ESPN 51th Annual Meeting

ESPN 2018


 
Severe IgA nephropathy presenting as acute thrombotic microangiopathy (TMA)
MARGAUX LHOSTE 1 JEAN-DANIEL DELBET 1 TIM ULINSKI 1

1- TROUSSEAU HOSPITAL
 
Introduction:

Here we report the case of a 13-year-old boy with TMA revealing severe IgA nephropathy.

Material and methods:

We report a 13 year-old adolescent boy with African origin, who presented first at our emergency department with vomiting, abdominal pain, and asthenia. His blood pressure was 155/110 mmHg and severe renal failure with serum creatinine 1 498 µmol/l, BUN 42.6 mmol/l, Hb 5.1 g/dl, LDH 1352, low haptoglobine, schistocytes 4%, reticulocytes 140.000/ml, platelets 77.000/ ml.

Results:

Initially, hemolytic uremic syndrome was suspected. There was no history of diarrhea. As soon as blood pressure was rapidly controlled using an intravenous calcium inhibitor, switched to oral amlodipine after 2 days, symptoms decreased and TMA related blood parameters improved and  normalized about 7 to 10 days later. Serum complement and ADAMTS13 analysis were normal.

Hemodialysis was started immediately via a femoral catheter, later replaced by a jugular vein catheter and AV-fistula. A renal biopsy was in favor for IgA nephropathy with a very advanced glomerular sclerosis with TMA lesions and 90% interstitial fibrosis. 

Our patient did not have any episode of macroscopic hematuria before presentation. It is possible that TMA has accelerated the development towards ESRD in our patient.

Conclusions:

TMA in IgA nephropathy patients has been described in adults. Arterial hypertension seems to be present in the majority of cases. The association of IgA nephropathy and arterial hypertension may be of particular risk for TMA.

Thrombotic microangiopathy may occur in children with IgA nephropathy and presumably accelerates the development towards ESRD .