ESPN 50th Annual Meeting

ESPN 2017


 
Severe acute postinfectious glomerulonephritis: is there a place for Eculizumab?
MARC FILA 1 JULIE TENENBAUM 1 LYDIA ICHAY 1 CYRIL AMOUROUX 1 DENIS MORIN 1

1- CHU ARNAUD DE VILLENEUVE -MONTPELLIER
 
Introduction:

Postinfectious glomerulonephritis (PIGN) represents the most frequent cause of acute glomerulonephritis in childhood. The outcome is good in the majority of cases but, occasionally, PIGN could lead to ESRD. Activation of classical and alternative complement pathway in PIGN has been clearly proved. Eculizumab could be considered as a possible treatment in severe cases of PIGN.

Material and methods:

A 12 years old girl was admitted in ICU for seizures. Clinical examination found a severe hypertension, moderate edema and macroscopic hematuria. Laboratory exams revealed an acute renal failure (SCr 227µmol/l, urea 23mmol/l) with a nephrotic range proteinuria (985mg/mmol); isolated low level of C3 (0.12g/l). Cerebral MRI revealed lesions corresponding to a posterior reversible encephalopathy syndrome. Diagnosis of PIGN with neurological impairment was made. Supportive care were started and initial evolution was good with an improvement of glomerular filtration rate (GFR).

Results:

At day 7, the patient developed an oliguria and SCr level raised up to 400µmol/l. Kidney biopsy showed 10/18 sclerosed glomeruli. A moderate mesangial hypercellularity was present in the 8 others without any crescent formation. A moderate interstitial inflammation was also seen. Immunofluorescence study revealed a strong C3 and C5b-9 staining in the mesangium and in capillary walls without IgG deposits. sC5b9 was increased (571ng/ml); C3 Nephritic factor was negative. Treatment with steroids pulse was started without any glomerular filtration rate improvement. According to these results the diagnosis of a rapidly progressive C3 glomerulopathy (C3G) was suggested and, due to the severity of renal failure, eculizumab (ECZ) was quickly started. Renal function dramatically improved after the first infusion. At one month, SCr decreased to 100µmol/l. Sc5b9 and C3 level returned to normal range. Genetic complement abnormalities implicated in C3G were screened but no mutation was found. At 6 months, another renal biopsy was made revealing a moderate mesangial hypercellularity without C3 deposits in favour of a late PIGN.

Conclusions:

Atypical PIGN can be mistaked with a C3G. It has been demonstated that ECZ can be efficient in C3G. But ECZ should be considered as therapy in a severe form PIGN with a pathologic overactivation of complement alternative pathway.