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Biomedical subjects

T T Provost

Publications and source records attributed to T T Provost.

At least 55 records · Page 3Linked to original sources
PubMed1988

The relationship between anti-Ro (SS-A) antibody-positive Sjögren's syndrome and anti-Ro (SS-A) antibody-positive lupus erythematosus.

Ten Ro(SS-A) antibody-positive patients with Sjögren's syndrome and lupus erythematosus are described. These patients have a disease process characterized by the frequent appearance of annular polycyclic lupus lesions of subacute cutaneous lupus erythematosus (SCLE), as well as neurologic and pulmonary disease. The Ro(SS-A) antibody-positive patients may have Sjögren's syndrome for many years and then suddenly develop lupus erythematosus, and vice versa. These studies demonstrate that the patient with Ro(SS-A) antibody may exhibit a dynamic clinical disease expression over time and that there is a closer pathologic relationship between Sjögren's syndrome and SCLE in these patients with Ro(SS-A)-antibody than has previously been appreciated. Furthermore, Ro(SS-A)-positive patients with Sjögren's syndrome and lupus erythematosus appear to have a much more guarded prognosis than those Ro(SS-A)-positive lupus patients described under the classifications of antinuclear antibody-negative lupus erythematosus and SCLE.

Adult↗
PubMed1988

Thrombocytopenia in the neonatal lupus syndrome.

Thrombocytopenia has been documented infrequently in association with congenital heart block or lupus dermatitis in the neonatal lupus erythematosus syndrome. We report the cases of two infants with transient neonatal thrombocytopenia born to mothers with connective-tissue disease. Both mother/infant pairs were Ro(SS-A) antibody positive. Although the finding of neonatal thrombocytopenia in the presence of maternal connective-tissue disease suggests an autoimmune thrombocytopenia, platelet antibody studies were negative in both mother/infant pairs. We have found the Ro (SS-A) antibody with increased frequency in idiopathic thrombocytopenic purpura and thrombocytopenia associated with Sjögren's syndrome, but the nature of the association is unknown. We suggest that thrombocytopenia in our patients is a manifestation of the neonatal lupus erythematosus syndrome. This syndrome should be included in the differential diagnosis of neonatal thrombocytopenia.

Adult↗
PubMed1987

Sjögren's syndrome. Association of cutaneous vasculitis with central nervous system disease.

We describe a group of patients with Sjögren's syndrome, who commonly present to dermatologists with cutaneous manifestations of vasculitis. Two specific clinically recognizable forms of cutaneous vasculitis predominate: palpable purpura of the lower extremities (Waldenström's benign hypergammaglobulinemic purpura) and urticarialike vasculitis. Two pathologic types of cutaneous vasculopathy are demonstrated, one leukocytoclastic and the other mononuclear. The leukocytoclastic vasculopathy is associated with high titers of Ro(SS-A) and La(SS-B) autoantibodies (detected by gel double-diffusion techniques) and general serohyperreactivity. In marked contrast, the mononuclear vasculopathy is associated with low titers of Ro(SS-A) and La(SS-B) autoantibodies (detected by enzyme-linked immunosorbent assay but not gel double-diffusion techniques) and general serohyporeactivity. Approximately 70% of patients with Sjögren's syndrome and cutaneous vasculitis have also developed peripheral and/or central nervous system disease. The pathogenesis of the nervous system disease is unknown, but preliminary data suggest a vasculopathy.

Autoantibodies↗
PubMed1987

Sjögren's syndrome. Cutaneous, immunologic, and nervous system manifestations.

