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Abstract: FR-PO903

Study of Anti-Complement Factor H Mediated Disease at a Tertiary Care Centre

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Author

  • Lingaraj, Umesh, Institute of NeohroUrology, Bangalore, Karnataka, India
Background

Complement dysregulation is an important aetiology for glomerular diseases. Antibody against complement factor H which regulates the alternate complement pathway can cause atypical HUS and C3 glomerulopathy1.
Aim of the study :
To study the clinical profile and outcome of patients with anti complement factor H mediated disease at our centre.

Methods

Materials and methods :
We studied the clinical profile and outcome of patients with anti complement factor H mediated disease at a tertiary care centre over 24 months ( August 2016 to July 2018). We had a total of 18 cases during the study period. All patients were followed up to assess their response to therapy.

Results

A total of 28 cases of atypical HUS were seen during the study period of which anti factor H antibody was elevated in 18 (64.2%). Mean age of the patients was 26.6 +/- 3.2 yrs with 10 patients in the paediatric age group. There were 13 males (72.2%). 10 patients had a febrile prodrome (55.5%). All patients presented with hypertension with active urinary sediments and rapidly progressive renal failure. Mean serum creatinine at presentation was 6.8 +/- 1.2 mg/dl and all patients were oliguric at presentation and required haemodialysis. Serum C3 was low in all patients with a mean of 68 +/- 12.2 mg/dl with normal C4 levels. LDH was elevated in all patients with a mean of 2878 +/- 211.4 IU/ml. All patients had schistocytes in peripheral smear. Anti complement factor H antibody was elevated in all patients with a mean of 549 +/- 90 AU/ml. (normal - 0 to 100 AU/ml).Renal biopsy showed thrombotic microangiopathy in 12 patients (66.6%) while features were suggestive of C3 glomerulopathy in 6 patients (33.3%).

Conclusion

Our study shows that anti complement factor H mediated disease shows good response to plasmapheresis followed by immunosuppression with B cell targeted therapy. Anti CFH mediated disease should be ruled out in all patients with atypical HUS. It is more common in the paediatric age group with excellent response to plasmapheresis and immunosuppression in the form of oral steroids and cyclophosphamide. Also patients presenting with TMA have better prognosis compared to C3 glomerulopathy. Identification of the pathological clone of cells producing the anti factor H antibody would provide more insight into the nature of the disease. We also hypothesise that the clones producing antibody to the N and C terminals might be different.