Get 20M+ Full-Text Papers For Less Than $1.50/day. Start a 14-Day Trial for You or Your Team.

Learn More →

Hypocomplementemic (Membranoproliferative) Glomerulonephritis: Immunosuppressive Therapy

Hypocomplementemic (Membranoproliferative) Glomerulonephritis: Immunosuppressive Therapy Abstract Persistent glomerulonephritis with low serum hemolytic complement activity and serum β1C globulin level has been reported in children and young adults. The disease, characterized by intermittent episodes of gross hematuria and nephrotic syndrome, often progresses to severe renal failure. Depressed serum β1C globulin and deposits of β1C on the basement membrane indicate a possible immune etiology and favorable response to immunosuppressant drugs might be expected. Seven patients with persistent hypocomplementemic glomerulonephritis were treated with azathioprine and prednisone for periods up to 4½ years. Two patients are asymptomatic and have normal renal function; another has normal creatinine clearance. The other four patients progressed to renal failure; two of these have died despite vigorous therapy. Limited response to immunosuppressants and lack of correlation of β1C globulin levels with clinical status suggests a possible nonimmune origin of this disease. References 1. Holland NH, Bennet NM: Immunosuppressive therapy in hypocomplementemic glomerulonephritis. Read before the American Society of Nephrology, Washington, DC, 1969. 2. West CD, Holland NH, McConville JM, et al: Immunosuppressive therapy in persistent hypocomplementemic glomerulonephritis and in lupus nephritis . J Pediat 67:1113-1121, 1965.Crossref 3. Michael AF, Vernier RL, Drummond KN, et al: Immunosuppressive therapy of chronic renal disease . New Eng J Med 276:817-828, 1967.Crossref 4. White RHR, Cameron JS, Trounce JR: Immunosuppressive therapy in steroid-resistant proliferative glomerulonephritis accompanied by the nephrotic syndrome . Brit Med J 2:853-860, 1966.Crossref 5. Adams D, Gordon A, Maxwell MH: Azathioprine therapy of immunologic renal disease . JAMA 100:459-463, 1967.Crossref 6. Grupe WE, Heymann W: Cytotoxic drugs in steroid-resistant renal disease . Amer J Dis Child 112:448-458, 1966.Crossref 7. Shearn MA: Mercaptopurine in the treatment of steroid-resistant nephrotic syndrome . New Eng J Med 273:944-947, 1965.Crossref 8. Halpern PB, Milliez P, Lagrue G, et al: Chimiotherapie, immunosuppressive des nephropathies experimentales et humaines . Rev Ther 22:404-410, 1965. 9. Krueger RP, Gunnels JC: Azathioprine therapy of renal disease. Read before the American Society of Nephrology, Washington, DC, 1968. 10. Urizar RE, Tinglof B, McIntosh R, et al: Immunosuppressive therapy of proliferative glomerulonephritis in children . Amer J Dis Child 118:411-425, 1969. 