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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT  
Year : 2011  |  Volume : 22  |  Issue : 4  |  Page : 784-787
Necrotic crescentic glomerulonephritis and IGA nephropathy: Lee-Haas classification revisited


Department of Nephrology, NMC Specialty Hospital, Dubai, United Arab Emirates

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Date of Web Publication9-Jul-2011
 

   Abstract 

Capillary necrosis is frequently encountered in most forms of proliferative glomerulonephritis, and varies in severity from only occasional finding to severe form of glomerular vasculitis. We report a patient who presented with features of acute glomerulonephritis. Kidney biopsy showed crescents in 30% of the glomeruli, diffuse mesangial expansion and hypercellularity with IgA deposits on immunofluorescence. The relevance of the Lee-Haas classification is discussed in relation to assessing prognosis.

How to cite this article:
Jabur WL. Necrotic crescentic glomerulonephritis and IGA nephropathy: Lee-Haas classification revisited. Saudi J Kidney Dis Transpl 2011;22:784-7

How to cite this URL:
Jabur WL. Necrotic crescentic glomerulonephritis and IGA nephropathy: Lee-Haas classification revisited. Saudi J Kidney Dis Transpl [serial online] 2011 [cited 2019 Jul 19];22:784-7. Available from: http://www.sjkdt.org/text.asp?2011/22/4/784/82697

   Introduction Top


Capillary necrosis is frequently encountered in most forms of proliferative glomerulonephritis, and varies in severity from only occasional finding to severe prototype of the disease in the form of glomerular vasculitis. It is not clear whether capillary necrosis reflects the severity of the disease or it is a part of the pathological process that occurs in the mesangium. In IgA nephropathy, according to the Lee-Hass classification, the presence of crescents highlights the severity of the disease in general.


   Case Report Top


A 43-year-old female patient presented with a subtle history of non-blanchable skin purpura, epistaxis, arthritis of the right ankle, active urinary sediment, nephrotic syndrome, and barely normal kidney function. Blood pressure was slightly elevated at 160/95 mmHg. Screening for systemic disease, including anti-nuclear cytoplasmic antibodies (ANCA), cryoglobulin, anti-phospholipid antibodies and serum complement, was normal. Past medical history was remarkable for recurrent similar skin lesions without any evidence of kidney disease. Kidney biopsy was performed and renal histology showed normal to mildly increased amount of mesangial expansion, more than 20% of the glomeruli showing epithelial crescents, more than 30% of the glomeruli showing capillary necrotic lesions and one glomerulus having an intra-capillary microthrombus [Figure 1] and [Figure 2]. The glomerular basement membrane did not appear to be thickened and there was no double-contouring on periodic acid Schiff (PAS) staining. There was diffuse meangial expansion and hypercellularity [Figure 3]. Tubules showed features suggestive of acute tubular necrosis. Inter-lobular arteries and arterioles were unremarkable. Immunofluorescence study revealed the presence of diffuse mesangial IgA deposits. Electron microscopy showed the presence of diffuse foot process effacement and dense mesangial deposits [Figure 4]. Treatment with oral prednisolone (1 mg/kg body weight) was commenced. The patient showed a dramatic improvement with this treatment.
Figure 1: Kidney biopsy showing cellular crescents with glomerular vascular necrosis.

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Figure 2: Renal histology showing glomerular vascular necrosis.

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Figure 3: Electron microscopic study showing mesangial and sub-endothelial dense deposits.

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Figure 4: Renal histology showing mesangial expansion and hypercellularity.

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   Discussion Top


IgA nephropathy is the most common glomerular disease all over the world. Although its systemic vasculitic corollary of Henoch-Schonlein Pupura (HSP) is more common in children, it is not infrequently encountered in adults. [1] The diverse presentation of IgA nephropathy is well recognized. Despite the fact that the Lee-Hass classification helps in assessing the severity of the disease to a certain extent, [2] direct correlation of the clinical features to the underlying histopathology is inconsistent. Some histopathological features have not been categorized within the subdivisions of the aforementioned classification. One of the frequently encountered histopathological features in IgA nephropathy is the presence of crescents, although it is more common in HSP; [3] this has not been addressed clearly in the classification. It is conceivable that the histopathological grading in the Lee-Hass classification partly depends on the percentage of glomeruli showing crescents.

Despite the established evidence that the extra-capillary proliferation is secondary to capillary rupture and spillage of lymphocytes and other cells into the Bowman's space, with the consequent formation of cellular crescents, which is a hallmark of intra-glomerular vasculitis, it is still unclear whether crescents are encountered in the context of glomerular vasculitis or as part of a more extensive mesangial proliferation. Also, it is not clear whether crescents reflect chronicity or activity of the disease, and if crescents are ultimately the corollary of capillary inflammation, then the correlation between crescents and glomerular capillary necrosis needs to be addressed. It is yet unclear whether they are always concomitant findings. It might reflect a continuum of one disease starting in the mesangium and extending to the capillaries (because the mesangium and the sub-endothelial space are in direct continuation), resulting in a mosaic picture of crescentic glomerulonephritis and diffuse mesangial proliferation in the same field. However, the disease might be affecting mainly the mesangium, which is the most common presentation. Less-commonly, renal vasculitis may be the sole feature, presenting either alone or as part of the HSP. [4]

