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Biomedical subjects

R E Jordon

Publications and source records attributed to R E Jordon.

At least 55 records · Page 3Linked to original sources

Serologic and HLA associations in subacute cutaneous lupus erythematosus, a clinical subset of lupus erythematosus.

We studied the autoimmune serologic features and histocompatibility antigen associations of 27 patients who had a widespread, nonscarring and often photosensitive form of histologically specific cutaneous lupus erythematosus. We designated this disorder as subacute cutaneous lupus erythematosus. Skin lesions from this disorder can be distinguished from scarring discoid lupus erythematosus lesions both on a morphologic and histopathologic basis. Antinuclear and anticytoplasmic antibodies (Ro or Ro and La) and circulating immune complexes were frequently present in patients with subacute cutaneous lupus erythematosus, whereas rheumatoid factor and anti-lymphocyte, anti-DNA, anti-nRNP, and anti-Sm antibodies were found less frequently. Patients having annular skin lesions represented a particularly homogeneous subgroup in which there was a striking concordance of anti-Ro antibodies and the HLA-DR3 phenotype. These studies clearly establish that the presence of these lesions can serve as a cutaneous marker for a distinct subset of patients with lupus erythematosus who share similar clinical, serologic, and genetic features.

Adult↗

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.

Adult↗

Vasculitis in chronic urticaria: an immunopathologic study.

Forty-five patients with chronic urticaria were studied to determine: (1) the histologic incidence of leukocytoclastic vasculitis and (2) the clinical, laboratory and immunopathologic parameters that characterized this patient group. By histopathologic examination a spectrum of changes were noted as 9 patients showed leukocytoclastic vasculitis, 15 a dense perivascular infiltrate of lymphocytes and eosinophils, and 21 only a sparse lymphocytic perivascular infiltrate. Both the vasculitis and the dense infiltrate groups had an increased incidence of circulating immune complexes, as detected by Clq binding and monoclonal rheumatoid factor inhibition radioassays. Direct immunofluorescence showed blood vessel deposition of immunoglobulins, complement, and/or fibrin in 33% of the vasculitis group, 13% of the dense infiltrate group, and 9% of the sparse infiltrate group. These studies suggest that a meaningful number of patients with chronic urticaria have histologic and immunopathologic findings of vasculitis.

Adolescent↗

Dermatitis herpetiformis: immune complex detection with C1q and monoclonal rheumatoid factor.

To determine the significance of circulating immune complexes in dermatitis herpetiformis, serum samples from thirty patients with active disease were tested by a C1q binding radioassay, while serum samples from twenty-one of these patients were tested by a monoclonal rheumatoid factor (mRF) inhibition radioassay. By direct immunofluorescence, all patients demonstrated typical IgA deposition in dermal papillae. Using the C1q binding assay, only seven of forty-two serum samples had elevated C1q binding activity, while by the mRF inhibition assay, thirteen of twenty-five samples had elevated immune complex levels. Nine of these latter thirteen positive serum samples, however, were minimally elevated. Thus, IgG and/or IgM containing immune complexes are infrequently present, or at very low levels, in sera of patients with active dermatitis herpetiformis.

Antigen-Antibody Complex↗

An appraisal of routine direct immunofluorescence in vulvar disorders.

Sixty-four biopsies were obtained from patients with a variety of vulvar disorders for direct immunofluorescence study. Twelve of 24 vulvar biopsies from patients with lichen sclerosus demonstrated deposition of fibrin in the upper dermis, while 7 vulvar biopsies showed fibrin deposition at the dermal-epidermal junction. Blood vessel immunofluorescence was observed in several patients but was not a disease-specific finding. Patients with pemphigus and pemphigoid yielded characteristic reactions and served as positive controls. Direct immunofluorescence may be of value in assessing patients with vulvar disorders, and in particular lichen sclerosus.

Complement C3↗

Detection of circulating immune complexes in cutaneous disease using a monoclonal rheumatoid factor hemagglutination method.

A monoclonal rheumatoid factor (mRF) hemagglutination (HA) assay for the detection of circulating immune complexes is described. In this assay, human red blood cells coated with mRF were reacted in microtiter and, in the presence of circulating immune complexes, were agglutinated. Titers were determined and results were compared to a mRF inhibition (solid phase) radioassay (mRF-Inh). Serum samples [141] from patients with a variety of cutaneous diseases [78], systemic lupus erythematosus [19], rheumatoid arthritis [21] and normals [23] were tested by both methods. Eighty-four samples were positive by mRF-Inh and 75 mRF-HA. Seventy of the serum samples were positive by both methods for an overall correlation of 87%. When sucrose density gradient fractions were tested by both methods, results obtained by mRF-HA correlated with those obtained by mRF-Ihh in all patients studied. This procedure may be adapted for routine laboratory use and thus circumvents the need for costly equipment and hazardous radioactive materials.

Antibodies↗

Serum and blister fluid immune complexes in bullous pemphigoid: detection with C1q and monoclonal rheumatoid factor.

