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Biomedical subjects

T T Provost

Publications and source records attributed to T T Provost.

At least 73 records · Page 4Linked to original sources
PubMed1984

Sjögren's syndrome. Influence of multiple HLA-D region alloantigens on clinical and serologic expression.

The relationships of HLA-DR and the newer DS (second D locus) B cell alloantigens (MB and MT) to the clinical and serologic expression of primary and secondary forms of Sjögren's syndrome (SS) were examined in 102 patients (86 whites and 16 blacks). Although HLA-DR3 was significantly increased in whites (25 of 50, 50%) and blacks (4 of 5, 80%) with primary SS compared with race-matched normal controls, it was not appreciably elevated in those with systemic lupus erythematosus (SLE)-SS, rheumatoid arthritis (RA)-SS, or connective tissue disease-SS. The MT2 specificity, however, was more strongly associated with primary SS (86% of whites and 100% of blacks) and also with SLE-SS and RA-SS compared with race-matched normal controls. Furthermore, MT2 was significantly increased in SLE-SS and RA-SS when compared with non-sicca SLE and RA controls. Although primary and secondary SS were most strongly associated with this DS specificity (MT2), the anti-Ro (SS-A) and anti-La (SS-B) antibody responses were more closely allied to DR antigens. HLA-DR3 was increased in anti-Ro positive patients, both whites and blacks, with primary SS (74%) and in total anti-Ro positive subjects (54%) compared with their anti-Ro negative counterparts (38% and 31%, respectively). Among DR3 negative patients, HLA-DR2 correlated with anti-Ro in both primary SS (83%) and in the total SS group (58%). Thus, 96% of Ro antibody positive patients with primary SS had DR3 and/or DR2, as did 80% of anti-Ro positive subjects in all categories.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1984

Primary Sjögren's syndrome and other autoimmune diseases in families. Prevalence and immunogenetic studies in six kindreds.

The relationships of human leukocyte antigen (HLA) and heavy chain immunoglobulin (Gm) haplotypes to disease and autoantibody expression were examined in six large kindreds, each having one or more members with primary Sjögren's syndrome. Various other autoimmune diseases and autoantibodies occurred among the 117 relatives in these families. The HLA and Gm haplotypes did not necessarily segregate persons into those with Sjögren's syndrome, other autoimmune disorders, or serologic abnormalities, but HLA alleles DR3 and DR2 occurred in significant excess in relatives with Sjögren's syndrome, irrespective of HLA haplotype. Segregation analysis suggested a Mendelian dominant genetic defect common to the many autoimmune diseases and serologic reactions that was not linked to HLA or Gm. A significant effect of female sex was also documented. These studies suggest that Sjögren's syndrome results from the interaction of several HLA-linked and non-HLA-linked genes.

Adult↗
PubMed1984

Neonatal lupus erythematosus. A clinical, serological and immunogenetic study with review of the literature.

Neonatal lupus erythematosus (NLE) is an inflammatory disorder of neonates characterized by transient cutaneous lesions and/or congenital heart block. The cutaneous lesions usually heal with minimal scarring, but healing may be delayed for many months in occasional cases. Photosensitivity is recognized as a component of this syndrome. A large proportion of this maternal population is asymptomatic, although the mothers' potential risk for developing CTD in the future remains to be determined. Moreover, this maternal group may exhibit a tendency to fetal wastage. La(SSB) and/or Ro(SSA) antibody is almost universally present in the sera of the neonatal lupus mothers and their infants. Since these antibodies may have a pathogenetic role in NLE, screening of infants with isolated CHB and/or cutaneous lesions suggestive of LE, and their mothers, for the presence of Ro(SSA) and La(SSB) antibodies is strongly recommended. HLA studies reveal that infants of Ro-positive mothers bearing the HLA, A1, B8, DR3, MB2 and MT2 phenotypes are at increased risk of developing neonatal lupus, in sharp contrast to infants of Ro-positive mothers bearing the DR2 and/or MB1/MT1 phenotypes. Recognition of the protean manifestations of this complex disorder by obstetricians, pediatricians, cardiologists, and dermatologists will undoubtedly lead to increased detection of NLE and afford further opportunity to elucidate more fully the etiology of this syndrome.

