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Saudi Journal of Kidney Diseases and Transplantation
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Table of Contents   
CASE REPORT  
Year : 2012  |  Volume : 23  |  Issue : 2  |  Page : 343-345
Diffuse proliferative crescentic lupus nephritis with normal renal function


1 Department of Nephrology, Jawaharlal Nehru Medical College (JNMC), Aligarh Muslim University (AMU), Aligarh, India
2 Department of Pediatrics, Jawaharlal Nehru Medical College (JNMC), Aligarh Muslim University (AMU), Aligarh, India

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Date of Web Publication28-Feb-2012
 

   Abstract 

Crescentic nephritis can be a manifestation of Lupus Nephritis. It usually presents with rapid deterioration of renal functions over days to weeks with an active urinary sediment and confers a poor prognosis. Nephrotic range proteinuria is rare in crescentic nephritis. We present an interesting case of (crescentic) Diffuse Proliferative Lupus Nephritis (DPLN) with remarkably preserved renal function and nephrotic range proteinuria. Also noteworthy was the patient's excellent response to immunosuppressive therapy despite the high frequency of cellular crescents on biopsy.

How to cite this article:
Gupta V, Sachdeva S, Khan AS, Haque SF. Diffuse proliferative crescentic lupus nephritis with normal renal function. Saudi J Kidney Dis Transpl 2012;23:343-5

How to cite this URL:
Gupta V, Sachdeva S, Khan AS, Haque SF. Diffuse proliferative crescentic lupus nephritis with normal renal function. Saudi J Kidney Dis Transpl [serial online] 2012 [cited 2019 Oct 20];23:343-5. Available from: http://www.sjkdt.org/text.asp?2012/23/2/343/93171

   Introduction Top


Though Crescentic Glomerulonephritis is a characteristic of ANCA positive vasculitic syndromes, it may less commonly be a mani­festation of Lupus Nephritis, limited to mainly ISN class IV and III. [1] While ISN class IV Diffuse Proliferative Lupus Nephritis (DPLN) itself carries a poor prognosis if timely inter­vention is not carried out, the formation of crescents further warrants rapid deterioration of renal function and a very poor outcome. [1],[2] However, we present a case of class IV Crescentic Lupus Nephritis with nephrotic range proteinuria and preserved renal function who responded remarkably well to treatment.


   Case Report Top


An 18-year-old young girl presented with complaints of progressive swelling over body since previous 3-4 weeks, along with decreased urinary output. There was no history of hema-turia, sore throat, skin infection, or any other significant past events. On examination, she was hypertensive with a blood pressure of 160/100 mmHg and had anasarca. Her urine examination showed 4+ albumin with presence of 4-5 erythrocytes and 10-12 leukocytes/High Power Field with numerous erythrocytes and leukocyte casts. Her renal functions showed a blood urea nitrogen of 15 mg% (normal range: 10-20 mg%) and serum creatinine (SCr) of 1.2 mg% (normal: <1.5 mg%). The total urinary proteins (TUP) were 6 g/24 hours, total serum proteins were 3.9 g% (normal range: 5.5-8.5 g%), serum albumin was 1.6 g% (normal range: 3.5-5.5 g%), and serum globulin was 2.3 g% (normal range: 2.5-3.5 g%). Serum lipid profile revealed low-density lipoprotein (LDL) cholesterol 301 mg% and triglycerides 246 mg%. Immunological studies showed anti-streptolysin O negative, antinuclear antibody positive, and anti-ds DNA positive (140 IU/mL titer). Appropriate diuretic therapy was adminis­tered and a renal biopsy was performed when edema subsided. The biopsy report showed dif­fuse endo-capillary proliferation with 10 out of 16 glomeruli (60%) showing partial and cir­cumferential cellular crescents and one glome-rulus with fibrocellular crescent with moderate chronic tubulointerstitial nephritis and patchy tubular atrophy [Figure 1]. A diagnosis of Class IV crescentic DPLN was made and patient was started on monthly pulse cyclophosphamide and oral methylprednisolone 1 mg/kg body weight. The patient responded with reduction in proteinuria to 0.5 g/day in five months (till date) and resolution of edema and norma­lization of urinary sediment though hyperten­sion persisted.
Figure 1: Slide showing two glomeruli: one in the center, showing circumferential crescent formation and the other one in the periphery (11 o'clock position) with crescent formation.

