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Biomedical subjects

T T Provost

Publications and source records attributed to T T Provost.

At least 109 records · Page 6Linked to original sources

Sjögren syndrome: central nervous system manifestations.

We studied eight patients who had primary Sjögren syndrome and central nervous system (CNS) disorders that were not attributable to other causes. Focal cerebral deficits were observed in five patients. Aseptic meningoencephalitis was seen in five patients, recurrent in one. Spinal cord manifestations in three patients took several forms: acute transverse myelitis, chronic progressive myelopathy, and spinal subarachnoid hemorrhage. Precipitating antibodies to the Ro(SSA) cytoplasmic antigen were detected in the sera of seven of eight patients. This may be relevant to the pathogenesis of CNS disease in Sjögren syndrome, because there is a strong correlation between vasculitis and the presence of anti-Ro(SSA) antibodies in this connective tissue disorder.

Adult↗

Bullous scleroderma.

Bullae arose in two patients with scleroderma and in one patient with a similar condition, eosinophilic fasciitis. Biopsy specimens from two of the patients demonstrated prominent dermal lymphatic dilation. We hypothesize that these lesions occurred after the development of lymphatic obstruction secondary to dermal sclerosis.

Adult↗

Circulating immune complexes of IgA type in dermatitis herpetiformis.

There is some evidence that dermatitis herpetiformis may be mediated by circulating immune complexes. This study attempts to define the antibody class of these complexes. All patients studied demonstrated granular deposition of IgA in the papillary dermis on direct immunofluorescence. Serum immune complexes were detected using the qualitative Raji cell immunofluorescent assay, as well as the quantitative immunoradiometric assay. A group of 25 dermatitis herpetiformis patients was found to have higher levels of IgA containing complexes compared to a group of normals (p < .01). Higher levels of IgG containing complexes were also noted in dermatitis herpetiformis patients at a low level of statistical significance (p < .1). The relationship of these complexes to the pathogenesis of dermatitis herpetiformis is yet to be determined.

Antigen-Antibody Complex↗

Unusual cutaneous manifestations of systemic lupus erythematosus: I. Urticaria-like lesions. Correlation with clinical and serological abnormalities.

Ten of 143 systemic lupus erythematosus patients demonstrated urticaria-like lesions. Lesional biopsies in 7 of 9 patients tested revealed a leukocytoclastic angiitis and in 2, a mononuclear perivascular infiltrate. Direct immunofluorescent studies in 2 of 6 patients tested revealed IgM and C3 deposition in and about dermal blood vessels. Nine of the 10 systemic lupus erythematosus, patients displayed active clinical disease (e.g., arthritis, renal disease, etc.), a positive lupus band test, antibodies against deoxyribonucleic acid or Sm macromolecules, serum hypocomplementemia and markedly elevated quantities of serum immune complexes as determined by an immunoradiometric assay employing Raji cells. Similar lesions were not detected in 35 discoid lupus erythematosus patients. These studies strongly suggest: (1) urticaria-like lesions are uncommon cutaneous manifestations of systemic lupus erythematosus. (2) These urticaria-like lesions do not represent a classic IgE mediated urticaria. (3) These urticaria-like lesions generally occur in lupus erythematosus patients demonstrating clinical and/or serological evidence of systemic disease activity. (4) These lesions are probably secondary to immune complex deposition. We, therefore, conclude that all urticarial lesions in lupus erythematosus patients should be biopsied and the patient evaluated for active systemic disease.

Antibodies, Antinuclear↗

Lupus band test in untreated SLE patients: correlation of immunoglobulin deposition in the skin of the extensor forearm with clinical renal disease and serological abnormalities.

This study demonstrated that 88% of untreated systemic lupus erythematosus patients with clinical renal disease displayed the deposition of immunoglobulin and complement at the dermal epidermal junction of the noninvolved light exposed extensor surface of the upper 1/3 of the forearm (P less than 0.005) (positive lupus band test). Eighty-five percent of these untreated systemic lupus erythematosus patients with anti-deoxyribonucleic acid antibodies (native and/or single stranded) (P less than 0.001) and 96% of systemic lupus erythematosus patients with hypocomplementemia had a positive lupus band test (P less than 0.001). Those systemic lupus erythematosus patients with a negative lupus band test or a positive lupus band test composed of pure IgM had a decreased incidence of renal disease, serum hypocomplementemia and anti-DNA antibodies. Their sera, however, frequently contained antibodies directed against nuclear ribonuclear protein or against the cytoplasmic non-nucleic acid glycoprotein termed Ro. On the contrary, 85% of systemic lupus erythematosus patients with a positive lupus band test composed solely or in part of IgG, had anti-DNA antibodies (P less than 0.001). Their sera also frequently contained anti-Sm antibodies. The lupus band test was found to be dynamic. In general, the appearance as well as the disappearance or the marked decrease in intensity and complexity of a positive lupus band test was found to correlate with disease exacerbation, remission and the appearance and disappearance of DNA antibodies and serum hypocomplementemia.

Antibodies, Antinuclear↗

Subsets in systemic lupus erythematosus.

In recent years, evidence from several laboratories has indicated that various subsets of systemic lupus erythematosus exist. Some of these subsets have distinctive mucocutaneous features but more importantly they have different prevalence of renal disease--thus different prognosis. These subsets are defined by the specificity of the serum antibodies the SLE patient possesses.

Antibodies, Antinuclear↗

Unusual subepidermal bullous diseases with immunologic features of bullous pemphigoid.

