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.^[28]

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

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^[37] 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.

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.

Questions

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Author

Sreepada TK Rao, MD, FACP Professor, Department of Medicine, State University of New York Downstate Medical Center

Sreepada TK Rao, MD, FACP is a member of the following medical societies: American Society of Hypertension, International Society of Nephrology, American Society of Nephrology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Eleanor Lederer, MD, FASN Professor of Medicine, Chief, Nephrology Division, Director, Nephrology Training Program, Director, Metabolic Stone Clinic, Kidney Disease Program, University of Louisville School of Medicine; Consulting Staff, Louisville Veterans Affairs Hospital

Eleanor Lederer, MD, FASN is a member of the following medical societies: American Association for the Advancement of Science, American Society for Bone and Mineral Research, American Society of Nephrology, American Society of Transplantation, International Society of Nephrology, Kentucky Medical Association, National Kidney Foundation

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: American Society of Nephrology<br/>Received income in an amount equal to or greater than $250 from: Healthcare Quality Strategies, Inc.

Chief Editor

Vecihi Batuman, MD, FASN Professor of Medicine, Section of Nephrology-Hypertension, Deming Department of Medicine, Tulane University School of Medicine

Vecihi Batuman, MD, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

Additional Contributors

Chike Magnus Nzerue, MD, FACP Professor of Medicine, Associate Dean for Clinical Affairs, Meharry Medical College

Chike Magnus Nzerue, MD, FACP is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Society of Nephrology, National Kidney Foundation

Disclosure: Nothing to disclose.

Acknowledgements

Anjana S Soman, MD Staff Physician, Department of Pathology, Quest Diagnostics

Anjana S Soman, MD is a member of the following medical societies: American Society for Clinical Pathology and College of American Pathologists

Disclosure: Nothing to disclose.

Sections Focal Segmental Glomerulosclerosis
Overview
Presentation
DDx
Workup
Treatment
Medication
Follow-up
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References

Focal Segmental Glomerulosclerosis Follow-up

Further Outpatient Care

Complications

Prognosis

Patient Education

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