Diagnosis and Management of Nephrotic Syndrome in Adults

 


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Am Fam Physician. 2016 Mar 15;93(6):479-485.

  Patient information: See related handout on nephrotic syndrome, written by the author of this article.

Author disclosure: No relevant financial affiliations.

Nephrotic syndrome (NS) consists of peripheral edema, heavy proteinuria, and hypoalbuminemia, often with hyperlipidemia. Patients typically present with edema and fatigue, without evidence of heart failure or severe liver disease. The diagnosis of NS is based on typical clinical features with confirmation of heavy proteinuria and hypoalbuminemia. The patient history and selected diagnostic studies rule out important secondary causes, including diabetes mellitus, systemic lupus erythematosus, and medication adverse effects. Most cases of NS are considered idiopathic or primary; membranous nephropathy and focal segmental glomerulosclerosis are the most common histologic subtypes of primary NS in adults. Important complications of NS include venous thrombosis and hyperlipidemia; other potential complications include infection and acute kidney injury. Spontaneous acute kidney injury from NS is rare but can occur as a result of the underlying medical problem. Despite a lack of evidence-based guidelines, treatment consisting of sodium restriction, fluid restriction, loop diuretics, angiotensin-converting enzyme inhibitor or angiotensin receptor blocker therapy, and careful assessment for possible disease complications is appropriate for most patients. Renal biopsy is often recommended, although it may be most useful in patients with suspected underlying systemic lupus erythematosus or other renal disorders, in whom biopsy can guide management and prognosis. Immunosuppressive treatment, including corticosteroids, is often used for NS, although evidence is lacking. Routine prophylactic treatment to prevent infection or thrombosis is not recommended. A nephrologist should be consulted about use of anticoagulation and immunosuppressants, need for renal biopsy, and for other areas of uncertainty.

Nephrotic syndrome (NS) consists of peripheral edema, heavy proteinuria, and hypoalbuminemia, often with hyperlipidemia. Patients typically present with edema and fatigue, without heart failure or severe liver disease. Although there is limited evidence to guide management decisions, recent expert consensus guidelines and systematic reviews provide updated recommendations. This article focuses on diagnosis and management of NS in adults, which is different from that in children.

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SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingReferences

Spot urine protein-to-creatinine ratio should be used instead of 24-hour urine collection to confirm nephrotic-range proteinuria.

C

1

Although venous thrombosis is a common complication of NS, there is no evidence that anticoagulation is indicated in all patients with NS.

C

6, 7, 13

Although hyperlipidemia is a common complication of NS, there is no evidence that lipid-lowering therapy should be initiated solely to treat the manifestations of NS.

A

10

Therapy with sodium restriction, fluid restriction, loop diuretics, and angiotensin-converting enzyme inhibitors or angiotensin receptor blockers is a conservative management approach appropriate for most patients with NS.

C

1, 3

Corticosteroids and other immunosuppressant drugs may have some benefit in patients with NS, but the potential risks are significant and there is no evidence or guideline recommending use of these drugs in all patients.

B

15


NS = nephrotic syndrome.

A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to http://www.aafp.org/afpsort.

SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingReferences

Spot urine protein-to-creatinine ratio should be used instead of 24-hour urine collection to confirm nephrotic-range proteinuria.

C

1

Although venous thrombosis is a common complication of NS, there is no evidence that anticoagulation is indicated in all patients with NS.

C

6, 7, 13

Although hyperlipidemia is a common complication of NS, there is no evidence that lipid-lowering therapy should be initiated solely to treat the manifestations of NS.

A

10

Therapy with sodium restriction, fluid restriction, loop diuretics, and angiotensin-converting enzyme inhibitors or angiotensin receptor blockers is a conservative management approach appropriate for most patients with NS.

C

1, 3

Corticosteroids and other immunosuppressant drugs may have some benefit in patients with NS, but the potential risks are significant and there is no evidence or guideline recommending use of these drugs in all patients.

B

15


NS = nephrotic syndrome.

A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to http://www.aafp.org/afpsort.

Epidemiology

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The Author

CHARLES KODNER, MD, is an associate professor in the Department of Family and Geriatric Medicine at the University of Louisville (Ky.) School of Medicine.

Address correspondence to Charles Kodner, MD, University of Louisville School of Medicine, Med Center One Bldg., Louisville, KY 40292. Reprints are not available from the author.

Author disclosure: No relevant financial affiliations.

REFERENCES

show all references

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