Skip to main content
Log in

Membranoproliferative glomerulonephritis in systemic sclerosis of childhood

  • Case Report
  • Published:
Virchows Archiv A Aims and scope Submit manuscript

Summary

The renal lesions of a 5-year-old girl with progressive systemic sclerosis are described. The nephropathy was clinically characterised by moderate proteinuria, microscopic hematuria and transient hypertension. Light microscopy showed membranoproliferative glomerulonephritis of segmental character. On electron microscopy intramesangial, subendothelial and extramembranous glomerular deposits were observed. By immunofluorescence microscopy deposits of IgG, Clq, C4, C3, C5, C8 und C9 in a predominantly subendothelial location were found in all glomeruli. Vascular lesions were of minor degree. Histological and immunohistological findings are compatible with an immune complex disease.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  • Ansell, B.M., Nasseh, G.A., Bywaters, E.G.L.: Scleroderma in childhood. Ann. Rheum. Dis. 35, 189–197 (1976)

    Google Scholar 

  • Barnett, A.J., Coventry, D.A.: Scleroderma. I. Clinical features, course of illness and response to treatment in 61 cases. Med. J. Aust. 1, 992–1001 (1969)

    Google Scholar 

  • Beetham, W.P., Osment, L.S., Wisner, W.H.: Progressive systemic scleroderma in a child: A case report. Southern Med. J. 60, 236–240 (1967)

    Google Scholar 

  • Cannon, P.J., Hassar, M., Case, D.B., Casarella, W.J., Sommers, S.C., LeRoy, E.C.: The relationship of hypertension and renal failure in scleroderma (progressive systemic sclerosis) to structural and functional abnormalities of the renal cortical circulation. Medicine 53, 1–46 (1974)

    Google Scholar 

  • Chazen, E.M., Cook, C.D., Cohen, J.: Focal scleroderma. Report of 19 cases in children. J. Pediat. 60, 385–393 (1962)

    Google Scholar 

  • D'Angelo, W.A., Fries, J.F., Masi, A.T., Shulman, L.E.: Pathologic observations in systemic sclerosis (scleroderma). Amer. J. Med. 46, 428–440 (1969)

    Google Scholar 

  • Ehrenfeld, M., Licht,, A., Stessmann, J., Yanko, L., Rosenmann, E.: Post partum renal failure due to progressive systemic sclerosis trated with chronic hemodialysis. Nephron 18, 175–181 (1977)

    Google Scholar 

  • Fahr, Th.: über Nephrosklerose. Virch. Arch. 226, 119–178 (1919)

    Google Scholar 

  • Fennel, R.H., jr., Reddy, C.R.R.M., Vasquez, J.H.: Progressive systemic sclerosis and malignant hypertension. Immunohistochemical study of renal lesions. Arch. Path. 72, 209–215 (1961)

    Google Scholar 

  • Fisher, E.R., Rodnan, G.P.: Pathologic observations concerning the kidney in progressive systemic sclerosis. Arch. Path. 65, 29–39 (1958)

    Google Scholar 

  • Freedman, P., Peters, J.H., Kark, R.M.: Localization of gamma-globulin in the diseased kidney. Arch. Intern. Med. 105, 524–535 (1960)

    Google Scholar 

  • Gerber, M.A.: Immunohistochemical findings in the renal vascular lesions of progressive systemic sclerosis. Human Pathol. 6, 343–347 (1975)

    Google Scholar 

  • Hanson, V., Drexler, E., Kornreich, H.: Rheumatoid factor in children with focal scleroderma. Pediatrics 53, 945–947 (1974)

    Google Scholar 

  • Heptinstall, R.H.: Scleroderma. In: Pathology of the kidney. pp. 696–711. Boston: Little, Brown and Company, 1974

    Google Scholar 

  • Jablonska, St., Chorzelski, T.: Immunologische Untersuchungen bei Sklerodermie. Allergie u. Immunologie 18, 187–190 (1972)

    Google Scholar 

  • Jaffe, M.O., Winkelmann, R.K.: Generalized scleroderma in children. Arch. Derm. 83, 402–413 (1961)

    Google Scholar 

  • Kass, H., Hanson, V., Patrick, J.: Scleroderma in childhood. J. Pediat. 68, 243–256 (1966)

    Google Scholar 

  • Kennedy, W.P.: Cardiac death from progressive systemic scleroderma in a child. Canad. Med. Assoc. J. 90, 33–35 (1964)

