Home About us Current issue Ahead of Print Back issues Submission Instructions Advertise Contact Login   

Search Article 
  
Advanced search 
 
Saudi Journal of Kidney Diseases and Transplantation
Users online: 1056 Home Bookmark this page Print this page Email this page Small font sizeDefault font size Increase font size 
 

Table of Contents   
RENAL DATA FROM THE ASIA - AFRICA  
Year : 2022  |  Volume : 33  |  Issue : 1  |  Page : 122-131
Clinicohistological Profile and Outcomes of Rapidly Progressive Glomerulonephritis: A Prospective Study from North India


1 Department of Nephrology, Dayanand Medical College and Hospital, Ludhiana, Punjab, India
2 Department of Nephrology, Maharishi Markandeshwar Institute of Medical Sciences and Research, Mullana, Ambala, Haryana, India

Click here for correspondence address and email

Date of Web Publication16-Jan-2023
 

   Abstract 


The prevalence of this rare and fatal entity varies in different regions and ethnicities. The objective of this study was to determine clinicopathological characteristics and predictors of outcome in rapidly progressive glomerulonephritis (RPGN). We aimed to prospectively study the clinicopathological profile and determine the predictors of renal outcome in patients with RPGN. This study included 40 patients of biopsy-proven RPGN. The diagnosis of RPGN was based on renal histology showing crescents in >50% of glomeruli. All patients were given induction with intravenous (IV) methylprednisolone (0.5 g) for three days followed by maintenance with oral prednisolone (1 mg/kg/day) and six IV pulses of cyclophosphamide (0.5 g) given fortnightly followed by maintenance therapy with azathioprine (2 mg/kg/day). The outcomes were recorded. Three-fourth of the patients (77.4%) required renal replacement therapy (RRT) at diagnosis. More than half of patients (57.5%) were antineutrophil cytoplasmic antibodies mediated. Immune complex and anti-glomerular basement membrane (GBM) disease constituted 25% and 17.5%, respectively. Clinical features, biochemical parameters, histological features, and type of RPGN were analyzed for association with primary outcomes. Entry serum creatinine, entry estimated glomerular filtration rate (eGFR), RRT on admission, interstitial fibrosis, tubular atrophy, and interstitial infiltrates were the parameters which showed association with primary outcomes of the study (P <0.05). In secondary outcomes, infections were the most common (55%), followed by neutropenia (40%). One-fourth of the patients (25%) died during the course of the study. Cause of mortality was infections (50%), cardiovascular system (30%), stroke (10%), and unknown (10%). Our prospective study from north India shows that RPGN is not an uncommon cause of renal failure and there is preponderance in the elderly patients (>60 years). Pauci-immune RPGN is the most common cause of RPGN followed by immune-complex and anti-GBM disease. Entry serum creatinine, eGFR, and RRT on admission predicted the outcome.

How to cite this article:
Sharma D, Mehta S, Sandhu JS, Sohal PM, Makkar V. Clinicohistological Profile and Outcomes of Rapidly Progressive Glomerulonephritis: A Prospective Study from North India. Saudi J Kidney Dis Transpl 2022;33:122-31

How to cite this URL:
Sharma D, Mehta S, Sandhu JS, Sohal PM, Makkar V. Clinicohistological Profile and Outcomes of Rapidly Progressive Glomerulonephritis: A Prospective Study from North India. Saudi J Kidney Dis Transpl [serial online] 2022 [cited 2023 Jan 29];33:122-31. Available from: https://www.sjkdt.org/text.asp?2022/33/1/122/367805



