Abstract
Background
Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) has been recognized as a distinct entity in recent years. To the best of our knowledge, all patients with PGNMID reported thus far were older than 20 years of age. We now report five cases of PGNMID in patients under 20 years of age.
Methods
The clinical database was searched for patients with native kidney biopsies from 9/2011 to 8/2017, and cases with a diagnosis of PGNMID were retrieved. Light microscopy specimens and immunofluorescence and electron microscopy images were revisited. Clinical data and kidney biopsy findings for patients under the age of 20 were recorded.
Results
Five (0.78%) of a total of 637 patients younger than 20 with native renal biopsies had a diagnosis of PGNMID, including three males and two females with an average age of 14 years old (range 10–19). All five patients presented with microscopic hematuria and proteinuria. Three patients were nephrotic and their C3 levels were low. All five cases showed a membranoproliferative pattern with abundant mesangial and subendothelial monoclonal IgG3 deposits (3 κ and 2 λ light chain, respectively). The patients were followed up to 56 months. Two patients had re-biopsies 28 and 18 months after initial diagnosis and both showed similar morphologic changes. Various treatments were attempted including prednisone, mycophenolate mofetil, tacrolimus, rituximab, and eculizmab, with mixed responses.
Conclusions
PGNMID does occur in children and young adults. Membranoproliferative glomerulonephritis pattern with monoclonal IgG3 deposits is a common feature. Despite various immunosuppressive treatments, the disease appears slowly progressive.
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References
Nasr SH, Markowitz GS, Stokes MB, Seshan SV, Valderrama E, Appel GB, Aucouturier P, D'Agati VD (2004) Proliferative glomerulonephritis with monoclonal IgG deposits: a distinct entity mimicking immune-complex glomerulonephritis. Kidney Int 65:85–96
Lin J, Markowitz GS, Valeri AM, Kambham N, Sherman WH, Appel GB, D'Agati VD (2001) Renal monoclonal immunoglobulin deposition disease: the disease spectrum. J Am Soc Nephrol 12:1482–1492
Nasr SH, Colvin R, Markowitz GS (2006) IgG1 lambda light and heavy chain renal amyloidosis. Kidney Int 70:7
Nasr SH, Markowitz GS, Reddy BS, Maesaka J, Swidler MA, D'Agati VD (2006) Dysproteinemia, proteinuria, and glomerulonephritis. Kidney Int 69:772–775
Rosenstock JL, Markowitz GS, Valeri AM, Sacchi G, Appel GB, D'Agati VD (2003) Fibrillary and immunotactoid glomerulonephritis: distinct entities with different clinical and pathologic features. Kidney Int 63:1450–1461
Nasr SH, Satoskar A, Markowitz GS, Valeri AM, Appel GB, Stokes MB, Nadasdy T, D'Agati VD (2009) Proliferative glomerulonephritis with monoclonal IgG deposits. J Am Soc Nephrol 20:2055–2064
Barbour SJ, Beaulieu MC, Zalunardo NY, Magil AB (2011) Proliferative glomerulonephritis with monoclonal IgG deposits secondary to chronic lymphocytic leukemia. Report of two cases. Nephrol Dial Transplant 26:2712–2714
Bhutani G, Nasr SH, Said SM, Sethi S, Fervenza FC, Morice WG, Kurtin PJ, Buadi FK, Dingli D, Dispenzieri A, Gertz MA, Lacy MQ, Kapoor P, Kumar S, Kyle RA, Rajkumar SV, Leung N (2015) Hematologic characteristics of proliferative glomerulonephritides with nonorganized monoclonal immunoglobulin deposits. Mayo Clin Proc 90:587–596
