ANCA-associated crescentic glomerulonephritis with mesangial IgA deposits

Mark Haas, Jabeen Jafri, Sharon M. Bartosh, Sharon Karp, Sharon G. Adler, Shane M. Meehan

Research output: Contribution to journalArticle

81 Citations (Scopus)

Abstract

Antineutrophil cytoplasmic autoantibodies (ANCA) are commonly associated with a necrotizing and crescentic glomerulonephritis (GN) that is pauci-immune, with few or no glomerular immune complex deposits detectable by immunofluorescence (IF) or electron microscopy (EM). Immunoglobulin A (IgA) nephropathy may also be manifest as e crescentic GN, but it is characterized by mesangial immune complex deposits containing IgA and is rarely associated with myeioperoxidase (MPO)- or proteinase 3 (PR3)-specific ANCA when an enzyme immunoassay is used to detect these antibodies. This report describes six patients with severe crescentic GN with mesangial IgA deposits by IF and mesangial electron-dense deposits by EM in patients with positive ANCA serological test results (four patients, anti-PR3; one patient, anti-MPO; one patient, anti-PR3 and anti-MPO). Patients presented with acute or progressive renal insufficiency, hematuria, proteinuria (nephrotic range in two patients), and hypertension. Three patients had evidence of systemic vascuiitis: two patients at initial presentation and one patient later in the clinical course. Renal biopsy specimens showed crescents in greater than 50% of glomeruli in all cases, but only mild, focal and segmental mesanglal and endocapillary hypercellularity, more typical of ANCA-associated crescentic GN than of crescentic IgA nephropathy without associated ANCA. Semiquantitative analysis of mesangial and endocapillary cellularity performed on renal biopsy slides from these six patients and from eight ANCA-negative patients with IgA nephropathy and crescents in greater than 50% of glomeruli showed significantly greater hypercellularity in the ANCA-negative cases. Three of five ANCA-positive patients for whom follow-up clinical data were available showed improved renal function after treatment with cyclophosphamide and corticosteroids and have not developed end-stage renal disease 17, 20, and 25 months postbiopsy. The remaining two patients were dialysis dependent at the time of biopsy and have remained so despite treatment with cyclophosphamide and corticosteroids. The findings suggest an overlap syndrome of ANCA-associated crescentic GN and IgA nephropathy that resembles the former both histologically and in its potential to respond to aggressive therapy if detected relatively early in its course. (C) 2000 by the National Kidney Foundation, Inc.

Original languageEnglish
Pages (from-to)709-718
Number of pages10
JournalAmerican Journal of Kidney Diseases
Volume36
Issue number4
StatePublished - 2000

Fingerprint

Glomerulonephritis
Autoantibodies
Immunoglobulin A
IGA Glomerulonephritis
Myeloblastin
Antigen-Antibody Complex
Kidney
Biopsy
Cyclophosphamide
Electron Microscopy
Adrenal Cortex Hormones
Serologic Tests
Hematuria
Immunoenzyme Techniques
Proteinuria
Fluorescence Microscopy
Chronic Kidney Failure
Renal Insufficiency
Fluorescent Antibody Technique
Dialysis

Keywords

  • Crescents
  • Glomerulonephritis (GN)
  • Immunoglobulin A (IgA) nephropathy
  • Renal biopsy
  • Vasculitis

ASJC Scopus subject areas

  • Nephrology

Cite this

Haas, M., Jafri, J., Bartosh, S. M., Karp, S., Adler, S. G., & Meehan, S. M. (2000). ANCA-associated crescentic glomerulonephritis with mesangial IgA deposits. American Journal of Kidney Diseases, 36(4), 709-718.

ANCA-associated crescentic glomerulonephritis with mesangial IgA deposits. / Haas, Mark; Jafri, Jabeen; Bartosh, Sharon M.; Karp, Sharon; Adler, Sharon G.; Meehan, Shane M.

In: American Journal of Kidney Diseases, Vol. 36, No. 4, 2000, p. 709-718.

Research output: Contribution to journalArticle

Haas, M, Jafri, J, Bartosh, SM, Karp, S, Adler, SG & Meehan, SM 2000, 'ANCA-associated crescentic glomerulonephritis with mesangial IgA deposits', American Journal of Kidney Diseases, vol. 36, no. 4, pp. 709-718.
Haas, Mark ; Jafri, Jabeen ; Bartosh, Sharon M. ; Karp, Sharon ; Adler, Sharon G. ; Meehan, Shane M. / ANCA-associated crescentic glomerulonephritis with mesangial IgA deposits. In: American Journal of Kidney Diseases. 2000 ; Vol. 36, No. 4. pp. 709-718.
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AB - Antineutrophil cytoplasmic autoantibodies (ANCA) are commonly associated with a necrotizing and crescentic glomerulonephritis (GN) that is pauci-immune, with few or no glomerular immune complex deposits detectable by immunofluorescence (IF) or electron microscopy (EM). Immunoglobulin A (IgA) nephropathy may also be manifest as e crescentic GN, but it is characterized by mesangial immune complex deposits containing IgA and is rarely associated with myeioperoxidase (MPO)- or proteinase 3 (PR3)-specific ANCA when an enzyme immunoassay is used to detect these antibodies. This report describes six patients with severe crescentic GN with mesangial IgA deposits by IF and mesangial electron-dense deposits by EM in patients with positive ANCA serological test results (four patients, anti-PR3; one patient, anti-MPO; one patient, anti-PR3 and anti-MPO). Patients presented with acute or progressive renal insufficiency, hematuria, proteinuria (nephrotic range in two patients), and hypertension. Three patients had evidence of systemic vascuiitis: two patients at initial presentation and one patient later in the clinical course. Renal biopsy specimens showed crescents in greater than 50% of glomeruli in all cases, but only mild, focal and segmental mesanglal and endocapillary hypercellularity, more typical of ANCA-associated crescentic GN than of crescentic IgA nephropathy without associated ANCA. Semiquantitative analysis of mesangial and endocapillary cellularity performed on renal biopsy slides from these six patients and from eight ANCA-negative patients with IgA nephropathy and crescents in greater than 50% of glomeruli showed significantly greater hypercellularity in the ANCA-negative cases. Three of five ANCA-positive patients for whom follow-up clinical data were available showed improved renal function after treatment with cyclophosphamide and corticosteroids and have not developed end-stage renal disease 17, 20, and 25 months postbiopsy. The remaining two patients were dialysis dependent at the time of biopsy and have remained so despite treatment with cyclophosphamide and corticosteroids. The findings suggest an overlap syndrome of ANCA-associated crescentic GN and IgA nephropathy that resembles the former both histologically and in its potential to respond to aggressive therapy if detected relatively early in its course. (C) 2000 by the National Kidney Foundation, Inc.

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