   Introduction Top


Rapidly progressive glomerulonephritis (RPGN) is a clinical syndrome characterized by a rapid loss of renal function, often accompanied by oliguria or anuria, by features of glomerulonephritis, including dysmorphic erythrocyturia, erythrocyte cylindruria, and glomerular proteinuria.[1] Aggressive GN that causes RPGN usually has extensive crescent formation. For this reason, the clinical term RPGN is sometimes used interchangeably with the pathological term crescentic GN.[2] Crescentic GN is characterized clinically by a sudden and progressive decline in renal function and histopathologically by crescents in 50% or more of glomeruli. This disease can accompany most forms of primary GN, but it is also associated with various systemic diseases.[3] Crescentic GN is typically associated with the syndrome of RPGN, which can occur in most forms of inflammatory glomerular injury, including postinfectious GN, IgA nephropathy, lupus nephritis (LN), renal vasculitis, membranoproliferative GN, and anti-glomerular basement membrane (GBM) antibody disease. Crescentic GN is a disease with grave prognosis. Retrospective biopsy based series has reported a prevalence varying from 2.1% to 4.2%[4],[5] There is significant heterogeneity in the etiology and outcomes of[6],[7] with limited data from India.[7],[8],[9] The aim of the present study was to analyze our experience with patients with RPGN that were diagnosed and followed in one single center. Furthermore, an attempt was made to identify clinical, biochemical, histological, and immunohistochemical findings predictive of response to treatment and outcome.


   Materials and Methods Top


The prospective study was done in the Department of Nephrology, Dayanand Medical College and Hospital, Ludhiana over a period of 15 months from January 1, 2018, to March 31, 2019. A total of 40 patients of RPGN were enrolled in the study after approval from institutional ethical committee. The diagnosis of RPGN was based on renal histology showing crescents in >50% of glomeruli. Adults patients (>18 years of age) with rapidly progressive renal failure with biopsy-proven RPGN were included in this study. Patients with rapidly progressive renal failure with crescents <50% in renal biopsy and those, who refused to give consent for renal biopsy were excluded.

Methods of collection of data

After a written informed consent, a detailed history of the patient was taken, with respect to symptoms of various organ manifestations, duration of symptoms before presentation, and complete general physical and systemic examination was done. Investigations were done at the time of presentation, including complete blood count with erythrocyte sedimentation rate, renal function tests, lipid profile, liver function tests, blood sugar, urine microscopy, dipstick analysis, and 24 h urine protein or spot protein to creatinine ratio. The estimated glomerular filtration rate (eGFR) was calculated by the Chronic Kidney Disease Epidemiology Collaboration equation. Immunological profile done include antineutrophil cytoplasmic antibodies (ANCA) and anti-GBM antibodies [by Dot-blot strip test and indirect immunofluorescence (IF) test/ELISA], antinuclear antibody (ANA) [by indirect IF technique], anti-double-stranded DNA antibodies (using indirect IF test), ANA profile (by immunoblot using Euroline test Kit) and serum complement levels (by nephelo-metry). Electrocardiogram, chest X-ray (PA), and ultrasonography of the abdomen were done in all patients, and echocardiography was done wherever deemed necessary.

An ultrasound-guided percutaneous renal biopsy was done in all the patients, using an automated biopsy gun. The specimen was prepared for light microscopy and IF studies. Renal biopsy specimens were fixed in 4.5% buffered formaldehyde for light microscopy. Consecutive serial 3 μm specimens were used for histological staining. Stains employed included hematoxylin-eosin, periodic acid–Schiff, Jones silver methenamine, and Masson’s trichrome. Number and type of crescents were looked as per the definition of RPGN.

Depending on the type of RPGN and overall clinical status, the patients were treated for the disease as per standard protocol. Patients were started on induction therapy with three methylprednisolones (0.5 g) pulses followed by oral prednisolone plus six intravenous cyclophosphamide fortnightly pulses (500 mg each). After induction phase, patients were started on maintenance therapy with azathioprine (1.5–0.5 mg/kg/day). Patients were followed up for a period of six months. Complete blood counts, serum creatinine, blood urea nitrogen, urine routine examination, and 24 h urine protein or spot urine to protein creatinine ratio were done according to predefined intervals.