Therefore, we think that it is inappropriate to classify IgA nephropathy as a vasculitic process based on its histopathological findings of necrotic crescentic glomerulonephritis. This is in agreement with the suggestions of D Amico et al. [3] Additionally, features suggestive of mesangial proliferative glomerulonephritis, reflected by variable degrees of mesangial proliferation, are a feature of IgA nephropathy. It is well recognized that both the lesions might overlap, although our concern is that of the unique IgA vasculitic disease. Based on this thinking, we believe that the presentation and prognosis might be different. Because the LeeHass classification seems to delineate the grades of IgA nephropathy based on chronicity, depending on the sclerotic and the crescentic lesions to categorize variable grades, [5] the classification might reflect the clinical features in a parallel manner, as indicated by several researches. [6] The histologically active lesions have not been appraised thoroughly, particularly the glomerular capillary necrosis, which is omitted from the classification. Although the necrotic crescentic lesion is well recognized in almost all kinds of severe proliferative immune-complex glomerulonephritis, we believe that in IgA nephropathy, crescentic lesions reflect a peculiar acute vascular involvement accompanying glomerular capillary necrosis in the presence or absence of significant mesangial proliferative lesions. This is widely recognized in the context of HSP (as in the patient we are presenting). On the other hand, absence of ANCA, AGBM and ANF antibodies would hamper the suggestion of superimposed ANCA-related systemic vasculitis, Goodpasture's syndrome and systemic lupus erythematosis-related anti-phospholipid antibody syndrome as the harbinger for capillary necrosis. [7] However, it is well recognized that there is an association between ANCA and necrotic IgA nephropathy.

Thus, it is conceptualized that the necrotic crescentic lesion in the context of IgA nephropathy cannot be considered as a kind of rapidly progressive glomerulonephritis because it does not always fulfill the criteria for small-vessel vasculitis (crescentic lesion in more than 50% of the glomeruli). For this reason, the treatment strategy for IgA nephropathy with prominent crescentic lesions is still controversial.

We might therefore speculate that the prognosis of IgA nephropathy is divergent, and depends on whether it is predominantly vascular or mesangial, as well as on the acuteness of the crescentic lesions, particularly with cellular kind of crescents. Contrary to the report of D Amico et al, [3] we are inclined to believe that the vascular lesion might indicate a better prognosis (because it is an acute lesion) than the mesangial lesions (especially mesangial expansion and mesangial sclerosis), which are more chronic in nature.

The use of aggressive therapy is still debatable, and we believe that it should be used for patients with cellular crescents because, without treatment, they might get transformed into fibrous crescents; further studies are needed to uncover this issue. It is commonly recognized that the severity of proteinuria is directly related to a worse prognosis, and it is frequently reported in association with severe proliferative glomerulonephritis with extensive crescentic formation. However, the absence of massive crescentic lesions, presence of normal kidney function and the dramatic improvement seen with steroid treatment would denote that it is predominantly a steroid-responsive minimal change disease. Also, the widespread foot process effacement shown on electron microscopy would foster this proposition. To the best of our knowledge, the association of minimal change disease with HSP is not widely recognized.

 
   References Top

1.Davin JC, Ten Berge IJ, Weening JJ. What is the difference between IgA nephropathy and Henoch-Schonlein purpura nephritis? Kidney Int 2001;59:823-34.  Back to cited text no. 1
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2.Barratt J, Feehally J. IgA Nephropathy. J Am Soc Nephrol 2005;16:2088-97.  Back to cited text no. 2
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3.Amico GD, Napodano P, Ferrario F, et al. Idiopathic IgA nephropathy with segmental necrotizing lesions of the capillary wall. Kidney Int 2001;59:682-92.  Back to cited text no. 3
    
4.Tumlin JA, Madaio MP, Hennigar R. Ideopathic IgA nephropathy: Pathogenesis, histopathology, and therapeutic options. Clin J Am Soc Nephrol 2007;2(5):1054-61.  Back to cited text no. 4
    
5.Lee Hs, Lee MS, Lee SM, et al. Histopathological grading of IgA nephropathy predicting renal outcome: Revisiting H.S Lees glomerular grading system. Nephrol Dial Transplant 2005;20(2):342-8.  Back to cited text no. 5
    
6.Manno C, Strippoli GF, D'Altri C, Torres D, Rossini M, Schena FP. A novel Simpler Histological Classification for Renal Survival in IgA Nephropathy: A Retrospective Study. Am J Kidney Dis 2007;49(6):769-75.  Back to cited text no. 6
    
7.Lamprecht P, Schmitt WH, Gross WL. Mixed cryoglobulinaemia, glomerulonephritis, and ANCA: essential cryoglobulinemic vasculitis or ANCA-associated vasculitis. Nephrol Dial Transplant 1998;13:213-21.  Back to cited text no. 7
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Correspondence Address:
Wael Latif Jabur
NMC Specialty Hospital, P. O. Box 7832 Dubai
United Arab Emirates
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PMID: 21743230

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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    Abstract
   Introduction
   Case Report
   Discussion
    References
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