Biomedical subjects
T T Provost
Publications and source records attributed to T T Provost.
Necrotizing arteritis and spinal subarachnoid hemorrhage in Sjögren syndrome.
A 37-year-old woman with primary Sjögren syndrome developed mixed cryoglobulinemia and systemic vasculitis. Subarachnoid hemorrhage occurred as a result of necrotizing anterior spinal arteritis. Although rarely seen in mixed cryoglobulinemia, central nervous system complications have recently been documented in Sjögren syndrome. The patient's serum contained antibodies to the Ro(SSA) cytoplasmic antigen, and these antibodies were concentrated in the cryoglobulin fraction. Anti-Ro(SSA) antibodies are associated with the occurrence of vasculitis in patients with Sjögren syndrome, which suggests that the spinal arteritis and subarachnoid hemorrhage in this patient may have been directly related to the underlying connective tissue disorder.
Antibodies to single-stranded DNA in patients with discoid lupus erythematosus.
Sera from 31 patients with discoid lupus erythematosus were examined for the presence of antibodies to single-stranded and native DNA. None of these sera had antibodies to native DNA. When rabbit anti-human IgM was used, 6 of 28 patients had elevated binding, whereas only 1 of 29 patients had abnormal binding when antiIgG was used. The IgM nature of this response was confirmed when we demonstrated the binding activity in heavy fractions from gel filtration. We concluded that at least one-fifth of patients with discoid lupus erythematosus have IgM antibodies to single-stranded DNA.
Interrelationships of HLA-DR, MB, and MT phenotypes, autoantibody expression, and clinical features in systemic lupus erythematosus.
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Pemphigus foliaceus: a case with serologic features of Senear-Usher syndrome and other autoimmune abnormalities.
The two variants of superficial pemphigus, pemphigus erythematosus (Senear-Usher syndrome) and pemphigus foliaceus, share common histopathologic and indirect immunofluorescence findings. They differ in that pemphigus erythematosus is limited in distribution and usually has concomitant basement membrane zone deposition of immunoglobulin and complement in lesional skin in addition to intercellular space staining in the epidermis, whereas pemphigus foliaceus is a generalized process that reputedly lacks basement membrane zone staining. A 49-year-old man with an array of immunologic abnormalities, including pernicious anemia and Sjögren's syndrome consistent with broad immunologic dysfunction, developed generalized superficial pemphigus (pemphigus foliaceus) with concomitant basement membrane zone immunoreactant deposition. The demonstration of basement zone immunofluorescent staining lends additional support to the basic viewpoint that pemphigus erythematosus and pemphigus foliaceus are variable phenotypic expressions of the same basic disease process.
Neurologic complications of primary Sjögren's syndrome.
Although peripheral nervous system disease has been well documented in Sjögren's syndrome (SS), central nervous system (CNS) involvement is considered distinctly uncommon. Sixteen patients with primary SS and CNS disorders not attributable to other causes were the subjects of this study. Cerebral manifestations, both focal and diffuse, as well as spinal cord disease, were observed. Peripheral vasculitis occurred in 12 patients (75%), 83% of whom had anti-Ro(SSA) antibodies. The high proportion of patients with concomitant peripheral vasculitis, and the observed association with antibodies to the Ro(SSA) antigen system which, in other studies, has been linked to vasculitis in SS, suggest that an immune vasculopathy may play a role in the pathogenesis of the CNS disease of SS.
Induction of IgA circulating immune complexes after wheat feeding in dermatitis herpetiformis patients.
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Urticaria and Urticaria-like lesions.
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C2 deficient systemic lupus erythematosus: its association with anti-Ro (SSA) antibodies.
C2 deficiency is the most common complement component deficiency. While individuals with C2 deficiency may be completely normal, a lupus erythematosus-like disease process has developed in some. A 28-year-old woman had a chronic photodermatitis, arthralgias, and mesangial lupus nephritis, She has C2 deficiency associated with an HLA-Dw2 transplantation antigen. Her serum has shown antibodies to the macromolecule Ro (the A antigen in Sjögren's syndrome), while failing to demonstrate antinuclear antibodies in routine laboratory determinations.
Cutaneous immunoglobulin deposition in primary biliary cirrhosis.
