AMEI's Current Trends in Diagnosis & Treatment

Register      Login

VOLUME 6 , ISSUE 1 ( January-June, 2022 ) > List of Articles

CASE REPORT

C1q Nephropathy: A Rare Cause of Nephrotic Syndrome in Adults

Gurinder Mohan, Hargurdas Singh, Kapeesh Khanna, Sankalp Harish Jagga, Karandeep Kaur

Keywords : C1q nephropathy, Focal segmental glomerulosclerosis, Nephrotic syndrome, Proteinuria, Systemic lupus erythematosus

Citation Information : Mohan G, Singh H, Khanna K, Jagga SH, Kaur K. C1q Nephropathy: A Rare Cause of Nephrotic Syndrome in Adults. Curr Trends Diagn Treat 2022; 6 (1):15-17.

DOI: 10.5005/jp-journals-10055-0146

License: CC BY-NC 4.0

Published Online: 04-06-2022

Copyright Statement:  Copyright © 2022; The Author(s).


Abstract

C1q nephropathy first described in 1985 is a rare cause of glomerular diseases, especially in adults, but still not much is known about and lacks specific guidelines. The diagnosis is based on mesangial C1q deposition either dominant or codominant pattern in the absence of systemic lupus erythematosus (SLE). Here, we report a 28-year-old female who presented with anasarca, frank nephrotic syndrome, and hypertension. Also, antinuclear antibodies (ANA) was negative and renal biopsy revealed focal segmental glomerulosclerosis (FSGS) morphological pattern along with mesangial C1q predominant staining with primary podocytopathy and mesangial electron-dense immune deposits on electron microscopy. The diagnosis of C1q Nephropathy was made and oral steroids were started. The minimal change disease (MCD) pattern has better outcomes than FSGS. Focal segmental glomerulosclerosis might respond poorly or gets dependent on oral steroids and often requires second-line immunosuppressive therapy.


HTML PDF Share
  1. Jennette JC, Hipp CG. Clq Nephropathy: A distinct pathologic entity usually causing nephrotic syndrome. Am J Kidney Dis 1985;6(2): 103–110. DOI: 10.1016/s0272-6386(85)80150-5.
  2. Devasahayam J, Erode-Singaravelu G, Bhat Z, et al. C1q nephropathy: The unique underrecognized pathological entity. Anal Cell Pathol (Amst) 2015;2015:490413. DOI: 10.1155/2015/490413.
  3. Kanodia KV, Vanikar AV, Patel RD, et al. C1q nephropathy in India: a single-center study. Saudi J Kidney Dis Transpl 2015;26(2):398–403. DOI: 10.4103/1319-2442.152562.
  4. Malleshappa P, Vankalakunti M. Diverse clinical and histology presentation in c1q nephropathy. Nephrourol Mon 2013;5(3):787–791. DOI: 10.5812/numonthly.8308.
  5. Vizjak A, Ferluga D, Rozic M, et al. Pathology, clinical presentations, and outcomes of C1q nephropathy. J Am Soc Nephrol 2008;19(11): 2237–2244. DOI: 10.1681/ASN.2007080929.
  6. Gaur S, Patrick R, Vankalakunti M, et al. C1q nephropathy in children with nephrotic syndrome: Treatment strategies and outcomes. Indian J Nephrol 2022;32(1):54–59. DOI: 10.4103/ijn.IJN_578_20.
  7. Kim K, Son HE, Ryu JY, et al. C1q nephropathy in adults is a form of focal segmental glomerulosclerosis in terms of clinical characteristics. PLoS One 2019;14(4):e0215217. DOI: 10.1371/journal.pone.0215217.
  8. Zhao Y, Fan H, Bao BY, et al. C1q nephropathy in an old woman with acute renal failure: A case report and literature review. Ren Fail 2014;36(7):1136–1138. DOI: 10.3109/0886022X.2014.917944.
  9. Ito Y, Inoue T, Okada H. Successful treatment of C1q nephropathy by low-density lipoprotein apheresis: C1q nephropathy treated with apheresis. Ther Apher Dial 2016;20(5):530–531. DOI: 10.1111/1744-9987.12411.
  10. Ramachandran R, Bharati J, Jha V. Successful treatment of C1q nephropathy with CD19 targeted Rituximab therapy: Rituximab in C1q nephropathy. Nephrology (Carlton) 2017;22(3):265. DOI: 10.1111/nep.12757.
  11. Sinha A, Nast CC, Hristea I, et al. Resolution of clinical and pathologic features of C1q nephropathy after rituximab therapy. Clin Exp Nephrol 2011;15(1):164–170. DOI: 10.1007/s10157-010-0377-x.
  12. Wong CS, Fink CA, Baechle J, et al. C1q nephropathy and minimal change nephrotic syndrome. Pediatr Nephrol. 2009;24(4):761–767. DOI: 10.1007/s00467-008-1058-9.
PDF Share
PDF Share

© Jaypee Brothers Medical Publishers (P) LTD.