Eighty serum samples and 24 blister fluids from 51 patients with active bullous pemphigoid were tested for the presence of immune complexes by both a monoclonal rheumatoid factor (mRF) inhibition radioassay and a C1q-binding radioassay. Forty-two of the 80 serum samples were positive by the mRF assay, while 27 were positive by the C1q-binding assay. Antibody titres to the basement membrane zone did not correlate with levels of circulating immune complexes. Thirteen of 24 blister fluids had detectable immune complexes by the C1q assay, while only seven of 24 blister fluids were positive by the mRF assay. Sucrose density-gradient ultracentrifugation studies suggest that the mRF- and C1q-reactive substances in both bullous pemphigoid sera and blister fluids are of a size compatible with immune complexes. Although immune complexes are detectable in a high percentage of bullous pemphigoid patients, their role in this disease may be epiphenomenal rather than pathogenetic, merely reflecting the presence of autoantibody and soluble antigen.

Antigen-Antibody Complex↗

Cutaneous vascular immunofluorescence in rheumatoid arthritis. Correlation with circulating immune complexes and vasculitis.

The presence of immunoglobulin and complement in the cutaneous blood vessels of clinically uninvolved forearm skin was studied in 70 patients with rheumatoid arthritis, using immunofluorescent techniques. Patients with evidence of these immune deposits had a greater prevalence of circulating immune complexes, vasculitic skin lesions, subcutaneous nodules, high titer rheumatoid factor and other findings suggestive of active vasculitis. Biopsy of uninvolved forearm skin may be a useful tool in assessing those patients with rheumatoid arthritis suspected of having a systemic vasculitis.

Adult↗

Inflammation in acne vulgaris: leukocyte attraction and cytotoxicity by comedonal material.

The chemoattraction of comedonal material for leukocytes was evaluated. Material from open comedones attracted mononculear leukocytes but did not attract polymorphonuclear leukocytes. At higher concentrations, comedonal material was cytotoxic for leukocytes of both types. Of the comedonal components tested, free fatty acids produced the greatest cytotoxicity. The attraction and killing of leukocytes by comedonal components may be the mechanisms for the initiation or the enhancement (or both) of inflammation in acne vulgaris.

Acne Vulgaris↗

The complement system in bullous pemphigoid: VII. Fixation of the regulatory protein beta 1H globulin by pemphigoid antibody.

Using in vitro complement immunofluorescent staining methods, serum samples from 5 active cases of bullous pemphigoid, with pemphigoid antibody titers of 320 or greater, were tested for their ability to fix the regulatory protein beta 1H globulin in addition to C4 and C3. All 5 samples yielded positive C3, C4 and beta 1H staining reactions in a linear fashion along the basement membrane zone. Heat inactivation or treatment of the complement source (fresh normal human serum) with EDTA, Mg2-EGTA abolished all 3 staining reactions. Substitution of C2-deficient serum as the source of complement inhibited both C3 and beta 1H staining but had no effect on C4 staining. Use of serum devoid of beta 1H (R beta 1H) minimally enhanced C3 staining while no beta 1H staining was observed. The addition of beta 1H to R beta 1H restored positive beta 1H staining. Skin biopsies of perilesional skin from 6 patients with bullous pemphigoid demonstrated heavy in vivo deposition of beta 1H in addition to C3. These studies suggest that pemphigoid antibodies will fix the regulatory protein beta 1H in addition to other complement components, a phenomenon which requires activation of the classical complement pathway and generation of the C3b amplification convertase.

Antibodies↗

Erythema multiforme: direct immunofluorescence studies and detection of circulating immune complexes.

The immunologic parameters of 23 patients with erythema multiforme who were seen by us (17 patients) or who had biopsies sent for immunofluorescence testing (6 cases) are reviewed. Biopsy specimens were sectioned and tested with labeled antisera to human IgG, IgA, IgM, C3 and fibrin. Fourteen biopsies showed IgM deposits in the superficial blood vessels, 13 demonstrated C3, 15 showed fibrin deposition, and 1 biopsy showed IgA deposition. All biopsies were negative for IgG. Eight serum samples tested by indirect IF were negative for skin-reactive antibodies. In addition to IF testing, serum samples from 20 patients were tested for circulating immune complexes with a Clq binding radioassay and a monoclonal rheumatoid factor (mRF) inhibition assay. Immune complexes were not detected by the Clq binding assay, but 6 of 20 serum samples demonstrated low to moderate levels of immune complexes by the mRF inhibition assay. By sucrose density gradient ultracentrifugation in the mRF-reactive material in one serum sample sedimented in high molecular weight fractions and also demonstrated anticomplementary activity. These findings suggest that immune complex formation and subsequent deposition in the cutaneous microvasculature may play a role in the pathogenesis of erythema multiforme.

Antigen-Antibody Complex↗

Circulating immune complexes in systemic scleroderma and generalized morphea.

There is growing evidence that pathologic changes in the vascular system are implicated in the pathogenesis of systemic scleroderma. It has been suggested that immune complex deposition may be responsible for such changes. We measured circulating immune complexes in 10 patients with severe systemic scleroderma, 1 of whom had clinical evidence of renal disease, and in 3 patients with generalized morphea. None of the patients had significantly elevated levels. Our findings suggest that although circulating immune complexes are of diagnostic and prognostic value in other collagen vascular diseases, they do not play a major role in the pathogenesis of systemic scleroderma in patients who lack clinical evidence of renal disease.

Adult↗

Childhood pemphigus foliaceus. Report of a case.

A case is reported of a 4-year-old black boy with pemphigus foliaceus. The patient is unusual because of age, sex, race, and distribution of lesions, Confirmation of diagnosis was made by both routine histopathology and direct immunofluorescence microscopy. The patient responded rapidly to prednisone therapy.

Black People↗