ESPN 50th Annual Meeting

ESPN 2017


 
POSSIBLE ROLE OF COMPLEMENT CASCADE IN HSPN/IgAN; A CASE REPORT
AYSE SEDA PINARBASI 2 ISMAIL DURSUN 2 ZUBEYDE GUNDUZ 2 HAKAN POYRAZOGLU 2 HULYA AKGUN 1 AFIG BERDELI 3 RUHAN DUSUNSEL 2

1- ERCIYES UNIVERSITY, FACULTY OF MEDICINE, DEPARTMENT OF PATHOLOGY
2- ERCIYES UNIVERSITY, FACULTY OF MEDICINE, DEPARTMENT OF PEDIATRIC NEPHROLOGY
3- EGE UNIVERSITY, FACULTY OF MEDICINE, LABORATORY OF MOLECULAR MEDICINE
 
Introduction:

Henoch-Schonlein purpura (HSP) is the most common vasculitis in children and skin, joints, gastrointestinal tract and kidneys are involved. Renal involvement, range from asymptomatic microscopic hematuria to severe renal disease, is the main origin of the resulting chronic disease.  It is believed that HSPN is an immune complex nephritis and the activation of the complement system appears to play a major role in the pathophysiology. 

Material and methods:

In this case report, we discuss a patient with HSPN who had a complement system defect and did not give complete response to Eculizumab.

 

Results:

The nine years old girl presented with typical clinical picture of HSPN verified with kidney biopsy. Her complements levels were normal. She was given prednisolone, cyclosporine, AZA, cylophosphamide, PLEX and RTX. However, she did not give reasonable response to immunosuppressive medications. Repeated kidney biopsy showed sclerosis in 17 of 26 glomeruli and cellular crescent in the rest of the glomeruli. So, we decided to start eculizumab with an experience coming from IgA nephritis. Initially, she gave a partial response to Eculizumab. Finally, ESRD was developed. Homozygous mutation in Factor H gene was detected in a study of a complement system. While she was on peritoneal dialysis, she experienced with osteomyelitis on the left foot. She died possibly because of complication of osteomyelitis.

Conclusions:

We detected a mutation in factor H gene showing the activation of the complement system appears to play a major role in etiopathogenesis of HSPN. We think Eculizumab may be a rescue treatment option in children who are unresponsive to conventional treatment if it can be given early period of disease