Abstract
A 62-year-old woman with nephrotic syndrome underwent a renal biopsy. Under light microscopy, the biopsy findings included lobulation and enlargement of glomeruli, occasional thickening of glomerular capillary walls, and narrowing of the capillary lumen by swollen endothelial cells. Congo red staining was negative for amyloid. No significant intraglomerular fibrin deposition was found by phosphotungstic acid hematoxylin staining. Immunofluorescence microscopy showed no deposition of immunoglobulin G, A, or M; no κ or λ light chains; and no C3 or C1q. Electron microscopy revealed distinctive subendothelial and mesangial fibrillar deposits, mesangial cell interposition, and swelling and vacuolization of endothelial cells resulting in capillary lumen narrowing. Although some curvilinear fibrillar deposits mimicked the bundles of type III collagen fibers seen in collagenofibrotic glomerulopathy, neither glomerular deposition of type III collagen nor elevation of serum procollagen III peptide was noted. This glomerulopathy does not fulfill any known disease entities with non-amyloid non-immunoglobulin-derived organized glomerular deposits.
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References
Herrera GA, Turbat-Herrera EA. Renal diseases with organized deposits: an algorithmic approach to classification and clinicopathologic diagnosis. Arch Pathol Lab Med. 2010;134:512–31.
Schwartz MM. Glomeular diseases with organized deposits. In: Jennete JC, Olson JL, Schwartz MM, Silva FG, editors. Heptinstall’s pathology of the kidney. 6th ed. Philadelphia: Lipponcott Williams & Wilkins; 2007. p. 911–36.
Burgin M, Hofmann E, Reutter FW, Gurtler BA, Matter L, Briner J, Gloor F. Familial glomerulopathy with giant fibrillar deposits. Virchows Arch A Pathol Anat Histol. 1980;388:313–26.
Strøm EH, Banfi G, Krapf R, Abt AB, Mazzucco G, Monga G, Gloor F, Neuweiler J, Riess R, Stosiek P, et al. Glomerulopathy associated with predominant fibronectin deposits: a newly recognized hereditary disease. Kidney Int. 1995;48:163–70.
Curtiss J, Heilig JS. DeLIMiting development. BioEssays. 1998;20:58–69.
Alchi B, Nishi S, Narita I, Gejyo F. Collagenofibrotic glomerulopathy: clinicopathologic overview of a rare glomerular disease. Am J Kidney Dis. 2007;49:499–506.
Tamura H, Matsuda A, Kidoguchi N, Matsumura O, Mitarai T, Isoda K. A family with two sisters with collagenofibrotic glomerulonephropathy. Am J Kidney Dis. 1996;27:588–95.
Soylemezoglu O, Wild G, Dalley AJ, MacNeil S, Milford-Ward A, Brown CB, el Nahas AM. Urinary and serum type III collagen: markers of renal fibrosis. Nephrol Dial Transplant. 1997;12:1883–9.
Yoshioka K, Takemura T, Tohda M, Akano N, Miyamoto H, Ooshima A, Maki S. Glomerular localization of type III collagen in human kidney disease. Kidney Int. 1989;35:1203–11.
Gubler MC, Dommergues JP, Foulard M, Bensman A, Leroy JP, Broyer M, Habib R. Collagen type III glomerulopathy: a new type of hereditary nephropathy. Pediatr Nephrol. 1993;7:354–60.
Khubchandani SR, Chitale AR, Gowrishankar S. Banded collagen in the kidney with special reference to collagenofibrotic glomerulopathy. Ultrastruct Pathol. 2010;34:68–72.
Cohen AH. Collagen type III glomerulopathies. Adv Chronic Kidney Dis. 2012;19:101–6.
Vogt BA, Wyatt RJ, Burke BA, Simonton SC, Kashtan CE. Inherited factor H deficiency and collagen type III glomerulopathy. Pediatr Nephrol. 1995;9:11–5.
Yoshida F, Yuzawa Y, Shigematsu H, Ito A, Yamazaki C, Yoshioka K, Ooshima A, Matsuo S. Nephrotic syndrome with massive accumulation of type I and type III collagen in the glomeruli. Intern Med. 1993;32:171–6.
Imbasciati E, Gherardi G, Morozumi K, Gudat F, Epper R, Basler V, Mihatsch MJ. Collagen type III glomerulopathy: a new idiopathic glomerular disease. Am J Nephrol. 1991;11:422–9.
Morita H, Hasegawa T, Minamoto T, Oda Y, Inui K, Tayama H, Nakao N, Nakamoto Y, Ideura T, Yoshimura A. Collagenofibrotic glomerulopathy with a widespread expression of type-V collagen. Virchows Arch. 2003;442:163–8.
Ohtani H, Wakui H, Komatsuda A, Goto H, Tada M, Ozawa M, Kobayashi R, Sawada K. Progressive glomerulopathy with unusual deposits of striated structures: a new disease entity? Nephrol Dial Transplant. 2010;25:2016–9.
Nasr SH, D’Agati VD. Nodular glomerulosclerosis in the nondiabetic smoker. J Am Soc Nephrol. 2007;18:2032–6.
Salvatore SP, Troxell ML, Hecox D, Sperling KR, Seshan SV. Smoking-related glomerulopathy: expanding the morphologic spectrum. Am J Nephrol. 2015;41:66–72.
Acknowledgments
The authors thank Dr. Akira Michibe and Dr. Sayaka Ishigaki for their clinical works, Mr. Masanori Handa for providing technical assistance with immunohistochemistry and Mr. Isao Ohta for providing technical assistance with electron microscopy.
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Yamamoto, T., Togawa, A., Eguchi, M. et al. Glomerulopathy with distinctive fibrillar deposits but lacking glomerular deposition of type III collagen. CEN Case Rep 5, 163–167 (2016). https://doi.org/10.1007/s13730-016-0217-2
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DOI: https://doi.org/10.1007/s13730-016-0217-2