23 year old female patient presented with severe nephrotic syndrome as a result of steroid resistant recurrent FSGS. She relapsed 4 times over the past 10 years. She has received maximum dose of cyclophosphamide and was resistant each time to steroids and CNI but had marginal response to MMF. The only drug that put her in remission 3 years ago was Rituximab. Now, her immunoglobulin profile is quite low with IgG
ACTH could be tried. Some patients with steroid-dependent or steroid-resistant FSGS may respond to ACTH therapy with a total response rate of 30-35%. In Europe we have a syntethic analogue of ACTH (Synachten), we administer 1 or 2 grams a week for at least six months. Otherwise plasmapheresis should be performed in conjunction with immunosuppression therapy (in this case with MMF), hoping for a synergistic effect in an attempt to remove permeability factors.
Rituximab could be worthy, as it worked in the past. Low IgG is probably due to heavy proteinuria, not to persistant hypogammaglobulinemia induced by rituximab. It depends on how many doses she had received previously, maybe it is a good idea to minimize the number of infusions, due to lack of long-term therapy experience.
Appart from what has been commented previously, lipid apheresis is a promising therapy (associated with corticosteroids and immunosupressants).
Treatment with oral galactose has also been effective in some patients.
Good luck!
in my experience in relapsed FSGS, treatment is not beneficial. rituximab is used only in steroid dependent FSGS not in steroid resistance cases. gene profile is not valuable in adult nephrology. it is not a guide for treatment so far. I think - and apply for my patients- life style modification, ACEI or ARB and other mild immunosupressive drug are enough and wait for ESRD... unfortunatly
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