Treatment of Primary FSGS in Adults

Abstract

Over the last 20 years, primary FSGS has emerged as one of the leading causes of idiopathic nephrotic syndrome in adults, particularly among African Americans. In nephrotic patients, progression to ESRD often occurs over the course of 5-10 years, whereas non-nephrotic patients and those entering a remission have an extremely favorable prognosis. As a result, it is in patients who remain persistently nephrotic despite conservative therapy that a more aggressive therapeutic approach is taken. Primary FSGS was once considered an entity nonresponsive to prednisone or immunosuppressive agents, but it has become apparent over the last 20 years that a substantial portion of nephrotic adults with primary FSGS do respond to treatment with a significantly improved prognosis. The recent histologic classification proposed for FSGS has provided additional insights into the prognosis and response to therapy. This article reviews the current knowledge regarding the presentation, prognosis, and therapeutic approach in adults with primary FSGS.

Focal Segmental Glomerulosclerosis

“…These therapies are summarized in Tables 4 and 5 and discussed below. A comprehensive analysis of approaches to steroid-sensitive and -resistant FSGS is available from the Kidney Diseases Improving Global Outcomes initiative, and the reader is referred to this initiative for a detailed and thoughtful discussion (100) as well as recent reviews (13). FSGS may be responsive to therapy with glucocorticoids, although relapses are typical.…”

“…Many other agents have been tested in small trials or reported as case series, and they have been the subject of recent reviews (13,(108)(109)(110)(111). These agents include the following: adalimumab, an anti-TNF mAb (112); pirfenidone, an antifibrotic agent that suppresses TGF-b signaling (113); fresolimumab, an anti-TGF-b mAb (114); pulse steroids plus cyclophosphamide (115); and saquinivir (116).…”

“…Critical is the elimination of other systemic diseases or primary renal diseases that may result in a similar presentation. Many reviews cover various aspects of FSGS and include comprehensive reviews (1)(2)(3)(4), disease mechanisms (5)(6)(7)(8), pediatric disease (9), immunologic aspects (10), treatment in children (11), and treatment in adults (12)(13)(14). We focus here on a practical approach to FSGS assessment on clinical and histopathologic grounds in the context of our current understanding of disease mechanisms and genetics.…”

Focal Segmental Glomerulosclerosis: A Single Center Experience

“…), bu tedavinin yanında steroid tedavisi tek başına veya immünsüpresif ajanlarla kombine (CsA, mikofenolat mofetil, azotiopürin, takrolimus, siklofosfamid vb.) kullanılmaktadır (3,5,7).…”

“…Tedaviye yanıt alınamayan olgularda immünsüpresif tedavi uygulanmaktadır (steroid, kalsinörin inhibitörleri, mikofenolat mofetil vb.). Remisyona giren hastalarda genel sağkalım %90 iken, remisyona girmeyenlerde bu oran %35'in altındadır (5).…”