Antibodies, Antinuclear↗
PubMed1983

Antinuclear antibody determinations in Ro(SSA)-positive, antinuclear antibody--negative lupus and Sjögren's syndrome patients.

Antinuclear antibody determinations were performed on the sera of twenty-eight Ro(SSA)-positive patients, who failed to demonstrate significant antinuclear antibody titers on mouse liver, with the use of various heterologous and homologous substrates. These studies demonstrated the following: (1) The existence of substrate specificity among various tissues and cells employed in the detection of antinuclear antibodies in Ro(SSA)-positive patients. (2) The existence of a significant group of Ro(SSA)-positive lupus patients with prominent cutaneous findings and systemic disease who, despite the employment of a variety of antinuclear substrates, failed to demonstrate significant antinuclear antibody titer.

Animals↗
PubMed1983

Serologic studies in patients with lupus erythematosus and psoriasis.

We present four patients with coexistent lupus erythematosus (LE) and psoriasis. This is an unusual combination. All four patients had antibodies to Ro, which were absent in twenty-four control psoriatics. Antibodies to Ro occur in only 25% to 30% of unselected SLE patients, but occur in approximately 60% of "antinuclear antibody (ANA)-negative" LE patients, many of whom are highly photosensitive. The increased frequency of anti-Ro in our patients suggests that this may be a specific serologic marker for the LE/psoriasis overlap. Also, since anti-Ro correlates with photosensitivity, LE/psoriasis overlap patients may be at increased risk for photosensitivity, which occurred in two of our patients, one of whom developed severe systemic disease following ultraviolet (UVB) phototherapy. Screening for anti-Ro antibodies may be appropriate in psoriatics prior to UVB phototherapy.

Adult↗
PubMed1983

Value of immunofluorescent techniques in studies of bullous disease.

The findings from immunofluorescent techniques have made a profound contribution to our knowledge of bullous diseases. The techniques, once considered investigational, are now routinely employed in dermatologic diagnostic tests. Direct immunofluorescent methods applied to specimens of perilesional skin are extremely reliable to demonstrate characteristic deposition of various immunoreactants in pemphigus vulgaris, bullous pemphigoid, and dermatitis herpetiformis. Autoantibodies in serum revealed by indirect immunofluorescent techniques, are demonstrable in most cases of pemphigus vulgaris and bullous pemphigoid. The disappearance of these antibodies from the skin and serum of patients so afflicted appears to herald remission of their diseases. Thus, detection of these antibodies may be employed to regulate therapy for maximum effect.

Dermatitis Herpetiformis↗
PubMed1983

Familial systemic lupus erythematosus: immunogenetic studies in eight families.

Familial SLE provides a unique opportunity to study the relationships of previously associated genetic factors (HLA and complement component deficiencies) to the occurrence of SLE, other immune disorders, and autoantibodies in families. Thus, eight families containing two or more affected members with SLE (n = 22) and their relatives (n = 40) were examined for HLA genotypes, complement components and autoantibodies. Among the 40 non-SLE relatives, 7 (18%) had other immune diseases, including thyroid disease in 2, rheumatoid arthritis in 2, ITP in 2, and Henoch-Schönlein purpura in one. This compared to 4 of the 22 SLE patients (also 18%) of whom 3 had thyroid disease and one PSS. Eleven non-SLE relatives (28%) had ANA, which was in high-titer in 5 (13%). Eleven (28%) had antibodies to ssDNA, and one had a BFP. Only the SLE patients had antidsDNA, Ro(SSA), Sm or nRNP. A heterozygous C2 deficiency (C2D) was found in only one of the seven kindreds studied, and followed an A25, B18 DR7 haplotype. Heterozygous C2D, however, was inherited by only one of three family members with SLE. HLA-DR2 occurred in 36% and DR3 in 36% of SLE patients, which was not significantly different from either non-SLE family members or unrelated local controls. Sib pair analysis of seven sets presented here and seven from two published reports, demonstrated only random distribution with SLE. Similarly, other immune disorders and autoantibodies followed no consistent HLA haplotypes or DR specificities. Interestingly, DR2 and/or DR3 were found in five of the six patients (83%) having anti-ro(SSA) antibodies (p = NS). These data strongly suggest that genetic factors (other than HLA and complement component deficiencies) and/or environmental factors are necessary for the expression of SLE and other immune abnormalities in lupus families.

