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.
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)
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)
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)
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)
Chazen, E.M., Cook, C.D., Cohen, J.: Focal scleroderma. Report of 19 cases in children. J. Pediat. 60, 385–393 (1962)
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)
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)
Fahr, Th.: über Nephrosklerose. Virch. Arch. 226, 119–178 (1919)
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)
Fisher, E.R., Rodnan, G.P.: Pathologic observations concerning the kidney in progressive systemic sclerosis. Arch. Path. 65, 29–39 (1958)
Freedman, P., Peters, J.H., Kark, R.M.: Localization of gamma-globulin in the diseased kidney. Arch. Intern. Med. 105, 524–535 (1960)
Gerber, M.A.: Immunohistochemical findings in the renal vascular lesions of progressive systemic sclerosis. Human Pathol. 6, 343–347 (1975)
Hanson, V., Drexler, E., Kornreich, H.: Rheumatoid factor in children with focal scleroderma. Pediatrics 53, 945–947 (1974)
Heptinstall, R.H.: Scleroderma. In: Pathology of the kidney. pp. 696–711. Boston: Little, Brown and Company, 1974
Jablonska, St., Chorzelski, T.: Immunologische Untersuchungen bei Sklerodermie. Allergie u. Immunologie 18, 187–190 (1972)
Jaffe, M.O., Winkelmann, R.K.: Generalized scleroderma in children. Arch. Derm. 83, 402–413 (1961)
Kass, H., Hanson, V., Patrick, J.: Scleroderma in childhood. J. Pediat. 68, 243–256 (1966)
Kennedy, W.P.: Cardiac death from progressive systemic scleroderma in a child. Canad. Med. Assoc. J. 90, 33–35 (1964)
Kincaid-Smith, P.: Participation of intravascular coagulation in the pathogenesis of glomerular and vascular lesions. Kidney Int. 7, 242–253 (1975)
Kincaid-Smith, P.: Scleroderma. In: The kidney. A clinico-pathological study. pp. 302–308. Oxford: Blackwell Scientific Publications 1975
McCoy, R.C.: Scleroderma kidney: Light, immunofluorescence and electron microscopy. Lab. Invest. 32, 430 (1975)
McGiven, A.R., deBoer, W.G.R.M., Barnett, A.J.: Renal immune deposits in scleroderma. Pathology 3, 145–150 (1971)
Medsger, T.A., Masi, A.T.: Epidemiology of systemic sclerosis (scleroderma). Ann. Int. Med. 74, 714–721 (1971)
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)
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)
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)
Mukherjee, S.K., Lahiri, K., Sen, M.K.: Scleroderma in a boy of 9. J. Indian Med. Assoc. 47, 132–135 (1966)
Oliver, J.A., Cannon, P.J.: The kidney in scleroderma. Nephron 18, 141–150 (1977)
Richardson, J.A.: Hemodialysis and kidney transplantation for renal failure from scleroderma. Arthritis Rheum. 16, 265–271 (1973)
Seelig, H.P.: Immunhistologische Methoden. In: Praxis der Immunologie. (Vorlaender K.O., Ed.), p. 131. Stuttgart: Thieme 1976
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)
Velayos, E.E., Cohen, B.S.: Progressive systemic sclerosis. Amer. J. Dis. Child. 123, 57–60 (1972)
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)
Winkelmann, R.K.: Classification and pathogenesis of scleroderma. Mayo Clin. Proc. 46, 83–91 (1971)
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)
Stögmann, W., Sandhofer, M., Fritz, J.: Immunological studies in childhood scleroderma. Europ. J. Pediat. 124, 223–230 (1977)
Tuffanelli, D.L., Winkelmann, R.K.: Systemic scleroderma: A clinical study of 727 cases. Arch. Derm. 84, 359–371 (1961)
Author information
Authors and Affiliations
Rights 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
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00432486