    Google Scholar 

  • Kincaid-Smith, P.: Participation of intravascular coagulation in the pathogenesis of glomerular and vascular lesions. Kidney Int. 7, 242–253 (1975)

    Google Scholar 

  • Kincaid-Smith, P.: Scleroderma. In: The kidney. A clinico-pathological study. pp. 302–308. Oxford: Blackwell Scientific Publications 1975

    Google Scholar 

  • McCoy, R.C.: Scleroderma kidney: Light, immunofluorescence and electron microscopy. Lab. Invest. 32, 430 (1975)

    Google Scholar 

  • McGiven, A.R., deBoer, W.G.R.M., Barnett, A.J.: Renal immune deposits in scleroderma. Pathology 3, 145–150 (1971)

    Google Scholar 

  • Medsger, T.A., Masi, A.T.: Epidemiology of systemic sclerosis (scleroderma). Ann. Int. Med. 74, 714–721 (1971)

    Google Scholar 

  • Medsger, T.A., Masi, A.T.: Survival with scleroderma. II: A life-table analysis of clinical and dermographic factors in 358 male U.S. veteran patients. J. chron. Dis. 26, 647–660 (1973)

    Google Scholar 

  • Meffert, H., Barthelmes, H., Klug, H., Thormann, Th., Schwarz, R., Sönnichsen, N.: Beziehungen zwischen morphologischen, immunologischen und klinischen Befunden bei progressiver Sklerodermie, dargestellt an der Muskulatur. Derm. Mschr. 161, 556–566 (1975)

    Google Scholar 

  • Merino, G.E., Sutherland, D.E.R., Kjellstrand, C.M., Simmons, R.L., Najarian, J.S.: Renal transplantation for progressive systemic sclerosis with renal failure. Case report and review of previous experience. Amer. J. Surg. 133, 745–749 (1977)

    Google Scholar 

  • Mukherjee, S.K., Lahiri, K., Sen, M.K.: Scleroderma in a boy of 9. J. Indian Med. Assoc. 47, 132–135 (1966)

    Google Scholar 

  • Oliver, J.A., Cannon, P.J.: The kidney in scleroderma. Nephron 18, 141–150 (1977)

    Google Scholar 

  • Richardson, J.A.: Hemodialysis and kidney transplantation for renal failure from scleroderma. Arthritis Rheum. 16, 265–271 (1973)

    Google Scholar 

  • Seelig, H.P.: Immunhistologische Methoden. In: Praxis der Immunologie. (Vorlaender K.O., Ed.), p. 131. Stuttgart: Thieme 1976

    Google Scholar 

  • Sönnichsen, N., Friedrich, C., Barthelmes, H., Meffert, H., Schulze, P.: Ergebnisse immunologischer Untersuchungen bei der Sklerodermie und ihre Bedeutung für Diagnostik und Therapie. Dt. Gesundh.-Wesen 32, 839–843 (1977)

    Google Scholar 

  • Velayos, E.E., Cohen, B.S.: Progressive systemic sclerosis. Amer. J. Dis. Child. 123, 57–60 (1972)

    Google Scholar 

  • Vidt, D.G., Robertson, A.L., Deodhar, S.D.: Renal changes in progressive systemic sclerosis. Report of five cases. Cleve. Clin. Q. 38, 141–152 (1971)

    Google Scholar 

  • Winkelmann, R.K.: Classification and pathogenesis of scleroderma. Mayo Clin. Proc. 46, 83–91 (1971)

    Google Scholar 

  • Woodhall, P.B., McCoy, Gunnels, J.C., Seigler, H.F.: Apparent recurrence of progressive systemic sclerosis in a renal allograft. JAMA 236, 1032–1034 (1976)

    Google Scholar 

  • Stögmann, W., Sandhofer, M., Fritz, J.: Immunological studies in childhood scleroderma. Europ. J. Pediat. 124, 223–230 (1977)

    Google Scholar 

  • Tuffanelli, D.L., Winkelmann, R.K.: Systemic scleroderma: A clinical study of 727 cases. Arch. Derm. 84, 359–371 (1961)

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Waldherr, R., Seelig, H.P., Klare, B. et al. Membranoproliferative glomerulonephritis in systemic sclerosis of childhood. Virchows Arch. A Path. Anat. and Histol. 379, 169–179 (1978). https://doi.org/10.1007/BF00432486

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00432486

Key words

Navigation