Outcomes

Response to therapy was studied in terms of primary and secondary outcomes. Primary outcomes were remission and death. Complete remission was defined as 24 h urine protein <500 mg/day and serum creatinine <1.4 mg/dL. Partial remission was defined as stable or decreasing serum creatinine in nondialysis patients, dialysis independence, and serum creatinine <5.8 mg/dL in dialysis dependents. No response was defined as increasing serum creatinine or dialysis dependency or ESRD in nondialysis patients and dialysis dependency or serum creatinine >5.8 mg/dL in dialysisdependent patient. Death of a patient during the next six months after enrollment was another primary outcome of the study. Secondary outcomes were infections, hematological complications (anemia, neutropenia or thrombocytopenia), endocrinological complications (development of diabetes mellitus), cardiovascular complications (cardiac failure, dilated cardiomyopathy), dermatological complications (alopecia, rash), and gastrointestinal complications (vomiting, diarrhea).


   Statistical Analysis Top


Results were expressed as mean ± standard deviation continuous data and as percentage for categorical data. For comparison of clinical and pathological features of patients, Student’s t-test and one-way ANOVA analysis of variance were used. T-test was used to compare two mean values. ANOVA was performed to compare more than two mean values at a time. Kruskal–Wallis test was used for the analysis of nonparametric variables. The association between categorical variables was assessed using Chi-square test (Fisher’s exact test and Yates corrected). Kaplan–Meier survival analysis was used for patient survival. All the statistical analysis was done using the Statistical Package for the Social Sciences (SPSS) software version 17.0 (SPSS Inc., Chicago, Ill, USA). P <0.05 was considered as statistically significant.


   Results Top


Forty-three patients of RPGN were included. There were 25 males (58.1%) and 18 females (41.9%), thus giving a male-to-female ratio of 1.3:1. Three patients were lost to follow-up after renal biopsy. Finally, 40 patients were followed for a minimum study period of six months. The mean age of patients was 46.4 ± 16.3 years, with a range of 20 to 75 years. The mean age of patients was 44.8 ± 15.8 and 48.2 ± 17.1 years in males and females respectively. There was a slight male preponderance in all age groups except in older than 60 years. Age difference in both the groups was not statistically significant (P = 0.06). The mean duration of symptoms before diagnosis was 37 .1 ± 38.3 days. Above half of our patients (55%) had pedal edema, followed by oliguria seen in 50% of the patients. Gross hematuria was present in only 15% of patients. About half (52.5%) of our patients had constitutional symptoms, in which fever was the most common (40%). Dyspnea (37.5%) and hemoptysis (15%) are also observed. Vomiting and myalgia were reported by 22.5% and 20% of patients and wrist drop was seen in only one. About one-third of patients (32.5%) had hypertension at presentation [Table 1] and [Table 2].
Table 1: Incidence of renal symptoms.

Click here to view
Table 2: Incidence of extra renal symptoms.

Click here to view


Renal failure was present in all the patients on admission. The mean serum creatinine was 9.1 ± 4.7 mg/dL with a range from 1.52 to 25.24 mg/dL. One-third (32.5%) of the patients in our study had serum creatinine >9.5 and one-fourth (27.5%) had in the range of 5.6–7.5 mg/dL. The creatinine between 1.5 and 3.5 mg/dL was seen in three patients. 80% of our patients presented with the serum creatinine of >5.5 mg/dL indicating severe renal failure on admission. The mean eGFR on admission was 9.34 ± 9.0 mL/min/1.73 m2. Almost all the patients (92.5%) presented with eGFR <15 mL/min/1.73 m2 in our study. Only one patient had eGFR between 45 and 59 mL/min/1.73 m2. Proteinuria is present in all the patients. The mean 24 h urine protein was 2.7 ± 2.1 g/day. More than half of our patients (52.5%) had proteinuria between 1 and 3 g/day and one-third (32.5%) had more than 3 g/day [Table 3]. Only six patients (15%) had <1 g of proteinuria. More than three-fourths of the patients (80%) had nephritic presentation while in the remaining eight patients (20%) nephrotic-nephritic presentation was observed.
Table 3: 24 h urine protein.

Click here to view


As reported in the western data, pauciimmune was the most common cause of RPGN observed in our study, involving more than half (57.5%) of patients followed by immune-complex and anti-GBM in 25% and 17.5% of the patients respectively [Table 4]. Anti-GBM disease was the most severe type of RPGN seen in our study, which was present in seven patients and four out of seven were in combination with ANCA. Three were with myeloperoxidase (MPO)-ANCA positive and one with proteinase 3 (PR3)-ANCA positive. MPO-ANCA positivity in more than one-third (43.5%) of patients, however, one-fourth (26.1%) of the patients were PR3-ANCA positive. ANCA negative and dual ANCA positivity were present in 21.7% and 8.7% patients, respectively [Table 5].
Table 4: Etiology of rapidly progressive glomerulonephritis.