We studied the skin of six patients with primary biliary cirrhosis (PBC) to determine if there is cutaneous deposition of complement and immunoglobulins in PBC. We also investigated serum samples from these patients for the presence of circulating antibodies against nuclear and cytoplasmic antigens. Three of five patients demonstrated immunoglobulins (IgM or IgG) or complement (C3) deposition at the dermoepidermal junction (DEJ) in clinically normal, light-protected skin. Two of six patients had 2- to 5-mm papular-pustular skin lesions as a manifestation of PBC. Serological studies in these patients disclosed low antinuclear antibody titers in three patients and anti-single-stranded DNA antibody titers in the two patients with skin lesions. These histological, immunofluorescent, and serological findings offer further evidence of the occurrence of circulating immune complexes in at least some patients with PBC.
Pemphigus vulgaris. Superior sensitivity of monkey esophagus in the determination of pemphigus antibody.
This study demonstrates that monkey esophagus is a more sensitive substrate than guinea pig esophagus for the demonstration of serum antibodies, in patients with pemphigus vulgaris. In this study, for example, five patients with active pemphigus who had demonstrable pemphigus antibodies on monkey esophagus substrate failed to demonstrate pemphigus antibody activity when guinea pig esophagus was employed as a substrate. Furthermore, pemphigus antibody titers determined on monkey esophagus substrate frequently exceeded, by several dilutions, titers detected with guinea pig esophagus. By contrast, in no instance did we detect pemphigus antibody titer activity on guinea pig esophagus in excess of the pemphigus antibody activity detected on monkey esophagus. The reason for the disparity in the sensitivity of the guinea pig and monkey esophagus substrates is unknown.
Ro (SSA) and La (SSB) antibodies.
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Antinuclear antibody-negative systemic lupus erythematosus. I. Anti-Ro(SSA) and anti-La(SSB) antibodies.
Anti-Ro(SSA) and anti-La(SSB) antibodies are found in approximately 25% of systemic lupus erythematosus patients. These antibodies are also detected in lupus patients who fail to demonstrate a significant antinuclear antibody (ANA) titer when mouse liver is employed as the substrate. Approximately 5% to 10% of systemic lupus patients are in this category. These ANA-negative, Ro(SSA)-positive lupus patients generally demonstrate a prominent widespread photosensitive dermatitis and are frequently initially seen and evaluated by a dermatologist.
Serological findings in patients with "ANA-negative" systemic lupus erythematosus.
Serological studies were performed on sera from 66 patients with the clinical picture of systemic lupus erythematosus (SLE). These sera failed to give a positive antinuclear antibody test when tested on cryostat sections of mouse liver and thus these patients' sera appear to be ANA negative. Precipitating antibodies to the cytoplasmic antigen Ro were found in 41 cases and of the remaining 25 sera, 18 were found to have antibodies to single stranded DNA detectable by radioimmunoassay. Thus, 50 of the 66 patients exhibited serological findings which are commonly found in ANA positive SLE patients. Studies with KB cells as immunofluorescent substrate revealed that 66% of these sera were positive for nuclear staining demonstrating that at least part of the failure of these sera to stain mouse liver is due to antigenic deficiency of this substrate. The clinical picture of these patients was dominated by a severe photosensitive dermatitis but more than half of the patients had widespread multisystem disease. As a group these patients had a low frequency of nephritis and neuropsychiatric disease. Detection of these antibodies relates these patients serologically to other SLE patients and suggests that they are best perceived as part of the clinical spectrum of SLE.
Neonatal lupus erythematosus: new serologic findings.
A child with neonatal lupus was evaluated and found to possess serum anti Ro(SSA) antibodies. The cutaneous lesions and anti Ro(SSA) antibodies disappeared during the next 5 mo. The infant's mother was asymptomatic but possessed anti Ro(SSA) and anti La(SSB) antibodies.
Epidermolysis bullosa acquisita: ultrastructural and immunological studies.
Four patients with epidermolysis bullosa acquisita were investigated using immunofluorescence, routine electron microscopic and immunoelectron microscopic techniques. Immunofluorescence studies demonstrated linear immunoglobulin and complement deposition along the dermal-epidermal junction. These findings are similar to those seen in skin of patients with bullous pemphigoid. Immunoelectron microscopic studies demonstrated that the immunoreactants were localized below the subbasal lamina-anchoring fibril zone of the basement membrane, thereby clearly distinguishing the immunopathology of epidermolysis bullosa acquisita from that seen in bullous pemphigoid. Indirect immunoelectron microscopic findings suggest that epidermal basal cells of affected patients may secrete the dermal substances to which the antibodies bind.