Laboratory Studies

In patients with focal segmental glomerulosclerosis (FSGS), urinalysis reveals large amounts of protein, along with hyaline and broad waxy casts, whereas red blood cell (RBC) casts are generally absent. Broad casts may be observed in patients with advanced cases. Serum creatinine (SCr) concentration or creatinine clearance (CrCl) is usually within reference ranges in early stages.

In patients with idiopathic FSGS, investigational findings for an underlying etiology are generally negative. Such conditions may include the following:

Systemic lupus erythematosus (serum complement C4/C3 levels, antinuclear antibody/anti-DNA titers)
Hepatitis B or C infection
Vasculitis (antineutrophil cytoplasmic antibody titers, serum protein electrophoresis)

In patients thought to have secondary FSGS, obtain HIV antibody, CD4, and viral load studies; serology for hepatitis B and C; and parvovirus testing. Diagnosis of FSGS in morbidly obese patients is by excluding other causes. FSGS can be considered in patients with proteinuria on the basis of nephrotic syndrome; however, in young patients with an absence of RBC casts and negative serologic study findings, definitive diagnosis rests on a kidney biopsy.

Histologic Findings

Kidney biopsy is the most definitive way to establish the diagnosis. The characteristic lesion in FSGS is segmental solidification of the glomerular tuft, usually in the perihilar region and sometimes in the peripheral areas, including the tubular pole. In the affected glomeruli, capillaries are segmentally obliterated by accumulation of acellular matrix and hyaline deposits, along with adhesion to the Bowman capsule. Coarsely granular deposits of IgM and C3 are often found in these areas.

Diffuse foot process fusion occurs, predominantly in the sclerotic segments, although partial effacement may be observed overlying normal-appearing lobules. Many morphologic subsets, such as a cellular variant (endocapillary and extracapillary hypercellularity), a collapsing variant (FSGS with mesangial hypercellularity), and FSGS with tip lesions (localized sclerotic lesions limited to the proximal tubular pole of the glomerulus) have been described (see Overview/Pathophysiology). Whether these diverse lesions reflect different pathogeneses or can account for the differences in the prognosis in patients with FSGS is unclear.

In HIV-associated FSGS, in addition to collapsing glomerular lesions with microcystic dilatation of renal tubules, electron microscopy of the kidney reveals tubuloreticular inclusions in endothelial and mesangial cells, an indirect marker of viral disease.^{[4, 5]}

Ultrasonography

In the early stages of FSGS, ultrasound examination reveals normal or large kidneys with increased echogenicity, suggesting diffuse intrinsic medical renal disease. In patients with advanced renal failure, kidneys are small and shrunken, indicating severe glomerular scarring and interstitial fibrosis. In HIV-associated FSGS, ultrasound generally reveals large echogenic kidneys.

Treatment & Management

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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
Questions & Answers
References

Focal Segmental Glomerulosclerosis Workup

Laboratory Studies

Histologic Findings

Ultrasonography

References

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