11. McCluskey RT, Vassalli P, Gallo G, et al: An immunofluorescent study of pathogenic mechanisms in glomerular diseases . New Eng J Med 274:695-701, 1966.Crossref 12. Dixon FJ: The pathogenesis of glomerulonephritis . Amer J Med 44:493-498, 1968.Crossref 13. Unanue E, Dixon FJ: Experimental glomerulonephritis: IV. Participation of complement in nephrotoxic nephritis . J Exp Med 119:965-982, 1964.Crossref 14. Hammer DK, Dixon FJ: Experimental glomerulonephritis: II. Immunologic events in the pathogenesis of nephrotoxic serum nephritis in the rat . J Exp Med 117:1019-1034, 1963.Crossref 15. West CD, Northway JD, Davis ND: Serum levels of beta, C globulin, a complement component in the nephritides lipoid nephrosis and other conditions . J Clin Invest 43:1507-1517, 1964.Crossref 16. Gotoff SP, Fellers FX, Vawter GF, et al: The beta, C globulin in childhood nephrotic syndrome, laboratory diagnosis of progressive glomerulonephritis . New Eng J Med 273:524-529, 1965.Crossref 17. West CD, McAdams JA, McConville JM, et al: Hypocomplementemic and normocomplementemic persistent chronic glomerulonephritis; clinical and pathologic characteristics . J Pediat 67:1090-1112, 1965. 18. Michael AF, Drummond KN, Good RA, et al: Acute poststreptococcal glomerulonephritis: Immune deposit disease , J Clin Invest 45:237-248, 1966.Crossref 19. Lachman PJ, Muller-Eberhard HK, Kunkel HG, et al: The localization of in vivo bound complement in tissue sections , J Exp Med 115:63-82, 1962.Crossref 20. Paronetto F, Koffler D: Immunofluorescent localization of immunoglobulins, complement, and fibrinogen in human diseases: I. Systemic lupus erythematosus , J Clin Invest 44:1657-1663, 1965.Crossref 21. Koffler D, Paronetto F: Immunofluorescent localization of immunoglobulins, complement, and fibrinogen in human disease: II. Acute, subacute, and chronic glomerulonephritis . J Clin Invest 44:1665-1671, 1965.Crossref 22. West CD: Serum complement and chronic glomerulonephritis , Hosp Pract 5:75-87, 1970. 23. Stiehm ER, Trygstad CW: Split products of fibrin in human renal disease , Amer J Med 46:774-786, 1969.Crossref 24. West CD, McAdams AJ: Serum beta, C globulin levels in persistent glomerulonephritis with low serum complement; variability unrelated in clinical course . Nephron 7:193-202, 1970.Crossref 25. Ogg CS, Cameron JS, White RHR: The C'3 component of complement (β1C globulin) in patients with heavy proteinuria . Lancet 11:78-81, 1968.Crossref 26. Alper CA, Levin AS, Rosen FS: Beta, C globulin: Metabolism in glomerulonephritis . Science 153:180-182, 1966.Crossref 27. West CD, Winter S, Forristal J, et al: Evidence for in vivo breakdown of beta, C globulin in hypocomplementemic glomerulonephritis . J Clin Invest 46:539-548, 1967.Crossref 28. Spitzer RE, Vallota EH, Forristal J, et al: Serum C'3 lytic system in patients with glomerulonephritis . Science 164;436-437, 1969.Crossref 29. Mueller-Eberhardt JH, Dalmasso AP, Olcott MA: The reaction mechanism of beta, C globulin (C'3) in immune hemolysis . J Exp Med 123:33-53, 1966.Crossref 30. Northway JD, McAdams AJ, Forristal J, et al: A "silent" phase of hypocomplementemic persistent nephritis detectable by reduced serum beta, C globulin levels . J Pediat 74:28-38, 1969.Crossref 31. Levitt JI: Deterioration of renal function after discontinuation of long-term prednisone-azathioprine therapy in primary renal disease . New Eng J Med 282:1125-1127, 1970.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Hypocomplementemic (Membranoproliferative) Glomerulonephritis: Immunosuppressive Therapy