Click here to view
Table 5: Pauci-immune.

Click here to view


IgA nephropathy was the most common cause of immune complex-mediated RPGN in our study. One patient presented as posttransplant recurrence with crescentic transformation. LN and MPGN was basic disease in two patients each. Three patients presented as post-infective GN (PIGN), C3 GN, and unknown etiology respectively [Table 6].
Table 6: Types of immune complex-mediated diseases.

Click here to view


Histology

For histological interpretation kidney biopsy sample should be adequate, preferably containing 10–12 glomeruli. Mean number of glomeruli in our study was 18 ± 9.7. The mean number of sclerotic glomeruli observed in our study was 27.2% ± 21.4%, suggesting an advanced stage of disease. Diagnosis of RPGN is based on >50% glomeruli showing crescents. The mean value of crescents in our study was 70.4% ± 17.5%. The mean value of cellular, fibro cellular and fibrous crescents were 70.4% ± 17.5%, 32.1% ± 27.3%, and 16.1 ± 24.7 respectively. Interstitial fibrosis and tubular atrophy generally presents together and indicates chronicity. Almost half of our patients had interstitial fibrosis (50%) and tubular atrophy (52.5%) suggesting chronic injury. Interstitial infiltrates were present in all the patients in the study. About half (45%) of the patients in our study had moderate inflammatory infiltrate. However, mild and severe infiltration by inflammatory cells was observed in 25 and 30 % of patients respectively [Table 7].
Table 7: Histological parameters.

Click here to view


Outcome

The primary outcome (response to treatment) after treating patient on standard line of the protocol was accessed after six months. In our study, two-third of patients (65%) showed noresponse. One-third of patients (35%) responded to treatment and had remission. About one-fourth (22.5%) of patients showed partial remission and 12.5% had complete remission. In our study more than three-fourth (85%) of the patients showing remission were older than 40 years. Non-responders were seen maximum in the age group of 21–30 years. There were total of 10 deaths including seven in female and three in males respectively. Half (50%) of patients who presented after more than 75 days died as compared to 15% who presented in 25 days. Oliguria (urine output <400 mL/24 h) was present in equal number of patients, both in responders and nonresponders. Majority of patients (92.5%) with remission presented with nephritic syndrome on admission. Three-fourth (72%) of patients with no response/died are nephritic. The degree of proteinuria had no correlation with outcomes [Table 8].
Table 8: Primary outcomes.

Click here to view


Majority (80%) of patients with complete remission had serum creatinine <5.5 mg/dL at entry. Whereas all the patients who had no response to treatment or expired had creatinine >5.5 on admission, complete remission (P = 0. 001), and no response (P = 0.003).

Three-fourth (78%) of patients with remission had eGFR (CKD-EPI) <15 mL/min compared to the patients with no response to treatment and those who died were admitted with eGFR <15 mL/min. Association of eGFR with outcomes were found significant, complete remission (P = 0.025), partial remission (P = 0.024), and no response (P = 0.049).

More than three-fourth (80%) of the patients with complete remission did not require renal replacement therapy (RRT) on admission compared to the requirement for RRT was present in all the patients with no response to treatment and those who expired The association was found to be highly significant. complete remission (P = 0.001), no response (P = 0.000) and death (P = 0.049). Almost two-thirds (61%) of non-responders and one-third of patients who died had >35% of the sclerosed glomeruli (P >0.05).

