RT Journal Article
SR Electronic
T1 Clinicopathological features and outcome of antibody-negative anti-glomerular basement membrane disease
JF Journal of Clinical Pathology
JO J Clin Pathol
FD BMJ Publishing Group Ltd and Association of Clinical Pathologists
SP 31
OP 37
DO 10.1136/jclinpath-2018-205278
VO 72
IS 1
A1 Dandan Liang
A1 Shaoshan Liang
A1 Feng Xu
A1 Mingchao Zhang
A1 Xiaomei Li
A1 Yiyao Tu
A1 Zhihong Liu
A1 Caihong Zeng
YR 2019
UL http://jcp.bmj.com/content/72/1/31.abstract
AB 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.