Dr Brady Thomson, Dr Audilia Sujana, Dr Hannah Wallace, Dr Nicole Lioufas
1Western Health, , Australia
Biography:
Brady is a second-year advanced trainee in Nephrology at the Royal Melbourne Hospital. She has a keen interest in glomerular diseases and immunopathology.
Background:
Focal segmental glomerulosclerosis (FSGS) is a histopathological pattern of disease representing a podocytopathy with a multitude of aetiologies. Primary FSGS is characterized by sudden onset nephrotic syndrome secondary to diffuse foot process effacement and podocyte injury leading to sclerosis. It has long been postulated primary FSGS occurs due to a circulatory permeability factor causing podocyte injury. Given primary FSGS may be autoantibody mediated there is increasing interest in the role of rituximab, a B-cell depleting antibody in the treatment of the disease. Despite this, there is minimal evidence supporting the use of rituximab as an alternative to high dose glucocorticoids to achieve remission.
Case Report:
We present the case of a female in her 20’s with relapsed primary FSGS on maintenance tacrolimus with a history of steroid-induced hypomania. Given the patient’s past history of steroid-induced hypomania the patient was commenced on Prednisolone 10mg mane with Olanzapine 5mg BD aiming to prevent mood disturbance. Tacrolimus was continued with a target trough level of 4-6 and Rituximab 1000mg IV was administered with a further dose of 1000mg 2 weeks later. The patient subsequently achieved rapid clinical and biochemical improvement with the addition of Rituximab and remains in remission. This case poses rituximab as a potential alternative to high dose glucocorticoids to achieve remission in primary FSGS, particularly in patients with significant glucocorticoid related adverse effects.
Conclusions:
Further studies are required to determine which patients are most likely to benefit from Rituximab administration, however, in the absence of more highly powered studies B-cell depletion therapy with Rituximab may be effective at achieving remission, particularly in patients where high-dose glucocorticoids are contraindicated.