More than one-third (41%) of non-responders and two-thirds (61%) of those, who died had >70% of their glomeruli with crescents (P >0.05). In two-fifths (41%) of nonresponder and those who died had >65% of cellular crescents (P >0.05). Forty-five percent of nonresponders had >35% fibrocellular crescents. Fifty percent of nonresponders had >35% of fibrous crescents (P >0.05). More than three-fourths (78.5%) of the patients with remission showed the absence of interstitial fibrosis. Non-responders (61%) and those who died (71%) during the course of follow-up had significant interstitial fibrosis. In two-thirds (62%) of patients with no response and half of the patients (50%) who expired, tubular atrophy was observed. However, almost all the patients (excluding one) with no response and all patients, who died had moderate-to-severe interstitial infiltrate. Association found with primary outcome was highly significant in complete remission (P = 0.000), partial remission (P = 0.048) and no response group (P = 0.000).

In the study, complete remission was obtained in 17.3% of patients in pauci-immune group and one patient in the immune complex group. No patient with anti-GBM disease achieved remission. Almost one-third (30%) of patients had partial remission in pauci-immune disease versus immune complex disease (20%). No response to treatment was seen in anti-GBM disease. Three-fourths (70%) of patients with immune complex disease and half of the patients (52%) with pauci-immune disease were nonresponsive. Mortality was maximum in anti-GBM disease, including almost three-fourth (71%) of patients. Patient survival was maximum in pauci-immune group in our study. Correlation was obtained in mortality (P = 0.007).

The three-fourths of the patients (78.5%) who achieved remission were pauci-immune in etiology. Half of them (54.5%) were MPO-ANCA positive and more than two-fifths (45.4%) were PR3-ANCA positive. ANCA negative and dual ANCA positive (MPO + PR3) patients showed no remission in the study. The incidence of death in pauci-immune patients was 30% and all of them were MPO-ANCA type. No remission was observed in anti-GBM disease patients in our study. Half (50%) of the patients, who died during the study were anti-GBM positive.

Complications

More than half (55%) of our patients had infections followed by neutropenia seen in two-fifths (40%) of the patients and anemia in one-third (35%). Diabetes, cardiac failure, and vomiting were observed in 30%, 25%, and 40% of patients, respectively. In two-fifths (40.9%) of the patients, pneumonia was seen as the most common infection in our study followed by septicemia seen in one-fourth (27.9%) of the patients, and cellulitis, reactivation of pulmonary Kochs and urinary tract infection (UTI) in 9%, 9%, and 13.6% patients respectively.

Half (50%) of the patients in our study died because of Infections. Cardiac disease (30%) constitutes the second important cause of mortality followed by stroke (10%) and unexplained cause (10%). Mortality was 40% at 5th month and 30% of them died at 4th month of follow up.


   Discussion Top


More than half (58%) of our patients were males giving a male-to-female ratio of 1.3:1. Similar male preponderance has also been reported by others. Lv et al,[10] and de Lind van Wijngaarden et al.[11] The mean age of patients in our study was 46.3 ± 16.3 with an age range of 20–75 years. Similar observations have been reported by Fischer and Lager[12] in a study on anti-GBM patients and Naidu et al from North India in their study of 43 patients with pauci-immune GN.[13] Apart from the renal failure in all our cases, the symptomatology included pedal edema in 55%, oliguria in 50% and gross hematuria in 15%. One-third of the patients were hypertensive. Tang et al in their study reported oliguria in 49.3%, and hypertension in 60% of patients.[14] In another study Tang et al reported oliguria in 25%, and hypertension in 48% of pauci-immune crescentic GN patients.[15] In the present study, the mean value of urine proteins was 2.7 ± 2.1 g/day. About half (52.5%) had nonnephrotic proteinuria. One-third of patients had nephrotic range proteinuria of >3 g. Similarly, in their study from North India Choudhury et al[7] reported mean value of 24 h urine protein to be 2.59 ± 1.47 g with nephrotic presentation in 15% of patients. Rampelli et al[16] in their prospective study on crescentic GN reported mean 24 h urine proteinuria of 1.66 ± 1.77l.[16]

