral blood T cells (average 2.6% by fluorescence and 10.8% by rosette assay). Elevated levels up to 40% by both assays were observed in a high proportion of patients with rheumatoid arthritis. Increases also were found in patients with systemic lupus erythematosus and various types of infections. The increases were evident with a specific heteroantiserum, a hybridoma reagent, and DR specific alloantisera. Normal levels were present in multiple sclerosis and an assortment of metabolic and other disorders. A rise in similarly positive T cells occurred in normal individuals after immunization with tetanus toxoid or PPD. The cells primarily involved in all of these instances were small lymphocytes, which stained relatively weakly with the fluorescent reagents and were readily distinguishable from T-cell blasts. They were found to be enriched in isolated T gamma fractions but were also found in other T cells. The accumulated evidence indicated that these cells represent an expansion of one or more subsets of T cells found in normal individuals, and that their level in the peripheral blood may serve as an index of immunological stimulation.

Arthritis, Rheumatoid↗
PubMed1979

Association of HLA-DRw2 with autoimmune thrombocytopenic purpura.

Peripheral blood lymphocytes from 38 patients with autoimmune thrombocytopenic purpura (AITP) were tested for HLA-A, -B, and -C alloantigens. Isolated B lymphocytes from 20 of these patients were tested for HLA-DRw (Ia) alloantigens. The profile of HLA alloantigens in the patients with AITP was significantly different from that of a matched control population. The most significant finding was the presence of the HLA-DRw2 alloantigen in 75% of patients as compared with 23% in the control population, P less than 0.001, relative risk 10.0 (A relative risk of 1 would indicate no association between the presence of the antigen and the disease.) The co-occurrence of either A3 and B7 (known to be in linkage disequilibrium with DRw2) or A26 and Bw38 was significantly increased as compared with the control population (P less than 0.001). Of the patients positive for DRw2, 47% had the association A26 and Bw38 as compared with the control population association incidence of 21% (P less than 0.1). Thus, in the patient population, A26-Bw38 appears to be a haplotype that is in linkage disequilibrium with DRw2 (as presumably is the case with A3-B7). These data indicate that a predisposition to AITP is inherited with a DRw2 gene of the major histocompatibility system.

Autoimmune Diseases↗
PubMed1979

Chronic Lyme arthritis. Clinical and immunogenetic differentiation from rheumatoid arthritis.

Ten patients with Lyme arthritis have developed chronic involvement of one or both knees. Lyme arthritis was diagnosed by onset with erythema chronicum migrans (six patients); residence in Lyme, Connecticut (eight); seasonal onset in summer and early fall (nine); early periods of short recurrent attacks (nine); absence of rheumatoid factor (nine); and absence of symmetrical polyarthritis, morning stiffness, subcutaneous nodules, or antinuclear antibodies (in all). Five patients had synovectomies; pannus formation and underlying cartilage erosion were present in all. Seven of the 10 patients had the same B-cell alloantigen, DRw2 (frequency in normal control subjects, 22% [P less than 0.005]), but did not have an increased frequency of the alloantigens associated with rheumatoid arthritis. Chronic Lyme arthritis, the result of an apparent tick-transmitted infection, resembles rheumatoid arthritis pathologically but generally differs from it in both prearticular and immunogenetic characteristics.

Adolescent↗
PubMed1978

Disease associations of the Ia-like human alloantigens. Contrasting patterns in rheumatoid arthritis and systemic lupus erythematosus.

Increasing evidence has been obtained of the special value of Ia-like B-cell alloantisera for demonstrating disease associations with histocompatibility antigens. This was particularly evident for the study of the immunogenetics of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), two conditions frequently considered related. The profiles of antigens recognized by the alloantisera in patients from each disease group was distinctive. Two types of alloantisera were obtained that illustrated the divergence between the twod iseases. One type showed a higher than normal incidence in RA but lower than normal in SLE; the other showed a higher incidence in SLE. While these sera were not totally defined, evidence was obtained that the SLE-reactive alloantiserum related to two alleles of the major histocompatibility complex DRw2 and DRw3, while the RA-reactive alloantiserum related to a common specificity shared by cells positive for either DRw4, DRw7, or DRw10. The data indicate that immunogenetic factors are relevant to the development of both RA and SLE, but that these are distinct for each disease.

Antigens, Surface↗
PubMed1978

Contrasting patterns of newer histocompatibility determinants in patients with rheumatoid arthritis and systemic lupus erythematosus.

The Ia alloantigens as measures of different alleles of loci in the major histocompatibility complex were determined in patients with systemic lupus erythematosus (SLE) or rheumatoid arthritis (RA). The Ia specificities of reagents used were defined by their pattern of reaction with lymphoblastoid lines derived from normal donors homozygous for HLA-D determinants. The reagent specificities included those associated with a single Dw type as well as those reacting with a single specificity shared by several Dw types. Patients with RA had a marked elevation in the frequency of alloantigens detected by reagent sera that recognize various determinants shared by cell lines from HLA-Dw4, Dw7, or Dw10 individuals (Ia 4-7-10). The frequency of mixed lymphocyte culture alleles Dw4 and Dw10 was found to be increased; however this elevation did not approach the higher frequency for the serologically determined antigens of the Ia 4-7-10 group. In contrast, patients with SLE had an increased frequency of reactions with the reagent alloantisera defined by reactions with either HLA-Dw2 or Dw3 positive cell lines. The data suggest that immunogenetic factors are relevant to both groups of patients, but that these are entirely distinct for each disease.

Arthritis, Rheumatoid↗
PubMed1978

Expression of Ia-like antigens on cultured human malignant melanoma cell lines.

Human malignant melanoma cell lines were found to bear Ia-like cell surface determinants demonstrable by hetero- or alloantisera and by direct identification of the characteristic bimolecular glycoprotein complex. Immunoprecipitation confirmed the presence of Ia determinants on the bimolecular complex. The quantity of Ia molecules determined by these methods and by absorption experiments was relatively constant for each cell line. Among different lines, however, the amount of Ia antigens ranged from a level equal to that expressed by B-cell lines to a small fraction of this amount. This variation in level of Ia contrasted with the more uniform amount of beta2-microglobulin detected on the cell surface. The Ia alloantigen specificity DRw2 was the most frequently encountered specificity. Ia determinants were also found on the surface of an epidermoid carcinoma line, but not on various other cell lines of normal or neoplastic origin.

Antigens, Surface↗
PubMed1975

The identification of HL-A antigens on fresh and cultured human endothelial cells.

Human endothelial cells were obtained from the umbilical cord veins of 16 newborns by methods previously described and tested for HL-A antigens by a microcytotoxicity method. HL-A antigens were present on all endothelial cell lines tested. When the HL-A phenotypes of fresh endothelial cells and autologous fetal lymphocytes were compared, a concordance of 70% was observed. When the HL-A phenotypes of maternal lymphocytes and fresh endothelial cells were compared, a maternal contribution to the endothelial cell phenotype was evident in 72% of the possible commmon antigens. Some HL-A antigens were deleted from 11 of 16 endothelial cell lines that were re-typed after 2 weeks in tissue culture. The majority (90%) of deleted antigens were from the second HL-A locus. When three lines of endothelials cells were again re-typed after 6 weeks in culture, no further changes in antigenicity were noted. These findings: a) demonstrate that HL-A antigens are present on human endothelium and suggest that endothelial cells are actively involved in establishing the immunogenicity of a graft, and b) demonstrate that the HL-A antigens on human endothelial cells may be modulated by in vitro culture.

Adult↗