The studies recounted in this review have demonstrated that cutaneous vasculitis is a frequent extraglandular manifestation of primary Sjögren's syndrome. Two histopathologic types of vasculitis have been detected. One type, a leukocytoclastic angiitis, is found in association with high-titer anti-Ro(SS-A) antibodies, rheumatoid factor, hypergammaglobulinemia, and hypocomplementemia. The second type, a mononuclear inflammatory vasculopathy, in sharp contrast, is found in association with low-titer Ro(SS-A) antibodies, normocomplementemia, and absence of hypergammaglobulinemia and rheumatoid factor. Both types of vasculitis are found in association with peripheral nervous system and CNS disease. The peripheral nervous system and CNS disease involves the entire neuroaxis and preliminary data indicate that a vasculopathy is the cause of the peripheral nervous system and CNS disease. Evoked sensory response testing, CSF analysis, and MRI have proved to be very valuable techniques in investigating these patients with Sjögren's syndrome. Preliminary data suggest that high doses of prednisone or immunosuppressive agents are effective in treating these patients.

Autoantibodies↗
PubMed1987

The neonatal lupus erythematosus syndrome.

Available data support the idea that the neonatal lupus erythematosus syndrome results from an autoantibody produced in the mother and passed across the placenta to the fetus. Despite the evidence for the presence of the Ro(SSA) autoantigen both in the skin and heart and the almost universal presence of the Ro(SSA) autoantibody in mothers of infants with neonatal lupus, there are emerging data to suggest that La(SSB) and, rarely, nRNP antibodies play an important pathologic role in some cases of the neonatal lupus syndrome.

Antibodies, Antinuclear↗
PubMed1986

Gene interaction at HLA-DQ enhances autoantibody production in primary Sjögren's syndrome.

Primary Sjögren's syndrome is an autoimmune disorder characterized by dryness of the mouth and eyes. The human leukocyte antigen (HLA) locus DQ is related to the primary Sjögren's syndrome autoantibodies that bind the RNA proteins Ro/SSA and La/SSB. Both DQ1 and DQ2 alleles are associated with high concentrations of these autoantibodies. An analysis of all possible combinations at DQ has shown that the entire effect was due to heterozygotes expressing the DQ1 and DQ2 alleles. These data suggest that gene interaction between DQ1 and DQ2 (or alleles at associated loci), possibly from gene complementation of trans-associated surface molecules, influences the autoimmune response in primary Sjögren's syndrome.

Alleles↗
PubMed1986

Anti-Ro (SS-A) and anti-La (SS-B) in patients with Sjögren's syndrome.

Clinical, serologic, and genetic findings in Sjögren's syndrome patients were correlated with quantitative determinations for antibody against Ro (SS-A), La (SS-B), and nRNP (Sm) using newly developed, sensitive solid-phase assays. In 86 Sjögren's syndrome patient sera, more than 96% had anti-Ro (SS-A), and 87% had anti-La (SS-B), spanning a 4.8 log10 range of autoantibody concentration, whereas only 95% of the patients had anti-nRNP (Sm). Low levels of anti-Ro (SS-A) and anti-La (SS-B) were found in 10% and 12.5%, respectively, of the 40 normal control sera. In Sjögren's syndrome patients, the level of anti-Ro (SS-A) correlated strongly with that of anti-La (SS-B) (r = 0.80; P less than 0.0001) but not with the level of anti-nRNP (Sm). We found much higher levels of anti-Ro (SS-A) and anti-La (SS-B) in patients with purpura, leukopenia, lymphopenia, and increased polyclonal gamma globulins than in those without these conditions (between 4.3-fold and 17-fold higher; P less than 0.001 to P less than 0.05). Anti-Ro (SS-A) and anti-La (SS-B) levels correlated with the rheumatoid factor titer and with the concentrations of total globulin, IgG, and IgA, but not with the IgM concentration. The association of rheumatoid factor titer with levels of anti-Ro (SS-A) and anti-La (SS-B) occurred only in patients with primary Sjögren's syndrome. Antinuclear antibody titers correlated with levels of anti-Ro (SS-A) and anti-nRNP (Sm). HLA-DR3-positive patients had higher levels of anti-Ro (SS-A) and anti-La (SS-B).

Antibodies, Antinuclear↗
PubMed1986

Primary Sjögren's syndrome in men. Clinical, serologic, and immunogenetic features.