Renal failure was present in all our patients on admission. Eighty percent of the patients in our study presented with the serum creatinine of more than 5.5 mg/dL and one-third (32.5%) of them had a creatinine of more than 9.5 mg/dL on admission. The mean eGFR (CKDEPI) on admission was 9.34 ± 9.0 mL/min. Almost all the patients (92.5%) presented with eGFR ≤15 mL/min. Li et al in their study on patients with ANCA-associated vasculitis[17] and Rampelli et al in their prospective study on 37 patients with crescentic GN reported similar findings.[16] More than three-fourths of the patients (77.5%) in our study required RRT on admission indicating severe renal failure in majority of them. Similar findings were observed by Rampelli et al[16] and Prabhakar et al.[18]

Almost half of our patients (52.5%) had interstitial fibrosis and tubular atrophy, suggesting long-standing illness. About half (45%) of our patients had moderate inflammatory infiltrate. As in our study, de Lind van Wijngaarden et al[11] in their study of ANCA-associated vasculitis reported tubular atrophy in more than half (57%) of patients. Interstitial infiltrates were reported in 70% of patients. Li et al in a study of 89 patients had interstitial infiltrates, interstitial fibrosis, and tubular atrophy in 40%, 34% and 39% of patients respectively.[17]

Pauci-immune was the commonest cause of RPGN in our study seen in more than half (58%) of patients Gupta et al. reported similar results in their study from India.[8] However in contrast to our study, Choudhury et al[7] in their study reported immune complex-mediated RPGN as the commonest form of RPGN seen in more than one-third (45%) of patients followed by pauci-immune (30%) and anti-GBM disease (26.5%). The difference can be due to more of elderly patients in the present study and may be because Punjab is a rich and prosperous state in the country and there is less incidence of infection-related complications due to better living conditions and good dietary habits in the population. IgA nephropathy was the most common cause of immune complex-mediated RPGN in our study. Similar results were found in studies by Rampelli et al[16] and Li et al.[17]

In our study, one-third of patients (35%) responded to treatment and complete and partial remission was noted in 12.5% and 22.5% of patients, respectively. Similar results were found in a study by Kapitsinou et al.[19] No association with the degree of proteinuria and outcome was observed in the study. In univariate analysis of renal survival in relation to 24 h urine protein <3 g when compared to >3 g was found significant but no association was found in multivariate analysis.

Renal failure was present in all the patients at the time of recruitment into the study. About three-fourths (80%) of patients with complete remission had serum creatinine <5.5 mg/dL at presentation (P = 0.001). However, all the nonresponders or those who have died had a severe renal failure (serum creatinine >5.5 mg/dL) on admission (P = 0.003). Similar results were found in a study by Levy et al.[20]

One-third (32%) of non-responders shows sclerosis in >35% of their glomeruli, suggesting a marker of advance disease (P >0.05). The percentage of fibrocellular and fibrous crescents was found more in nonresponders as well as in the mortality group. No specific type of crescent, when analyzed for primary outcomes and mortality was found to show any significant association (P >0.05).

Interstitial fibrosis and tubular atrophy are important predictors of chronicity and correlates with adverse renal and patient outcomes. One-third of nonresponders and two-third who have died had significant interstitial fibrosis and tubular atrophy on microscopic examination (P <0.05). Similar results were found in study by Levy et al.[20] In patients with immune complex-mediated RPGN 30% achieved remission during the study. Yu et al in their study found complete remission in 24.2%, partial remission in 48.5% and treatment failure in 27.3% of patients.[21] Better outcomes in their patients are because of presentation with less severe renal disease (mean creatinine = 3.74 ± 2.68 mg/dL) as compared to our patients with severe renal failure. Sumethkul et al in their study in 32 patients with crescentic GN reported remission in 40% of patients. Sixty percent failed to respond out of which seven patients died at three years’ follow-up.[22] The outcome was affected in pauci-immune RPGN by type of ANCA, presence in combination (MPO+PR3) and absence of ANCA. The remission rate in pauci-immune RPGN in the present study was 78.5%, which included MPO-ANCA (55%), PR3-ANCA (45%). No response to treatment was observed in dual positive (MPO+PR3) ANCA and those with ANCA-negative pauciimmune RPGN (P = 0.018).