Loading next page...
 
/lp/american-medical-association/hypocomplementemic-membranoproliferative-glomerulonephritis-P13ARnCSbR
Publisher
American Medical Association
Copyright
Copyright © 1972 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1972.02110110067001
Publisher site
See Article on Publisher Site

Abstract

Abstract Persistent glomerulonephritis with low serum hemolytic complement activity and serum β1C globulin level has been reported in children and young adults. The disease, characterized by intermittent episodes of gross hematuria and nephrotic syndrome, often progresses to severe renal failure. Depressed serum β1C globulin and deposits of β1C on the basement membrane indicate a possible immune etiology and favorable response to immunosuppressant drugs might be expected. Seven patients with persistent hypocomplementemic glomerulonephritis were treated with azathioprine and prednisone for periods up to 4½ years. Two patients are asymptomatic and have normal renal function; another has normal creatinine clearance. The other four patients progressed to renal failure; two of these have died despite vigorous therapy. Limited response to immunosuppressants and lack of correlation of β1C globulin levels with clinical status suggests a possible nonimmune origin of this disease. References 1. Holland NH, Bennet NM: Immunosuppressive therapy in hypocomplementemic glomerulonephritis. Read before the American Society of Nephrology, Washington, DC, 1969. 2. West CD, Holland NH, McConville JM, et al: Immunosuppressive therapy in persistent hypocomplementemic glomerulonephritis and in lupus nephritis . J Pediat 67:1113-1121, 1965.Crossref 3. Michael AF, Vernier RL, Drummond KN, et al: Immunosuppressive therapy of chronic renal disease . New Eng J Med 276:817-828, 1967.Crossref 4. White RHR, Cameron JS, Trounce JR: Immunosuppressive therapy in steroid-resistant proliferative glomerulonephritis accompanied by the nephrotic syndrome . Brit Med J 2:853-860, 1966.Crossref 5. Adams D, Gordon A, Maxwell MH: Azathioprine therapy of immunologic renal disease . JAMA 100:459-463, 1967.Crossref 6. Grupe WE, Heymann W: Cytotoxic drugs in steroid-resistant renal disease . Amer J Dis Child 112:448-458, 1966.Crossref 7. Shearn MA: Mercaptopurine in the treatment of steroid-resistant nephrotic syndrome . New Eng J Med 273:944-947, 1965.Crossref 8. Halpern PB, Milliez P, Lagrue G, et al: Chimiotherapie, immunosuppressive des nephropathies experimentales et humaines . Rev Ther 22:404-410, 1965. 9. Krueger RP, Gunnels JC: Azathioprine therapy of renal disease. Read before the American Society of Nephrology, Washington, DC, 1968. 10. Urizar RE, Tinglof B, McIntosh R, et al: Immunosuppressive therapy of proliferative glomerulonephritis in children . Amer J Dis Child 118:411-425, 1969. 11. McCluskey RT, Vassalli P, Gallo G, et al: An immunofluorescent study of pathogenic mechanisms in glomerular diseases . New Eng J Med 274:695-701, 1966.Crossref 12. Dixon FJ: The pathogenesis of glomerulonephritis . Amer J Med 44:493-498, 1968.Crossref 13. Unanue E, Dixon FJ: Experimental glomerulonephritis: IV. Participation of complement in nephrotoxic nephritis . J Exp Med 119:965-982, 1964.Crossref 14. Hammer DK, Dixon FJ: Experimental glomerulonephritis: II. Immunologic events in the pathogenesis of nephrotoxic serum nephritis in the rat . J Exp Med 117:1019-1034, 1963.Crossref 15. West CD, Northway JD, Davis ND: Serum levels of beta, C globulin, a complement component in the nephritides lipoid nephrosis and other conditions . J Clin Invest 43:1507-1517, 1964.Crossref 16. Gotoff SP, Fellers FX, Vawter GF, et al: The beta, C globulin in childhood nephrotic syndrome, laboratory diagnosis of progressive glomerulonephritis . New Eng J Med 273:524-529, 1965.Crossref 17. West CD, McAdams JA, McConville JM, et al: Hypocomplementemic and normocomplementemic persistent chronic glomerulonephritis; clinical and pathologic characteristics . J Pediat 67:1090-1112, 1965. 18. Michael AF, Drummond KN, Good RA, et al: Acute poststreptococcal glomerulonephritis: Immune deposit disease , J Clin Invest 45:237-248, 1966.Crossref 19. Lachman PJ, Muller-Eberhard HK, Kunkel HG, et al: The localization of in vivo bound complement in tissue sections , J Exp Med 115:63-82, 1962.Crossref 20. Paronetto F, Koffler D: Immunofluorescent localization of immunoglobulins, complement, and fibrinogen in human diseases: I. Systemic lupus erythematosus , J Clin Invest 44:1657-1663, 1965.Crossref 21. Koffler D, Paronetto F: Immunofluorescent localization of immunoglobulins, complement, and fibrinogen in human disease: II. Acute, subacute, and chronic glomerulonephritis . J Clin Invest 44:1665-1671, 1965.Crossref 22. West CD: Serum complement and chronic glomerulonephritis , Hosp Pract 5:75-87, 1970. 23. Stiehm ER, Trygstad CW: Split products of fibrin in human renal disease , Amer J Med 46:774-786, 1969.Crossref 24. West CD, McAdams AJ: Serum beta, C globulin levels in persistent glomerulonephritis with low serum complement; variability unrelated in clinical course . Nephron 7:193-202, 1970.Crossref 25. Ogg CS, Cameron JS, White RHR: The C'3 component of complement (β1C globulin) in patients with heavy proteinuria . Lancet 11:78-81, 1968.Crossref 26. Alper CA, Levin AS, Rosen FS: Beta, C globulin: Metabolism in glomerulonephritis . Science 153:180-182, 1966.Crossref 27. West CD, Winter S, Forristal J, et al: Evidence for in vivo breakdown of beta, C globulin in hypocomplementemic glomerulonephritis . J Clin Invest 46:539-548, 1967.Crossref 28. Spitzer RE, Vallota EH, Forristal J, et al: Serum C'3 lytic system in patients with glomerulonephritis . Science 164;436-437, 1969.Crossref 29. Mueller-Eberhardt JH, Dalmasso AP, Olcott MA: The reaction mechanism of beta, C globulin (C'3) in immune hemolysis . J Exp Med 123:33-53, 1966.Crossref 30. Northway JD, McAdams AJ, Forristal J, et al: A "silent" phase of hypocomplementemic persistent nephritis detectable by reduced serum beta, C globulin levels . J Pediat 74:28-38, 1969.Crossref 31. Levitt JI: Deterioration of renal function after discontinuation of long-term prednisone-azathioprine therapy in primary renal disease . New Eng J Med 282:1125-1127, 1970.Crossref

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: May 1, 1972

References