Although primary Sjögren's syndrome is a common rheumatic disorder in women, it is not well recognized in men. This study represents the first report of the clinical, serologic, and immunogenetic features of a group of 36 men with primary Sjögren's syndrome, which are contrasted with those of a group of 69 women with primary Sjögren's syndrome. The majority of male patients had extraglandular involvement including articular (78 percent), neurologic (39 percent), inflammatory vascular (25 percent), and lymphoproliferative disorders (17 percent). Although men were at the same risk for the development of extraglandular complications, there were significant serologic and immunogenetic differences. In sharp contrast to women with Sjögren's syndrome, men with Sjögren's syndrome were seronegative with respect to the presence of serum rheumatoid factor (p = 0.008) and antibodies to Ro(SS-A) (p = 0.016). The supertypic specificity, MT2 (DRw52), as in women, was strongly associated with primary Sjögren's syndrome in men when compared with race-matched control subjects (p = 0.0015). In men, however, the frequency of HLA-B8 and HLA-DR3, the most common DR locus specificity observed in women, was not statistically different from that observed in the normal control group.

Adult↗
PubMed1986

A sensitive and specific assay to detect Ro(SS-A) and La(SS-B) antibodies.

This paper describes a sensitive and specific immunoblotting procedure to detect Ro(SS-A) and La(SS-B) autoantibodies in the serum of patients with lupus erythematosus. In order to perform this procedure, we have partially purified the Ro(SS-A) and La(SS-B) antigens from human spleen extracts by DEAE-cellulose and Sephacryl S-300 chromatography. The Ro(SS-A) and La(SS-B) antigens were immobilized on nitrocellulose paper after sodium dodecyl sulfate-polyacrylamide gel electrophoresis and tested against a panel of sera at different dilutions: normal human sera (n = 14), Ro(SS-A) antisera (n = 6), La(SS-B) (n = 7), Ro(SS-A)/La(SS-B) (n = 8), Sm-nRNP-La(SS-B) (n = 2). We found that Ro(SS-A) antisera react with a protein of an approximate Mr of 58K, whereas the La(SS-B) antisera reacted with two bands of Mr 42 and 40K, respectively. Antisera with both autoantibodies [Ro(SS-A) and La(SS-B)] reacted with both antigens, whereas the control NHS, anti-nRNP, and anti-Sm did not stain the Ro(SS-A) and La(SS-B) protein bands. In addition, some of the positive sera continued reacting with the respective antigens at extremely high dilutions. This procedure can be easily adapted to test many serum samples and produce data which include Mr of the antigen and titer of autoantibodies in the patient's serum.

Antibodies, Antinuclear↗
PubMed1986

Primary Sjögren's syndrome with central nervous system disease mimicking multiple sclerosis.

Central nervous system involvement has occurred in approximately 20% of patients with primary Sjögren's syndrome evaluated at our institution. Characteristically, the neurologic dysfunction is multifocal, involving both the brain and spinal cord, and is recurrent over time. We present the features of 20 patients with primary Sjögren's syndrome and central nervous system involvement whose neurologic findings, evoked potential abnormalities, and cerebrospinal fluid profiles (elevated IgG indices, oligoclonal bands on agarose gel electrophoresis, and mild pleocytosis with reactive lymphoid cells) closely resembled those of multiple sclerosis. In fact, multiple sclerosis was considered the most likely diagnosis in each of these patients before diagnosis of Sjögren's syndrome, and each patient met criteria for definite multiple sclerosis. The clinical effects of corticosteroid treatment during episodes of acute neurologic dysfunction appeared to be beneficial in these patients.

Adult↗
PubMed1986

Molecular characterization of Ro(SSA) and La(SSB) proteins.

Ro(SSA) and La(SSB) proteins were characterized using human spleen extract as antigen and monospecific anti-Ro(SSA) and anti-La(SSB) sera. The antigens were partially purified by DEAE cellulose and Sephacryl chromatography, and then were electrotransferred to nitrocellulose sheets after SDS-PAGE electrophoresis. The anti-Ro(SSA) sera had reacted with a polypeptidic band of 58 kilodaltons (kDa), meanwhile the La(SSB) antisera reacted with 2 polypeptidic bands of 42 and 40 kDa. The molecular weight of the Ro(SSA) and La(SSB) proteins obtained by gel filtration chromatography was in agreement with the PAGE/electrotransfer results. These procedures are useful tools for the molecular analysis of the markers in the connective tissue diseases.

