An update on pathology of IgA nephropathy

Soares, Maria Fernanda

doi:10.5935/0101-2800.20160069

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Brazilian Journal of Nephrology

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Sumário

Review Article • J. Bras. Nefrol. 38 (4) • Oct-Dec 2016 • https://doi.org/10.5935/0101-2800.20160069 copy

An update on pathology of IgA nephropathy

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

IgA Nephropathy (IgAN) is the commonest of the glomerular diseases in the world. Its progression rate of 30-40% of the cases em 20-30 years makes IgAN an important healthcare issue in Nephrology. Diagnosis of IgAN depends on biopsy findings, particularly at immunofluorescence microscopy. The frequence of IgAN diagnosis is variable in different populations and depends on screening and biopsy indication policies. IgAN pathogenesis is considered multifactorial; its primordial defect is the production of galactosis-deficient IgA molecules. This review paper discusses the most uptodate aspects of the pathogenesis, pathological classification and clinical implications of IgAN.

Keywords:
glomerulonephritis; glomerulonephritis, IGA; immunoglobulin A; pathology

	Haas Subclasses	Lee Grades
I	Absent to minimal mesangial hypercellularity.	Absent to minimal mesangial hypercellularity. No tubulo-interstitial inflammation or atrophy. Focal and segmental
II	Focal segmental glomerulosclerosislike pattern.	mesangial hypercellularity. Sparse crescents. No tubulo-interstitial inflammation or atrophy.
III	Focal proliferative glomerulonephritis (< 50%) with ou without crescents.	Diffuse mesangial hypercellularity. Occasional crescents and synechiae. Focal tubulo-interstitial inflammation and oedema. Diffuse mesangial
IV	Diffuse proliferative glomerulonephritis (> 50%) with ou without crescents.	hypercellularity and matrix expansion. Frequent segmental sclerosis. Crescents in up to 45% of glomeruli. Tubulo-interstitial inflammation.
V	≥ 40% of globally sclerotic glomeruli or tubular atrophy/ interstitial fibrosis, with or without active glomerular lesions.	Similar to IV; > 45% of glomeruli with crescents. Severe tubular atrophy and interstitial fibrosis.

	Histological Parameter	Score
M	Mesangial proliferation	M0 (absent)/M1 (present in > 50% of glomeruli)
E	Endocapillary hypercellularity	E0 (absent)/E1 (present)
S	Segmental sclerosis/ synechiae	S0 (absent)/S1 (present)
T	Tubular atrophy/ interstitial fibrosis	T0 (0-25%^* * relative to cortical extension Pathology report should ideally include: % of globally sclerotic glomeruli % of crescents % of glomeruli with necrosis % of glomeruli with endocapillary hypercellularity. )/T1 (26-50%^* * relative to cortical extension Pathology report should ideally include: % of globally sclerotic glomeruli % of crescents % of glomeruli with necrosis % of glomeruli with endocapillary hypercellularity. )/T2 (> 50%^* * relative to cortical extension Pathology report should ideally include: % of globally sclerotic glomeruli % of crescents % of glomeruli with necrosis % of glomeruli with endocapillary hypercellularity. )

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[1] Correspondence to: Maria Fernanda Soares. Universidade Federal do Paraná. Rua Padre Camargo, nº 280, 6º Andar, Alto da Gloria, Curitiba, Brazil. CEP: 80060-240. E-mail: mafer.soares@ufpr.br