Dahan K, Albert C, Arlet JB, Callard P, Ronco P (2010) Non-Randall proliferative glomerulonephritis with humps and monotypic IgG deposits in primary Sjogren’s syndrome: a first case report. NDT Plus 3:558–563
Fujiwara T, Komatsuda A, Ohtani H, Togashi M, Sawada K, Wakui H (2013) Proliferative glomerulonephritis with monoclonal IgG deposits in a patient with autoimmune hemolytic anemia. Clin Nephrol 79:494–498
Hara S, Tsukaguchi H, Oka T, Kusabe M, Mizui M, Joh K (2017) Monoclonal immunoglobulin-associated proliferative glomerulonephritis characterized by organized deposits of striated ultra-substructures: a case report. Ultrastruct Pathol 41:301–307
Komatsuda A, Nara M, Ohtani H, Nimura T, Sawada K, Wakui H (2012) Proliferative glomerulonephritis with monoclonal immunoglobulin light chain deposits: a rare entity mimicking immune-complex glomerulonephritis. Intern Med 51:3273–3276
Komatsuda A, Wakui H, Ohtani H, Nimura T, Sawada K (2010) Steroid-responsive nephrotic syndrome in a patient with proliferative glomerulonephritis with monoclonal IgG deposits with pure mesangial proliferative features. NDT Plus 3:357–359
Oe Y, Joh K, Sato M, Taguma Y, Onishi Y, Nakayama K, Sato T (2013) Proliferative glomerulonephritis with monoclonal IgM-kappa deposits in chronic lymphocytic leukemia/small lymphocytic leukemia: case report and review of the literature. CEN Case Rep 2:222–227
Soares SM, Lager DJ, Leung N, Haugen EN, Fervenza FC (2006) A proliferative glomerulonephritis secondary to a monoclonal IgA. Am J Kidney Dis 47:342–349
Yahata M, Nakaya I, Takahashi S, Sakuma T, Sato H, Soma J (2012) Proliferative glomerulonephritis with monoclonal IgM deposits without Waldenstrom’s macroglobulinemia: case report and review of the literature. Clin Nephrol 77:254–260
Vignon M, Cohen C, Faguer S, Noel LH, Guilbeau C, Rabant M, Higgins S, Hummel A, Hertig A, Francois H, Lequintrec M, Vilaine E, Knebelmann B, Pourrat J, Chauveau D, Goujon JM, Javaugue V, Touchard G, El Karoui K, Bridoux F (2017) The clinicopathologic characteristics of kidney diseases related to monotypic IgA deposits. Kidney Int 91:720–728
Yamaguchi Y, Maeda K, Nagatoya K, Yamauchi A (2017) A case report of proliferative glomerulonephritis with monoclonal immunoglobulin M-kappa deposits without associated lymphoproliferative disorder or detectable paraproteinemia. CEN Case Rep. https://doi.org/10.1007/s13730-017-0291-0
Capra JD, Kunkel HG (1970) Aggregation of gamma-G3 proteins: relevance to the hyperviscosity syndrome. J Clin Invest 49:610–621
Nasr SH, Sethi S, Cornell LD, Fidler ME, Boelkins M, Fervenza FC, Cosio FG, D'Agati VD (2011) Proliferative glomerulonephritis with monoclonal IgG deposits recurs in the allograft. Clin J Am Soc Nephrol 6:122–132
Grey HM, Hirst JW, Cohn M (1971) A new mouse immunoglobulin: IgG3. J Exp Med 133:289–304
Vidarsson G, Dekkers G, Rispens T (2014) IgG subclasses and allotypes: from structure to effector functions. Front Immunol 5:520
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The study was approved by the Institutional Review Board of Baylor University Medical Center at Dallas and performed in accordance with the Declaration of Helsinki.
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Xing, G., Gillespie, R., Bedri, B. et al. Proliferative glomerulonephritis with monoclonal IgG deposits in children and young adults. Pediatr Nephrol 33, 1531–1538 (2018). https://doi.org/10.1007/s00467-018-3949-8
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DOI: https://doi.org/10.1007/s00467-018-3949-8