Chen et al in their retrospective study found poor survival in ANCA-negative patients then in ANCA-positive patients (P <0.05) as seen in our study.[23]

Anti-GBM disease, in our study was reported in seven patients. Four out of seven were in combination with ANCA (MPO in three and PR3 in one). All of them were nonresponders and contributed half of the mortality in the study. Prabhakar et al in a case series of 17 patients with anti-GBM disease with associated ANCA positivity reported remission in four patients (nondialysis dependent at admission) and rest of the patients had poor treatment outcome.[18] In our study all patients were dialysis dependent.

More than half (55%) of patients had infections during follow-up. Pneumonia was the most common (40%) source of infection in our study followed by septicemia (27%), UTI (13.6%), cellulitis (9%) and TB (9%). Neutropenia was observed in about one-third (40%) of patients. Pagnoux et al in a prospective multicentric trial reported infections were 24%.[24]

Ten patients (25%) in the present study died during follow-up. Infections were the common cause of mortality, observed in 50% of the patients died, followed by cardiovascular causes in 30%. The remaining patients died of hemorrhagic stroke (10%) and unexplained (10%). A significant correlation of infection with mortality was seen (P <0.05). Similar to our study, Li et al reported mortality in almost one-fourth of patients (23%) and cause of death was severe infection in half of them (48.8%).[17] On comparing various clinical, biochemical, and histological variables amongst survivors and nonsurvivors, only requirement of RRT on admission correlates with mortality (P <0.05).

In conclusion, results of our study show a male preponderance and increase incidence in the elderly population. Pauci-immune RPGN being the commonest cause followed by immune complex-mediated RPGN and anti-GBM disease. Most of the patients reached late to us affecting the outcome thus an early diagnosis and treatment would help in saving many lives in patients with RPGN.

Conflict of interest: None declared.



 
   References Top

1.
Couser WG. Rapidly progressive glomerulonephritis: Classification, pathogenetic mechanisms, and therapy. Am J Kidney Dis 1988; 11:449-64.  Back to cited text no. 1
    
2.
Jennette JC. Rapidly progressive crescentic glomerulonephritis. Kidney Int 2003;63:1164-77.  Back to cited text no. 2
    
3.
Dewan D, Gulati S, Sharma RK, et al. Clinical spectrum and outcome of crescentic glomerulonephritis in children in developing countries. Pediatr Nephrol 2008;23:389-94.  Back to cited text no. 3
    
4.
Habib MA, Badruddoza SM. Pattern of glomerular diseases among adults in Rajshahi, the Northern Region of Bangladesh. Saudi J Kidney Dis Transpl 2012;23:876-80.  Back to cited text no. 4
  [Full text]  
5.
Panichi V, Pasquariello A, Innocenti M, et al. The Pisa experience of renal biopsies, 1977-2005. J Nephrol 2007;20:329-35.  Back to cited text no. 5
    
6.
Oudah N, Al Duhailib Z, Alsaad K, et al. Glomerulonephritis with crescents among adult Saudi patients outcome and its predictors. Clin Exp Med 2012;12:121-5.  Back to cited text no. 6
    
7.
Choudhury TA, Singh RG, Usha, et al. Clinicopathologic spectrum of crescentic glomerulonephritis: A hospital-based study. Saudi J Kidney Dis Transpl 2014;25:689-96.  Back to cited text no. 7
[PUBMED]  [Full text]  
8.
Gupta R, Singh L, Sharma A, Bagga A, Agarwal SK, Dinda AK. Crescentic glomerulonephritis: A clinical and histomorphological analysis of 46 cases. Indian J Pathol Microbiol 2011;54:497-500.  Back to cited text no. 8
[PUBMED]  [Full text]  
9.
Sinha A, Puri K, Hari P, Dinda AK, Bagga A. Etiology and outcome of crescentic glomerulonephritis. Indian Pediatr 2013;50:283-8.  Back to cited text no. 9
    
10.
Lv J, Yang Y, Zhang H, et al. Prediction of outcomes in crescentic IgA nephropathy in a multicenter cohort study. J Am Soc Nephrol 2013;24:2118-25.  Back to cited text no. 10
    
11.
de Lind van Wijngaarden RA, Hauer HA, Wolterbeek R, et al. Clinical and histologic determinants of renal outcome in ANCA-associated vasculitis: A prospective analysis of 100 patients with severe renal involvement. J Am Soc Nephrol 2006;17:2264-74.  Back to cited text no. 11
    
