Advertisment
Indian Journal of Nephrology About us |  Subscription |  e-Alerts  | Feedback | Login   
  Print this page Email this page   Small font sizeDefault font sizeIncrease font size
 Home | Current Issue | Archives| Ahead of print | Search |Instructions |  Editorial Board  

Users Online:626

Official publication of the Indian Society of Nephrology
 ~   Next article
 ~   Previous article
 ~   Table of Contents

 ~   Similar in PUBMED
 ~  Search Pubmed for
 ~  Search in Google Scholar for
 ~Related articles
 ~   Citation Manager
 ~   Access Statistics
 ~   Reader Comments
 ~   Email Alert *
 ~   Add to My List *
 * Requires registration (Free)
 

 Article Access Statistics
    Viewed4166    
    Printed160    
    Emailed3    
    PDF Downloaded282    
    Comments [Add]    
    Cited by others 5    

Recommend this journal

 

 CASE REPORT
Year : 2010  |  Volume : 20  |  Issue : 1  |  Page : 43-47

ANCA negative pauci-immune glomerulonephritis with systemic involvement


Department of Nephrology, Meenakshi Mission Hospital and Research Centre, Madurai - 625 107, India

Correspondence Address:
K Sampathkumar
Meenakshi Mission Hospital and Research Centre, Melur Road, Madurai - 625 107
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-4065.62096

Rights and Permissions

Systemic vasculitides (SV) are a group of diseases with multi system involvement and varied clinical presentation. Anti-neutrophil cytoplasmic antibody (ANCA) testing has high sensitivity and specificity for SV. We describe the clinical course of four patients who had pauci-immune glomerulonephritis with systemic involvement without serological ANCA positivity; they were followed up for a cumulative 55 patient months. The mean Birmingham vasculitis score score was 23. All four had systemic symptoms with arthralgias and fever (100%). Neurological manifestations were seen in two patients (66%). Accelerated hypertension was seen in one. One patient had pulmonary renal syndrome. Renal manifestation was characterized by nephrotic range of proteinuria with glomerular hematuria in all (100%) and severe renal failure requiring dialysis in three (66%). At admission the mean blood urea was 146 ± 19 mg% and mean serum creatinine was 5.6 ± 1.9 mg%. Renal biopsy revealed focal proliferative glomerulonephritis with crescents only in 20-30% of glomeruli. There was significant chronic interstitial involvement in two patients (66%). Therapy with pulse steroids, cyclophosphamide, and mycophenolate mofetil (MMF) was effective in three patients while one died with lung hemorrhage. In conclusion, majority of patients with ANCA negative pauci-immune glomerulonephritis have multi-system involvement at admission. Renal biopsy is characterized by focal proliferative lesions with crescents and significant chronic interstitial fibrosis. Immunosuppressive drugs in the form of corticosteroids, MMF and cyclophosphamide bring about marked renal recovery in most patients.






[FULL TEXT] [PDF]*


        
Print this article     Email this article

Indian Journal of Nephrology
Published by Wolters Kluwer - Medknow
Online since 20th Sept '07