Article Text

PDF
Clinicopathological features and outcome of antibody-negative anti-glomerular basement membrane disease
  1. Dandan Liang1,2,
  2. Shaoshan Liang1,
  3. Feng Xu1,
  4. Mingchao Zhang1,
  5. Xiaomei Li1,
  6. Yiyao Tu1,
  7. Zhihong Liu1,2,
  8. Caihong Zeng1,2
  1. 1 National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China
  2. 2 National Clinical Research Center of Kidney Diseases, Jinling Clinical Medical College of Nanjing Medical University, Nanjing, China
  1. Correspondence to Dr Caihong Zeng, National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, Nanjing 200001, China; zengch_nj{at}hotmail.com

Abstract

Aim To explore the clinicopathological characteristics of patients with anti-GBM antibody-negative anti-GBM disease.

Methods The clinical and renal pathological findings were retrospectively studied in 19 patients. All patients met the following inclusion criteria: linear GBM IgG deposition on immunofluorescence(IF); and lack of serum anti-GBM antibodies by ELISA and indirect immunofluorescence assay.

Results There were 17 male and two female patients, with a median age of 36 years (range 15–61 years). Hypertension was present in 68% of cases, nephrotic-range proteinuria (> 3.5  g/24  hours) in 42%, nephrotic syndrome in 37%, microhaematuria in 95%, renal insufficiency in 63% and lung involvement in 16%. On biopsy all patients had linear GBM staining for polyclonal IgG by IF. The dominant IgG subtype was IgG4 or IgG1. By light microscopy, mesangial proliferative GN without crescents was seen in four patients; proliferative GN (mesangial proliferative GN in eight; endocapillary proliferative GN in two; and membranoproliferative GN in two) with crescents (focal in 11; diffuse in one) in 12 patients; and crescentic GN without mesangial or endocapillary proliferative or membranoproliferative changes in three patients. By electron microscopy, six patients showed scarce electron dense deposits in glomeruli and 11 patients had global podocyte effacement. Totally, 10 (53%) patients received immunosuppressive therapy. The median follow-up was 15 months and six (32%) patients progressed to end-stage renal disease.

Conclusions Anti-GBM antibody-negative anti-GBM disease was different from classic anti-GBM disease clinically and pathologically. The pathogenesis of the renal injury in these patients has not been elucidated until now and it should be studied and identified further.

  • anti-GBM disease
  • anti-GBM antibody
  • renal pathology

Statistics from Altmetric.com

Footnotes

  • Handling editor Dhirendra Govender.

  • Contributors CZ, ZH and DL designed the study. SL, FX, YT, XL and MZ were involved in data collection, analysis and interpretation. DL drafted the manuscript. All authors critically reviewed the manuscript and approved the final version. All authors can take responsibility for the integrity or the accuracy of any part of the work.

  • Funding This study was supported by National Natural Science Foundation of China (No. 81570644).

  • Competing interests None declared.

  • Patient consent Not required.

  • Ethics approval The Institutional Review Board of Jinling Hospital, Nanjing University School of Medicine.

  • Provenance and peer review Not commissioned; externally peer reviewed.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.