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   Discussion Top


Nephrotic range proteinuria occurs rarely in crescentic nephritis [3] which was a characteristic feature of this case. Also, several researchers have shown that class IV Lupus with extensive crescent formation carries a grave prognosis and most patients have gross derangement in renal functions. The percentage of glomeruli that show crescents usually correlates with the severity of renal failure in their studies. [4],[5],[6],[7],[8] This was in contrast with our observation of a relatively preserved renal function in spite of more than 60% crescents on biopsy. However, lately, research has shown that in almost all the crescentic nephritides, the extent of renal failure, and therefore the prognosis, are largely a function of the chronicity indices (i.e. tubulointerstitial scarring, followed in order by glomerular sclerosis and crescentic fibrosis), and the number of normal glomeruli, rather than any measure of crescentic glomeruli alone. [3],[8] It is, therefore, a possibility that the presence of only a moderate chronic tubulointerstitial neph­ritis resulted in a relatively well-preserved renal function in our case. Also, since all the crescents were active cellular crescents, timely initiation of immunosuppression must have re­sulted in regression of proteinuria and reso­lution of the disease process.

We infer that active cellular crescentic neph­ritis can respond well to timely immunosup-pression, irrespective of the number of cre-cents. Renal failure correlates better with the presence of markers of chronicity, i.e. glome-rular sclerosis, fibrous crescents, tubular atro­phy, and interstitial fibrosis, which were mini­mal in this case. [8],[9]

 
   References Top

1.Leaker B, Fairley KF, Dowling J, Kincaid-Smith P. Lupus nephritis: clinical and patho­logical correlation. Q J Med 1987;62:163-79.  Back to cited text no. 1
[PUBMED]  [FULLTEXT]  
2.Sumethkul V, Chalermsanyakorn P, Changsiri-kulchai S, Radinahamed P. Lupus nephritis: a challenging cause of rapidly progressive cres-centic glomerulonephritis. Lupus 2000;9:424- 8.  Back to cited text no. 2
    
3.Levy J, Pusey-Charles D. Crescentic nephritis. Oxford Textbook Of Clinical Nephrology 3 rd ed.(Vol. I),315.  Back to cited text no. 3
    
4.Couser WG. Rapidly progressive glomerulo-nephritis: Classification, Pathogenetic mecha­nisms and therapy. Am J Kidney Dis 1988;11 (6):449-64.  Back to cited text no. 4
    
5.Whitworth JA, Morel-Maroger L, Mignon F, Richet G. The significance of extracapillary proliferation. Clinicopathological review of 60 patients. Nephron 1976;16:1-19.  Back to cited text no. 5
[PUBMED]    
6.Beirne GJ, Wagnild JP, Zimmerman SW, Macken PD, Burkholder PM. Idiopathic crescentic glo-merulonephritis. Medicine 1977;56:349-81.   Back to cited text no. 6
    
7.Morrin, PA, Hinglais N, Nabarra B, Kreis H. Rapidly progressive glomerulonephritis. A cli­nical and pathologic study. Am J Med 1978; 65:446-60.  Back to cited text no. 7
    
8.Austin HA 3d, Muenz LR, Joyce KM, Anto-novych TT, Balow JE. Diffuse proliferative lupus nephritis: identification of specific pathologic features affecting renal outcome. Kidney Int 1984;25(4):689-95.  Back to cited text no. 8
    
9.Sasatomi Y, Kiyoshi Y, Takabeyashi S. A cli­nical and pathological study on the charac­teristics and factors influencing the prognosis of crescentic glomerulonephritis using a cluster analysis. Pathol Int 1999;49(9):781-5.  Back to cited text no. 9
    

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Correspondence Address:
Sandeep Sachdeva
Department of Pediatrics, Jawaharlal Nehru Medical College (JNMC), Aligarh Muslim University (AMU), Aligarh
India
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PMID: 22382232

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    Abstract
   Introduction
   Case Report
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    References
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