Abstract :
Background: Crescentic Glomerulonephritis (CGN) is produced by many factors like Antineutrophilic
Cytoplasmic Antibody (ANCA), Anti Glomerular Basement Membrane Antibodies (Anti GBM Ab) or
immune complexes (IC). Aim of the study was to see prevalence of ANCA in CGN and subtypes of CGN.
Methods: Total 50 cases of CGN were studied from February 2016 to July 2017. All auto antibodies
(ANCA, ANA, DNA) were performed by ELISA method. Formalin fixed, paraffin embedded tissue was
used for histopathology.
Result: In 50 cases of CGN (26 males, 24 females) age range was 13 – 86 years. ANCA was positive
in 72%. About 60% cases were of Type III (Pauci-immune) followed by Type IV (combined type 1 and
type 3), Type V (ANCA, immune-complex and anti GBM negative) (12% each), Type I (Anti GBM Ab
mediated) and Type II (immune complex mediated) (8% each).
About 14% patients were below 14 years of age, 52% were between 21 to 40 years, 24% were between 41
to 60 years and 10% were above 61 years of age.
In Type III CGN, 53.33% were MPO positive, 40% were PR3 positive and 6.7% were both MPO and PR3
positive, while in Type IV CGN, 83.33% were MPO positive and 16.66% were PR3 positive. Small vessel
vasculitis found in 83.3% of the patients of Type V, 69.9% patients of Type III and 50% of patients of Type
I and Type IV and 25% of patients of Type II CGN. Vasculitis of medium sized blood vessels was seen in
50% of Type IV, 20% of Type III CGN, 25% of Type I and 16.7% of Type V CGN.
About 41 cases of CGN, suspected highly for small vessel vasculitis, were subjected for CD34 staining. In
H&E stain, only 58% of the cases showed vasculitis and peritubular capillaritis; but after CD 34 staining,
all cases showed arteriolitis and peritubular capillaritis. About 33.33% of Type IV and 26.6% of Type III
had granulomas in glomeruli; while 26.6% of Type III, 25% of Type I and IV each, 16% of Type IV and
V each had granulomas in interstitium. Two cases (66.6%) of Type III had granulomas in blood vessels.
Out of 50 cases of CGN, 14 cases (28%) after investigations turned out to be cases of Lupus nephritis with
ANCA positivity in 85.5% of the cases. MPO ANCA was positive in 50%, PR3 ANCA in 28.57% and in
one case both ANCA and anti GBM Ab were positive.
Granulomatous Polyangitis (GPA) was seen in 8 cases while Eosinophilic Granulomatous Polyangitis
(EGPA) was found in one case and rest of the cases were of microscopic polyangiitis (MPA). PR3 ANCA
was positive in 87.5% cases of GPA, whereas in EGPA, MPO ANCA was positive.
Conclusion: SLE can also be associated with ANCA mediated CGN. ANCA produces small vessel
vasculitis which can be better visualised after CD34 staining. Detection of ANCA will help the clinicians
for planning aggressive immunosuppressive therapy.
Keyword :
SLE, Crescentic GN, Rapidly progressive GN, Vasculitis, Granulomatous polyangitis, Eosinophilic granulomatous
polyangitis.