Clinical significance of serum properdin levels and properdin deposition in the dermal-epidermal junction in systemic lupus erythematosus.

117

Thirty-two biopsies of kidneys and of normal skin were performed simultaneously on patients with systemic lupus erythematosus (SLE) to determine whether the deposition of immune reactants in skin was correlated with the severity of renal injury or with several serologic measures of systemic disease activity. Immunofluorescent deposits at the dermal-epidermal junction (lupus band test) did not correlate with any clinical or histologic measures of glomerulonephritis or with serologic abnormalities. Immune deposits in dermal venules were found in 56% of the skin biopsies and were correlated with hypocomplementemia and higher levels of immune complexes in serum, and possibly with glomerular subendothelial electron dense deposits. Azathioprine therapy was correlated with absence of both sub- epidermal and vascular immune deposits. Immunofluorescent findings on serial skin biopsies on 13 patients were highly inconsistent. It is concluded that the lupus band test is clinically useful only as a diagnostic aid but not helpful in assessing renal or serologic activity of lupus, and that dermal vascular deposits of immunoglobulin or complement are more frequent than previously recognized and correlate with measures of circulating immune complexes.Systemic lupus erythematosus (SLE) is a disease in which evidence of immunologic injury can be found in many organ systems. The prognosis is determined most often by the degree of renal, particularly glomerular, involvement (1-3).Lupus glomerulonephritis (GN) is believed to result from deposition in the glomeruli of circulating complexes of immunoglobulin and nuclear antigens with complement, and a subsequent phlogistic response (43).Immune reactants are frequently found at the dermal-epidermal (D-E) junction in lupus skin lesions (6,7). Cormane reported in 1964 that the clinically normal skin of lupus patients also contains subepidermal immunoglobulins and complement (8). Immunofluorescent demonstration of these deposits in normal skin has a high degree of specificity for SLE (7) and is often referred to as the lupus band test (LBT).Whether the immunoglobulins in the skin and glomeruli are antibodies to the same nuclear antigens is

Section: Discussionmentioning

confidence: 90%

Deposition of antibodies to a soluble cytoplasmic antigen in the kidneys of patients with systemic lupus erythematosus

Maddison¹,

Reichlin²

1979

117

“…Immunofluorescence studies were performed as previously described (5 human immunoglobulins IgG, IgM, IgA, and and the complement components Clq, C3, and C4 conjugated with fluorescein isothiocyanate were obtained from Meloy Laboratories, Inc. (Springfield, Virginia). Anti-properdin was used in the indirect technique as previously described (6,7). The specificity and sensitivity of these antisera had been previously determined (6).…”

Section: Methodsmentioning

confidence: 99%

“…The indirect fluorescent antibody technique for antinuclear antibodies (ANA) was carried out by using mouse liver as previously described (7).…”

Section: Methodsmentioning

confidence: 99%

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Familial discoid lupus erythematosus associated with heterozygote c2 deficiency

Belin

Bordwell

Einarson

et al. 1980

Two siblings with chronic discoid lupus erythematosus and several family members were found with heterozygous C2 deficiency. An association with histocompatibility markers HLA‐B 18 and HLA‐D w2 was demonstrated, and the slow allotype of factor B was present. Linkage studies in this family suggested a close linkage between the C2 deficiency gene and genes coding for B18, Dw2, and BfS antigens. One HLA‐A CB/DBF recombinant was observed showing closer linkage between HLA‐D and Bf than between HLA‐B and Bf.

“…Tissue was processed for viewing by light and fluorescence microscopy. Imrnunofluorescence studies were done within 5 days of the biopsy, with fluorescein isothiocyanate (FITC) conjugated rabbit antisera to IgG, IgM, IgA, C3, and C4 as previously described (14). Properdin was detected by indirect immunofluorescence with rabbit antiproperdin (kindly supplied by Dr. 1.…”

Section: Skin Biopsiesmentioning

confidence: 99%

Pathways of complement activation in chronic discoid lupus

Schrager¹,

Rothfield²

1977

To assess the relative roles of the classic and alternative pathways of complement activation in chronic discoid lupus erythematosus (CDLE), serum levels of properdin, C3, and C4, and deposition of these proteins in the dermal-epidermal junction (DFJ) of 20 CDLE patients were compared to the findings in patients with clinicajly active and inactive SLE. Properdin was demonstrated in the DEJ of 10 of 14 (71%) histologically typical skin lesions from patients with CDLE, usually in association with deposits of immunoglobulin, C3, and C4. Properdin levels in CDLE patients were significantly increased (137 f 34%) ( P < 0.05) when compared to normal controls (101 f 18%) or to patients with clinically active SLE (89 f 32%). C3, C4, DNA-binding, and antinuclear antibody tests in CDLE were indistinguishable from those in normals, but significantly different from patients with active SLE ( P < 0.05). The complement profiles of patients with ______