Steroid resistant minimal change nephrotic syndrome

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    The effect of B-cell depletion on T-cell function is unknown, but appears to be minimal. However, one study demonstrated increased numbers of T-regulatory cells after rituximab therapy in SLE patients [ 17 ]. Thus, the alleged permeability factor responsible for NS in some patients could be produced by B-cells or T-cells through pathways regulated or stimulated by B-cells. The former seems likely since Gilbert et al. [ 18 ] recently reported using rituximab to treat a child with steroid-dependent MCNS with resulting long-term remission, despite failure of multiple other therapies. The child remained in remission, while the CD19 (B-cell) counts were undetectable, but relapsed after 9 months when CD19 (+) cells returned. Francois et al. [ 10 ] demonstrated the efficacy of rituximab in a patient with MCNS diagnosed at age 6 and treated with rituximab at age 30 after frequent relapses and treatment failures with other agents. Thus, chronic childhood MCNS persisting into the adult-years may respond to rituximab and sustained remissions with rituximab are possible despite long-term disease and treatment failures. This article and our case suggest that B-cells play an important role in the pathogenesis of MCNS and represent a reasonable therapeutic target. This is in contrast to the long-held ideas of the primacy of T-cells in the mediation of MCNS [ 16 , 19 ]. Clearly, our findings and those of others suggests that B-cells and/or their products may be central to the pathogenesis of MCNS [ 18–24 ]. Table 1 represents a summary of the published case reports of the use of rituximab in MCNS and FSGS, usually steroid-resistant lesions. These reports, although uncontrolled, show that patients may have dramatic and sustained responses with complete or partial remissions to rituximab alone, despite prolonged courses of steroids, cytotoxic drugs, MMF and calcineurin inhibitors without good response prior to initiation of rituximab.

    In normal controls synaptopodin immunoexpression was seen in podocytes along the glomerular basement membrane in a finely linear pattern. No changes were found in synaptopodin immunoexpression in steroid-responsive MCD versus controls. In patients with steroid-resistant MCD and FSGS a granular pattern of synaptopodin immunoexpression was seen. Areas of sclerosis in patients with FSGS did not demonstrate synaptopodin expression. Statistical analysis showed significantly diminished synaptopodin immunoexpresion in glomeruli in patients with steroid-resistant MCD and FSGS as compared with steroid-responsive MCD group and controls. Moreover, in renal tissues in patients with FSGS the immunoexpression of synaptopodin was decreased in comparison with renal biopsies in patients with steroid-resistant MCD.

    Steroid resistant minimal change nephrotic syndrome

    steroid resistant minimal change nephrotic syndrome


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