ESPN 51th Annual Meeting

ESPN 2018


 
ANCA-ASSOCIATED CRESENTIC GLOMERULONEPHRITIS IN A CHILD WITH ISOLATED RENAL INVOLVEMENT
MEHTAP EZEL ÇELAKIL 1 BURCU BOZKAYA YÜCEL 1 KENAN BEK 1

1- KOCAELI UNIVERSITY FACULTY OF MEDICINE, DEPARTMENT OF PEDIATRIC NEPHROLOGY
 
Introduction:

 Pauci immune glomerulonephritis (GN) is more common in elderly people compared to children. Antineutrophil cytoplasmic antibody (ANCA) positivity occurs in 80% of the patients. 

Material and methods:

 We report a 7-year-old girl who presented with malaise and mildly elevated creatinine diagnosed as ANCA-associated pauci immune cresentic glomerulonephritis with crescents in 20 of 25 glomeruli (%80). Of these 20 crescents, 12 were cellular, 4 fibrocellular and 4 globally sclerotic. She did not have purpura, arthritis or systemic symptoms and she responded well to initial immunosuppressive treatment despite severe histopathology. The patient was given three pulses of intravenous methylprednisolone (30mg/kg on alternate days) initially and continued with cyclophosphamide (CYC; 2mg/kg/day) orally for 3 months with prednisone (1 mg / kg / day). In one month remission was achieved with normal serum creatinine. Prednisone was gradually tapered. Clinical follow-up of the patient continues in remission in the 6th month.

Results:

 We report this child with a relatively rare pediatric disease to emphasize the importance of early and aggressive immunosuppressive treatment in patients with renal-limited disease even if with a mild clinical presentation. 

Conclusions:

 As in our patient; clinical and laboratory findings might not always exactly reflect the severity of renal histopathology and thus kidney biopsy is a must in such children to guide the clinical management and predict the prognosis.