Adult↗
PubMed1983

Cutaneous manifestations of primary Sjögren's syndrome: a reflection of vasculitis and association with anti-Ro(SSA) antibodies.

Twenty-two symptomatic primary Sjögren's syndrome patients with clinical and histologic evidence of skin disease were studied. Purpura and urticaria were the most frequent clinical cutaneous manifestations. Most of these lesions were associated with a leukocytoclastic angiitis and, less commonly, a mononuclear (lymphocytic) vasculitis. Two main cutaneous syndromes were identified whose clinical, serologic, and histopathologic features were indistinguishable from Waldenström's benign hyperglobulinemic purpura and hypocomplementemic urticaria-like vasculitis. Eighty-four percent of primary Sjögren's syndrome patients with vasculitis demonstrated anti-Ro(SSA) antibodies. Thus, purpura and urticaria may reflect cutaneous vasculitis occurring in the clinical setting of Sjögren's syndrome.

Adolescent↗
PubMed1983

Sjögren's syndrome: association of anti-Ro(SS-A) antibodies with vasculitis, hematologic abnormalities, and serologic hyperreactivity.

The clinical significance of autoantibodies to Ro(SS-A) and La(SS-B) in Sjögren's syndrome was examined in a retrospective evaluation of 75 patients with symptoms of the sicca complex who had either primary Sjögren's syndrome or Sjögren's syndrome associated with another connective tissue disease. The clinical, hematologic, and serologic features associated with autoantibodies to the small molecular weight ribonucleoproteins Ro(SS-A) and La(SS-B) were ascertained. A striking clinical association of anti-Ro(SS-A) antibodies (found in 33 patients) with extraglandular disease (vasculitis, purpura, and lymphadenopathy) was seen. Hematologic abnormalities (anemia, leukopenia, and thrombocytopenia) were also associated with the presence of anti-Ro(SS-A) antibodies. Furthermore, anti-Ro(SS-A) antibody was associated with hyperglobulinemia, increased serologic reactivity in terms of rheumatoid and antinuclear factors, cryoglobulinemia, and hypocomplementemia. The presence of anti-Ro(SS-A) antibodies defines a subset of patients with Sjögren's syndrome who have systemic clinical manifestations including vasculitis, hematologic abnormalities, and serologic hyperreactivity.

Antibodies, Antinuclear↗
PubMed1983

Immunogenetics of the neonatal lupus syndrome.

Infants with neonatal lupus erythematosus have congenital heart block, transient cutaneous lesions, or both. Mothers of these infants have SSA/Ro autoantibodies that are passed across the placenta to the fetus and that have been temporarily associated with the syndrome. Six families with neonatal lupus were studied by HLA typing. All seven infants had transient cutaneous lesions, congenital heart block, or both. Five of six mothers were asymptomatic and one had Sjögren's syndrome. Six of seven infants and all six mothers had antibodies to SSA/Ro in their sera. The infants became seronegative by age 8 months. Five mothers were positive for HLA-DR3, five for HLA-MB2, six for HLA-MT2, and six for HLA-B8. No HLA associations were seen in infants. Gene products of the DR or similar regions may be associated with autoantibody production but not with other events in tissue injury.

Antibodies, Antinuclear↗
PubMed1983

Characterization of the inflammatory infiltrate in lupus erythematosus lesions using monoclonal antibodies.

The inflammatory infiltrates of cutaneous lupus lesions from 14 patients with benign cutaneous, subacute cutaneous or systemic lupus erythematosus were examined for T and B lymphocytes, macrophages and Ia positive cells using monoclonal antibodies and a peroxidase antiperoxidase technique. T cells and Ia positive cells were present in abundance followed by B cells then macrophages. Approximately equal numbers of helper/inducer T cells (OKT4, Leu3a) and suppressor/cytotoxic T cells (OKT8, Leu-2a) were present in the inflammatory infiltrate.

Adult↗