Antigens↗
PubMed1986

Fetal wastage in women with anti-Ro(SSA) antibody.

Retrospective obstetric histories were obtained in 50 anti-Ro(SSA) antibody positive women (aged 15-50 years) and 47 anti-nRNP antibody positive women of similar age range. Twenty (40%) anti-Ro(SSA) positive women and 33 (70%) anti-nRNP positive women fulfilled the revised ARA criteria for systemic lupus erythematosus (SLE). Thirty-four anti-Ro(SSA) positive women had a total of 84 pregnancies with a fetal loss of 28%; 29 anti-nRNP positive women had a total of 63 pregnancies with a fetal loss of 19%. Although the overall frequency of fetal wastage between the 2 groups was not significantly different, analysis of the subgroup of SLE patients demonstrated a significantly greater fetal wastage in black anti-Ro(SSA) positive women (15 of 21, 71%) compared with black anti-nRNP positive women (5 of 28, 18% p less than .001). No significant difference in fetal wastage was noted between either the white SLE or the non-SLE women in either antibody group. These data suggest that black SLE patients with anti-Ro(SSA) antibody may be at increased risk of fetal wastage.

Abortion, Spontaneous↗
PubMed1985

Two types of inflammatory vascular disease in Sjögren's syndrome. Differential association with seroreactivity to rheumatoid factor and antibodies to Ro (SS-A) and with hypocomplementemia.

Two types of inflammatory vascular disease (IVD) occur in Sjögren's syndrome: neutrophilic IVD (NIVD) and mononuclear IVD (MIVD). In 45 patients with Sjögren's syndrome, we examined the 2 types of IVD with respect to serologic associations. NIVD, unlike MIVD, was significantly associated with seroreactivity reflected by hyperglobulinemia (P = 0.01), rheumatoid factor (P = 0.002), antinuclear antibodies (P = 0.02), and antibodies to Ro (SS-A) (P = 0.00006), and with hypocomplementemia (P = 0.03). The differential association of the 2 types of IVD with serologic reactivity and hypocomplementemia suggests that there may be basic differences in the immunopathogenesis of these 2 forms of IVD in Sjögren's syndrome.

Adolescent↗
PubMed1985

Peripheral inflammatory vascular disease in Sjögren's syndrome. Association with nervous system complications.

Two histopathologic types of inflammatory vascular disease (IVD) occur in Sjögren's syndrome (SS): mononuclear IVD (MIVD) and neutrophilic IVD (NIVD). We describe 50 SS patients with IVD (30 with NIVD and 20 with MIVD). Thirty-three (66%) of the SS patients with biopsy-documented IVD had nervous system disease unattributable to other causes. Nineteen patients (58%) had involvement of both the central and peripheral nervous systems, while 9 had peripheral and 5 had central nervous system dysfunction alone. Patients with both histopathologic types of IVD were at risk for the development of nervous system abnormalities (57% of NIVD patients and 80% of MIVD patients). Indirect evidence is presented which suggests that IVD may play a role in the immunopathogenesis of nervous system disease, at least in a subset of SS patients.

Adolescent↗
PubMed1985

Nucleoprotein autoantibodies in lupus erythematosus.

