The complement system in chronic glomerulonephritis: Three newly associated aberrations

Pickering, R. J.; Michael, Alfred F.; Herdman, Roger; Good, Robert A.; Gewürz, Henry

doi:10.1016/s0022-3476(71)80261-5

Cited by 104 publications

(24 citation statements)

References 29 publications

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“…Clq (1-3), Clr (4-6), Cls (7), C2, and C3 deficient patients have been studied in our laboratories and also by other investigators (8)(9)(10)(11)(12)(13)(14)(15)(16)(17). In most of these patients increased susceptibility to infections, vascular lesions, renal disease, and lupus-like syndromes have been observed.…”

Section: Introductionmentioning

confidence: 79%

C2 Deficiency DEVELOPMENT OF LUPUS ERYTHEMATOSUS

Day¹,

Geiger²,

McLean³

et al. 1973

J. Clin. Invest.

104

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A B S T R A C T The study of serum from a patient with C2 deficiency is described. The patient had an episode of pneumococcal meningitis at 5 mo of age with seizures and transient hemiparesis and apparent purpuric skin lesions. He was first admitted to the University of Minnesota Hospitals at 10 yr of age following the discovery of proteinuria accidentally by his mother. Since then he has been admitted repeatedly to this hospital with numerous clinical findings including arthralgia, recurrent abdominal pain, proteinuria, membranous nephropathy, malar butterfly rash, seizures, personality aberrations, and recurrent fever. In June 1971, the patient developed positive DNA and DNP antibodies and positive LE cells. When the C profile was studied before and after recognition of lupus, Clq, Cls, and C4 dropped. C3 levels were elevated as were C5, C6, and C7. C3 proactivator had been reduced in the patient even before he developed lupus. Also because of a traumatic renal biopsy leading to a perirenal hematoma, he required surgery and a blood transfusion. 1 h after blood transfusion, a C2 titer of 23 hemolytic units was detected. Almost immediately levels of C3, C5, C6, and C7 dropped. C8 and C9 remained elevated. The addition of C2 from normal blood permitted dramatic activation of C3.These findings support the view that the rare deficiency in production of C2 predisposes to serious susceptibility to infection, vascular and mesenchymal disease as well as to renal disease and a lupus syndrome.

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Section: Introductionmentioning

confidence: 79%

C2 Deficiency DEVELOPMENT OF LUPUS ERYTHEMATOSUS

Day¹,

Geiger²,

McLean³

et al. 1973

J. Clin. Invest.

104

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“…Isolated deficiencies of C l q (19), C l r (14,20,21), and C8 (28) have been reported in a total of 23 patients with connective tissue diseases. In addition to these, 2 siblings with hereditary angioedema 0 inhibitor deficiency) have been reported with discoid lupus erythematosus; the mother of these children had died of systemic lupus erythematosus and had also manifested angioedema (29).…”

Section: Discussionmentioning

confidence: 99%

“…lonephritis (21). Furthermore, it has been shown that persons with partial (heterozygous) deficiency of C2 occur in groups of SLE patients in significantly higher numbers than would be expected if the association was a random one (30).…”

Section: Discussionmentioning

confidence: 99%

Familial deficiency of two subunits of the first component of complement. c1r and c1s associated with a lupus erythematosus‐like disease

Lee

Wallace

Barone

et al. 1978

Arthritis & Rheumatism

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Complete absence of Clr and almost complete absence of Cls were found in 4 of 8 living siblings. Two of the 4 suffer from a syndrome that combines discoid lupus erythematosus and nondeforming rheumatoid-like arthritis; one of the siblings has mild nephritis. The other 2 C1 deficient family members are clinically well. Evidence from this and other families suggests that deficiency of CI components or C4 is associated with higher risk of developing a lupus-like disease than is deficiency of C2. Recent reports have emphasized the occurrence

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“…Like the hereditary deficiencies of early proteins in the complement system, mutation in C1-INH inhibitor gene is sometimes accompanied by dysimmunity, resulting in an increase in the prevalence of autoimmune diseases and deposits of immune complexes [4,[12][13][14]. Indeed, the NIH team found 12% of dysimmune manifestations in a series of 157 patients with HAE, including 3 cases of membranoproliferative glomerulonephritis associated with circulating immune complexes [4].…”

Section: Discussionmentioning

confidence: 99%

Hereditary Angioedema Type 1 and Serious Vascular Lesions Associated with Segmental and Focal Hyalinosis: Is there a Causal Connection?

Guéye¹,

Yaya²,

Dahri³

et al. 2017

J Clin Exp Nephrol

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Hereditary angioedema (HAE) is rare; it is usually a monogenic, genetic disease with autosomal dominant transmission, resulting in C1-inhibitor deficiency (C1InH). Renal involvement associated with HAE has been known for nearly thirty years. We are reporting on two observations of a father and his daughter presenting a glomerulonephritis evolving towards to CKD class V. Renal needle biopsy had evidenced acute vascular lesions associated with segmental and focal hyalinosis. Since these lesions were not described, we discussed the possible causal links with HAE. The accumulation of bradykinin caused by mutation in C1-Inh could have been misleading, considering all the beneficial vascular and renal effects widely described in the literature, if we stick to that fact, in the study of vascular and glomerular lesions. On the other hand, taken as a whole, HAE appears to pave the way for the development of a glomerular and vascular disease: dysimmunity, circulating immune complexes, and vascular hyperpermeability favor deposition of immune complexes, overexpression and activation of B1 receptors.

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The complement system in chronic glomerulonephritis: Three newly associated aberrations

Cited by 104 publications

References 29 publications

C2 Deficiency DEVELOPMENT OF LUPUS ERYTHEMATOSUS

C2 Deficiency DEVELOPMENT OF LUPUS ERYTHEMATOSUS

Familial deficiency of two subunits of the first component of complement. c1r and c1s associated with a lupus erythematosus‐like disease

Hereditary Angioedema Type 1 and Serious Vascular Lesions Associated with Segmental and Focal Hyalinosis: Is there a Causal Connection?

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