12.
Fischer EG, Lager DJ. Anti-glomerular basement membrane glomerulonephritis: A morphologic study of 80 cases. Am J Clin Pathol 2006;125:445-50.  Back to cited text no. 12
    
13.
Naidu GS, Sharma A, Nada R, et al. Histopathological classification of pauciimmune glomerulonephritis and its impact on outcome. Rheumatol Int 2014;34:1721-7.  Back to cited text no. 13
    
14.
Tang Z, Wang Z, Zhang HT, et al. Clinical features and renal outcome in lupus patients with diffuse crescentic glomerulonephritis. Rheumatol Int 2009;30:45-9.  Back to cited text no. 14
    
15.
Tang Z, Wu Y, Yao XD, Hu WX, Chen HP, Li SL. The clinical and pathological characteristics of Chinese patients with pauciimmune crescentic glomerulonephritis. Nephrology 2000;5:155-9.  Back to cited text no. 15
    
16.
Rampelli SK, Rajesh NG, Srinivas BH, Harichandra Kumar KT, Swaminathan RP, Priyamvada PS. Clinical spectrum and outcomes of crescentic glomerulonephritis: A single center experience. Indian J Nephrol 2016;26:252-6.  Back to cited text no. 16
[PUBMED]  [Full text]  
17.
Li ZY, Gou SJ, Chen M, Zhao MH. Predictors for outcomes in patients with severe ANCA-associated glomerulonephritis who were dialysis-dependent at presentation: A study of 89 cases in a single Chinese center. Semin Arthritis Rheum 2013;42:515-21.  Back to cited text no. 17
    
18.
Prabhakar D, Rathi M, Nada R, et al. Antiglomerular basement membrane disease: Case series from a tertiary center in North India. Indian J Nephrol 2017;27:108-12.  Back to cited text no. 18
[PUBMED]  [Full text]  
19.
Kapitsinou PP, Ioannidis JP, Boletis JN, et al. Clinicopathologic predictors of death and ESRD in patients with pauci-immune necrotizing glomerulonephritis. Am J Kidney Dis 2003;41:29-37.  Back to cited text no. 19
    
20.
Levy JB, Turner AN, Rees AJ, Pusey CD. Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression. Ann Intern Med 2001;134:1033-42.  Back to cited text no. 20
    
21.
Yu F, Tan Y, Liu G, Wang SX, Zou WZ, Zhao MH. Clinicopathological characteristics and outcomes of patients with crescentic lupus nephritis. Kidney Int 2009;76:307-17.  Back to cited text no. 21
    
22.
Sumethkul V, Chalermsanyakorn P, Changsirikulchai S, Radinahamed P. Lupus nephritis: A challenging cause of rapidly progressive crescentic glomerulonephritis. Lupus 2000;9:424-8.  Back to cited text no. 22
    
23.
Chen M, Yu F, Wang SX, Zou WZ, Zhao MH, Wang HY. Antineutrophil cytoplasmic autoantibody-negative Pauci-immune crescentic glomerulonephritis. J Am Soc Nephrol 2007;18: 599-605.  Back to cited text no. 23
    
24.
Pagnoux C, Mahr A, Hamidou MA, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med 2008;359:2790-803.  Back to cited text no. 24
    

Top
Correspondence Address:
Preet Mohinder Sohal
Department of Nephrology, Dayanand Medical College and Hospital, Ludhiana, Punjab
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1319-2442.367805

Rights and Permissions



 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6], [Table 7], [Table 8]



 

Top
   
 
 
    Similar in PUBMED
    Search Pubmed for
    Search in Google Scholar for
    Email Alert *
    Add to My List *
* Registration required (free)  
 


 
    Abstract
   Introduction
    Materials and Me...
   Statistical Analysis
   Results
   Discussion
    References
    Article Tables
 

 Article Access Statistics
    Viewed130    
    Printed0    
    Emailed0    
    PDF Downloaded29    
    Comments [Add]    

Recommend this journal