Sixty-seven patients with histologic and immunologic features of bullous pemphigold (BP) were evaluated. Eleven patients had a localized blistering disease that was predominantly confined to one area of the body, most commonly the lower extremities. Two patients displayed a dapsone-responsive blistering disease that was characterized by a flexural distribution of ten to 20 1-cm or less, intensely pruitic, subepidermal bullae and linear IgA basement membrane zone deposition. Two patients had a chronic recalcitrant generalized scarring, hyperkeratotic, subepidermal blistering eruption that demonstrated serologic and direct immunofluorescence (IF) findings of BP. One patient displayed grouped small vesicles surmounted on an erythematous base; the clinical diagnosis was dermatitis herpetiformis, but direct IF examination of the biopsy specimen showed features of BP. One patient with epidermolysis bullosa acquisita had serologic and direct IF features suggestive of BP.

Adult↗

Fate of pemphigus antibody following successful therapy. Preliminary evaluation of pemphigus antibody determinations to regulate therapy.

Six of 17 pemphigus patients (35%) treated during a six-year period with immunosuppressive agents and/or corticosteroids have had prolonged clinical and immunologic remissions off all therapy. All were treated until serum and tissue bound pemphigus antibodies could no longer be detected. The length of remission has ranged from 1 1/2 years to 4 years. Three of these six patients relapsed after being clinically and serologically free of pemphigus for 19, 20, and 48 months. Seven additional patients are clinically free of disease with insignificant pemphigus antibody titers of 10 or less. Therapy now is being discontinued gradually in these patients. This preliminary study demonstrates that (1) a large percentage of pemphigus patients may have a prolonged clinical and immunological remission after successful therapy; (2) maintenance therapy may not be required to preserve the remission; (3) monitoring serum and in vivo bound pemphigus antibody is of value in regulating therapy in pemphigus patients.

Adrenal Cortex Hormones↗

Bullous pemphigoid. Clinical and immunologic follow-up after successful therapy.

Thirty-six patients with bullous pemphigoid (BP) have been periodically evaluated for four years. This study demonstrates that BP may occur in a transient predominantly localized form that remits spontaneously, and most BP patients after successful therapy remain in prolonged clinical remission. In this study, all patients with active or recurrent disease had IgG and/or C3 basement membrane zone (BMZ) deposition. Serum anti-BMZ antibodies was an inconstant feature. In most instances, clinical remission of BP was associated with disappearance of BMZ ig and C3 deposition and serum BMZ antibodies. Fluorescein-conjugated, antihuman C3 appears to be a more sensitive immunoreagent than antihuman, class specific, immunoglobulin antisera in detecting positive BMZ staining in BP. Combined therapy with azathioprine plus prednisone appears to be superior to prednisone alone in the treatment of BP.

Adult↗

The immunopathology of herpes gestationis. Immunofluorescence studies and characterization of "HG factor".

Nine skin biopsies from seven herpes gestationis patients were studied by immunofluorescence (IF) techniques. Basement membrane zone (BMZ) deposition of C3 and properdin was present in all nine skin specimens, while IgG deposition was apparent in only one. With in vitro C3 IF staining, positive BMZ staining (HG factor activity) was noted with all seven of our patients' serum samples tested. By standard indirect IF staining, however, only one of these serum samples contained BMZ antibodies of the IgG type. Two cord serum samples, tested by these same methods, yielded positive in vitro C3 staining (HG factor activity) but negative indirect IF staining (IgG). HG factor activity was found to be stable at 56 degrees C for 30 min and in two of three specimens at 56 degrees C for 1 h. Treatment of the complement source (normal human serum) used in the in vitro C3 staining assay with Mg2-EGTA or use of C2-deficient serum as the complement source inhibited HG factor activity. HG factor blocked the specific staining of the BMZ of normal human skin by labeled bullous pemphigoid antibodies. By sucrose density gradient ultracentrifugation and gel chromatography (Sephadex G-200), HG factor activity eluted with IgG-containing fractions. The highly purified IgG fraction of two herpes gestationis sera was also positive for HG factor activity. Our studies suggest that HG factor is an IgG antibody that may not be demonstrable by conventional IF methods, but which activates the classical complement pathway.

Basement Membrane↗

Gold nephropathy: serologic data suggesting an immune complex disease.

Servlogical tests preformed on a patient with gold-induced nephropathy revealed a multitude of immunological phenomena preceding the onset of proteinuria. These included formtaion of tissue antibodies, rheumatoid factors and circulating immune complexes. An antigen sharing immunological determinants with DOC-extractable tissue antigens was released into circulation before and during proteinuria. Precipitating antibodies against this circulating antigen were found in one serum sample obtained 6 weeks before the complication was diagnosed. In this serum specimen, antibodies were also found which combined with saline extracts, DOC extracts containing the ubiquitous tissue antigen (UTA), and preparations obtained from various human organs by extraction at 100 degrees C followed by precipitation at 71% ethanol concentration (BE preparations). Most of the activities disappeared before the onset of proteinuria. The possible significance of these phenomena in the pathogenesis of nephropathy is discussed.

Adult↗

Pemphigus and myasthenia gravis.

Various forms of true pemphigus have been reported to occur with myasthenia gravis, with and without thymoma, more frequently than can be ascribed to chance. A57-year-old woman with myasthenia gravis in remission developed pemphigus foliaceus. No roentgenologic evidence of a thymoma was present. Antinuclear antibodies caused interference with indirect immunofluorescent determination of pemphigus antibody. Indirect immunofluorescent studies showed no evidence for cross-reactivity between intercellular substance antibody and thymic components, as is found in the case of antiskeletal muscle antibodies with thymic myoid cells. The concurrence of these two diseases may involve the failure of a subgroup of thymic-dependent lymphocytes to suppress underlying autoimmune phenomena.

Animals↗