Autoantibodies against deoxyribonucleic acid (DNA) and ribonucleic acid (RNA) proteins are commonly detected in patients with lupus erythematosus (LE). Antibodies against native DNA are frequently detected in a subset of LE patients with a high prevalence of renal disease. Single-stranded DNA antibodies are also commonly detected in patients with systemic lupus erythematosus (SLE) but recent evidence indicates that approximately 25% of patients with benign, cutaneous (discoid) lupus also possess single-stranded DNA IgM autoantibodies. LE patients also frequently possess antibodies directed against a variety of ribonuclear proteins (RNP). These RNA protein autoantibodies are generally divided into two groups. One group is termed snRNPs (small nuclear ribonuclear protein); the other is termed scRNPs (small cytoplasmic ribonuclear protein). Anti-RNA protein autoantibodies occur as frequently in patients with SLE as do native DNA antibodies. Furthermore, in contradistinction to nDNA antibodies, lupus patients generally make large quantities (detected by gel precipitin techniques) of anti-RNP antibodies. The anti-RNP antibodies are directed against proteins that bind with specific RNA nucleotides. The best evidence at present indicates that these RNA proteins containing the specific RNA nucleotides are involved in RNA processing and post-translational activities such as protein synthesis. Furthermore, these SLE autoantibodies are now being employed, together with other autoantibody systems detected in other connective tissue diseases, to define the biological role of the respective RNA proteins.

Autoantibodies↗
PubMed1985

Neuropsychiatric dysfunction in primary Sjögren's syndrome.

Neuropsychiatric complications developed in 40 patients with primary Sjögren's syndrome, none of whom met American Rheumatologic Association criteria for systemic lupus erythematosus. Twenty-five patients had psychiatric abnormalities, the commonest of which were affective disturbances. Of 30 patients tested, 23 had an abnormal pattern in the Minnesota Multiphasic Personality Inventory, the commonest pattern being a "conversion V." In general, patients presented with hysteroid dysphoric features. Of 16 patients undergoing cognitive function testing, 7 showed mild memory impairment with attention and concentration deficits. On clinical evaluation, 27 patients had neurologic abnormalities unattributable to other causes (central and peripheral nervous system in 16 and 19 patients respectively). There was a significant correlation between psychiatric disturbances and neurologic dysfunction, suggesting a possible organic basis for psychiatric dysfunction. The diagnosis of primary Sjögren's syndrome should be considered in patients with unexplained neuropsychiatric illness.

Adolescent↗
PubMed1985

Systemic lupus erythematosus: a review of clinico-laboratory features and immunogenetic markers in 150 patients with emphasis on demographic subsets.

Clinical and laboratory features as well as immunogenetic markers were analyzed in 150 patients with SLE to determine if demographic factors--age at diagnosis, sex and race--influenced the expression of disease. The overall series included 103 white females, 35 black females, 10 white males and 2 black males; the mean age at diagnosis was 32.5 years. Males had a significantly older mean age at diagnosis than females (40.4 versus 31.8 years) and a significantly higher frequency of peripheral neuropathy (50% versus 18.8%). No other differences in clinical or laboratory features or HLA-DR or DQ phenotype frequencies were noted. Blacks had a significant younger mean age at diagnosis than whites (26.9 versus 33.4 years) as well as significantly higher frequencies of nephritis, hypertension, acute lupus pneumonitis, discoid rash, hyperglobulinemia and hypocomplementemia. There were no differences in autoantibody frequencies between race-specific subgroups. HLA-DR2, DRw52 and DQ1 were significantly associated with SLE in whites compared to controls; no HLA-DR or DQ associations were found with SLE in blacks. In whites, HLA-DR2 was associated with the presence of anti-Ro(SS-A) antibody while HLA-DR3 was associated with the presence of both anti-Ro(SS-A) and anti-La(SS-B) antibody. In blacks, HLA-DR2 was associated with the presence of anti-nDNA antibody. In whites, patients with late-onset SLE (age at diagnosis greater than or equal to 50 years) had significantly lower frequencies of nephritis and mesenteric vasculitis but, on the other hand, a higher frequency of secondary Sjögren syndrome than patients with age at diagnosis less than or equal to 22 years. Similar findings were noted when blacks aged 35 and above were compared to those aged 17 and below at diagnosis. In whites, the frequency of both anti-Ro(SS-A) and La(SS-B) antibodies increased with increasing age as did that of HLA-DR3; HLA-DR2, however, was more frequent in those with younger age at diagnosis. These data suggest the existence of two serologic-genetic subsets of SLE with different age at diagnosis.

Adolescent↗