Glomerular deposition of properdin in acute and chronic glomerulonephritis with hypocomplementemia

Westberg, N. Gunnar; Naff, George B.; Boyer, John T.; Michael, Alfred F.

doi:10.1172/jci106534

Cited by 110 publications

(34 citation statements)

References 20 publications

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“…Westberg et al demonstrated that properdin, in addition to proteins of the classical pathway, was present in the glomerular basement membrane of 3 of 13 SLE patients (25). We have confirmed this observation and have shown that properdin is also present in the DEJ of skin lesions from SLE patients (26).…”

Section: Introductionsupporting

confidence: 84%

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

Schrager¹,

Rothfield²

1976

J. Clin. Invest.

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A B S T R A C T 61 biopsies of normal skin from the deltoid area and lesional skin from various sites from 48 patients with systemic lupus erythematosus (SLE) were studied for the presence of properdin, C3, C4, and immunoglobulins (IgG, IgM, and IgA) in the dermalepidermal junction (DEJ) using direct and indirect immunofluorescence. Properdin was present in 50% of normal and 40% of lesional skins. Properdin was present without C4 in only 2 of 38 nonlesional skin biopsies and in only 2 of 20 lesions. There was no significant difference in incidence of deposition of any of the six proteins studied between nonlesional and lesional skin.The frequency of deposition of each of the proteins correlated with clinical disease activity. The presence of proteins in the DEJ did not correlate with the presence of active renal disease at the time of biopsy nor with previously documented active nephritis. In addition, no other single clinical manifestation correlated with the presence of DEJ deposition of any protein studied. IgA was not demonstrated in the DEJ of nonlesional skin of 16 patients in remission and was present in 7 of 23 patients with active disease (P < 0.05). Deposition of properdin in lesional skin correlated with the presence of extracutaneous disease activity (P < 0.05).Analysis of serologic studies on serum obtained at the time of biopsy revealed a statistically significant correlation between C4 and C3 (r = 0.67). This correlation was stronger than that between properdin and C3 (r = 0.49) which in turn was stronger than that be-

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Section: Introductionsupporting

confidence: 84%

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

Schrager¹,

Rothfield²

1976

J. Clin. Invest.

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“…Decreased levels of serum properdin factor B and/or deposition of properdin in skin and kidney have been observed in certain patients with SLE (46)(47)(48)(49). The present study would suggest that under appropriate conditions involvement of the properdin system may further result in severe and even fatal disease, although clearly this represents an unusual experience.…”

Section: Discussionmentioning

confidence: 48%

Homozygous C2 deficiency with fulminant lupus erythematosus. severe nephritis via the alternative complement pathway

Gewurz

Lint

Roberts

et al. 1978

Arthritis & Rheumatism

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Absence of C2 was identified in an 18-year-old white male with progressive and rapidly fatal lupus nephritis. Genetic studies of the patient and 8 family members linked this deficiency with the HLA haplotype A10/B18, for which the patient was homozygous. High titers (1 : 1600) of anti-RNP antibodies, as well as antibodies to double-stranded DNA, were present. Serum levels of properdin factor B were persistently decreased, whereas levels of Clq and C3 intermittently were low. Biopsies of skin and kidney showed typical SLE histopathology with deposition of immunoglobulins and early-and late-acting C components; properdin was deposited in the kidney. Despite the inability of the patient's serum to mediate lysis through the classic C pathway, C-dependent lysis through the alternative pathway was readily achieved USing either unsensitized rabbit cells in gels or mixtures of guinea pig cells and zymosan as the indicator system. These studies thus reveal a new association of fatal lupus nephritis with homozygous C2 deficiency and show From the

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“…As previously reported, the diagnosis of CMPGN was based on the clinical picture, the renal pathological findings by light and immunofluorescent microscopy, and the presence of hypocomplementemia at some time during the period of observation (9). Patients ranged in age from 8 to 35 yr.…”

Section: Patientsmentioning

confidence: 98%

“…Properdin has been found deposited on glomeruli in CMPGN, AGN, some cases of SLE and in other renal disease (9). Utilizing the zymosan assay, serum levels of properdin have been noted to be decreased in some forms of renal disease (8), but the zymosan assay shows a 26% incidence of low titers in normal subjects (7), and it is probable that this assay is influenced by other proteins besides properdin.…”

Section: Properdin and C3pa In Glomerulonephritismentioning

confidence: 99%

“…At 370C in the presence of polysaccharides such as zymosan or inulin, the properdin system activates C3. A definite role of the properdin system in human disease has been suggested by the demonstration of reduced serum levels in certain renal diseases (7,8) and its presence in glomerular deposits in acute poststreptococcal glomerulonephritis (AGN)1 and chronic membranoproliferative glomerulonephritis (CMPGN) associated with hypocomplementemia (9).…”

Section: Introductionmentioning

confidence: 99%

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Properdin and C3 Proactivator: Alternate Pathway Components in Human Glomerulonephritis

McLean¹,

Michael²

1973

J. Clin. Invest.

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A B S T R A C T Serological and immunopathological studies of human glomerulonephritis have suggested that alternate pathways of activation of the third component of complement may be important in some forms of glomerulonephritis. We have investigated the role of two alternate pathway proteins, properdin and C3 proactivator, in 22 patients with chronic membranoproliferative glomerulonephritis, 21 patients with systemic lupus erythematosus, 20 patients with acute poststreptococcal glomerulonephritis, and 19 patients with other forms of renal disease. C3 (measured at PA), properdin, and C3 proactivator were assayed by single radial immunodiffusion.In sera with low PA (< 2 SD), mean properdin was most significantly decreased in patients with acute poststreptococcal glomerulonephritis but was also significantly decreased in chronic membranoproliferative glomerulonephritis and in untreated systemic lupus erythematosus. Properdin levels in other renal disease, acute glomerulonephritis, and chronic membranoproliferative glomerulonephritis with normal fiA levels were not significantly different from normal. A positive correlation between PA and properdin levels in individual sera was present in all diseases except systemic lupus erythematosus.Serum C3 proactivator was markedly decreased in active systemic lupus erythematosus and there was a positive correlation between fi1A and C3 proactivator levels in systemic lupus erythematosus and other renal diseases but not acute poststreptococcal glomerulonephritis.Properdin in fresh sera from four patients with systemic lupus erythematosus and five with chronic membranoproliferative glomerulonephritis showed increased migration toward the cathode on immunoelectrophoresis, suggesting in vivo change of the properdin molecule.

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Glomerular deposition of properdin in acute and chronic glomerulonephritis with hypocomplementemia

Cited by 110 publications

References 20 publications

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

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

Homozygous C2 deficiency with fulminant lupus erythematosus. severe nephritis via the alternative complement pathway

Properdin and C3 Proactivator: Alternate Pathway Components in Human Glomerulonephritis

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