Focal Segmental Glomerulosclerosis Follow-up

Updated: Dec 07, 2020
  • Author: Sreepada TK Rao, MD, FACP; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Follow-up

Further Outpatient Care

Patients receiving long-term corticosteroid therapy must be monitored for adverse effects (eg, infections, hypertension, hyperglycemia). If cyclophosphamide is used, watch for leukopenia and hemorrhagic cystitis. During cyclosporine therapy, monitor renal function. Adjust diuretic doses according to fluid retention. During mycophenolate mofetil therapy, monitor white blood cell count and liver function. Patients with HIV infection will need periodic determination of viral load to assess the effectiveness of their antiretroviral therapy. [27]

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Complications

Complications of prednisone therapy include the following:

  • Infections
  • Hypertension
  • Hyperglycemia

Complications of cyclophosphamide therapy include the following:

  • Infections
  • Leukopenia
  • Hemorrhagic cystitis

Complications of cyclosporine therapy include the following:

  • Renal insufficiency
  • Gingival hyperplasia
  • Infections
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Prognosis

The natural history of focal segmental glomerulosclerosis (FSGS) varies a great deal. Patients with tip lesions generally respond to therapy. The collapsing form of FSGS is marked by severe hypertension, more massive proteinuria, a very poor response to corticosteroids, and a much faster rate of progression to ESRD. A typical course runs from edema that is difficult to manage, to proteinuria that is refractory to corticosteroids [35]  and other immunosuppressive agents, to worsening hypertension and a progressive loss of renal function. In nonresponders, the average time from the onset of proteinuria to end-stage renal disease (ESRD is 6-8 years, although wide variations in the time course occur. The presence of interstitial fibrosis on an initial kidney biopsy specimen is a uniform predictor of poor renal prognosis. Blacks have a worse prognosis than whites.

Spontaneous remissions are extremely rare, although the literature contains isolated case reports.

The level of proteinuria greatly influences the outcome in FSGS. In patients with non-nephritic proteinuria, kidney function remains stable and fewer than 15% progress to ESRD in 10 years. More than 50% of patients with persistent nephritic syndrome develop ESRD in 10 years. In those with massive proteinuria greater than 10-15 g/day, kidney function deteriorates even more rapidly (over 2-3 years).

In the early 1980s, before the introduction of antiretroviral drugs, patients with HIV-associated FSGS typicallydeveloped ESRD in less than a year. With the introduction of HAART, the natural history is now dramatically different, including both prevention of nephropathy and preservation of renal function in those with established disease.

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Patient Education

Educate patients about chronic kidney disease, control of hypertension and lipids, and options for renal replacement therapy, such as peritoneal dialysis, hemodialysis, and kidney transplantation. For further information, see Glomerulosclerosis and the Kidney Disease Directory.

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