Nephrotic Syndrome Treatment & Management

Specific treatment of nephrotic syndrome depends on the disease's cause. These are detailed in the Medscape Reference articles specific to each of these disorders. Treatment also varies somewhat between adult and pediatric patients.

A study using the Cochrane database has put into question whether prednisone treatment is beneficial in adult minimal-change nephropathy.^[32] Nonetheless, when the nephrotic syndrome causes illness, by uncomfortable edema or associated coagulopathy, treatment would appear needed.

The role of preventive anticoagulation in nephrotic syndrome has been reported, but there is no proof that it is beneficial.

Hyperlipidemia occurs in nephrotic syndrome, and it can be controlled with lipid-lowering agents. Older studies have reported a predisposition to atherosclerosis in patients with nephrotic syndrome, but there are no data to show that lipid-lowering drugs improve renal or patient outcomes.

Specific treatment

Specific treatment of nephrotic syndrome depends on the disease's cause. In minimal-change nephropathy, glucocorticosteroids, such as prednisone, are used.^[33] Children who relapse after successful use of prednisone or who do not respond to prednisone (ie, those with steroid-resistant disease) may be treated with rituximab, an antibody against B-cells. Rituximab has also been used in membranous nephropathy in adults.^[34]

In some forms of lupus nephritis, prednisone and cyclophosphamide are useful.

Secondary amyloidosis with nephrotic syndrome may respond to anti-inflammatory treatment of the primary disease.

In membranous nephropathy, expectant management without immunosuppression can be used for the first 6 months, in patients at low risk for progression (ie, those with serum creatinine level < 1.5 mg/dL). Patients with renal insufficiency (serum creatinine level > 1.5 mg/dL) are at greatest risk for the development of end-stage renal disease and should receive immunosuppressive therapy.^[35]

Diet and activity

The diet in patients with nephrotic syndrome should provide adequate energy (caloric) intake and adequate protein (1-2 g/kg/d). Supplemental dietary protein is of no proven value. A diet with no added salt will help to limit fluid overload.

Management of hyperlipidemia could be of some importance if the nephrotic state is prolonged. Fluid restriction per se is not required.

There are no activity restrictions for patients with nephrotic syndrome. Ongoing activity, rather than bedrest, will reduce the risk of blood clots.

With good parental and patient education and close outpatient follow-up care, hospitalization is not usually necessary. Hospitalization should be considered if any of the following are present:

• Generalized edema severe enough to cause respiratory distress
• Tense scrotal or labial edema
• Complications (eg, bacterial sepsis, peritonitis, pneumonia, thromboembolism
• Failure to thrive
• Uncertainty regarding patient or family compliance with treatment

Diuretics are needed. Furosemide (1 mg/kg/d) and spironolactone (2 mg/kg/d) will help when fluid retention is severe, provided no signs of renal failure or volume contraction are evident.

Achieving a satisfactory diuresis is difficult when the patient's serum albumin level is less than 1.5 g/dL. Albumin in a dose of 1 g/kg may be given, followed by intravenous furosemide. Complications may occur, including pulmonary edema.

Some evidence suggests that administration of albumin may delay the response to steroids and may even induce more frequent relapses, probably by causing severe glomerular epithelial damage. Fluid removal and weight loss remain transient unless proteinuria remits.

To prevent infection, oral penicillin can be prescribed for children with gross edema. Abdominal paracentesis should be performed if the patient develops signs of peritonitis, and any bacterial infection should be treated promptly. A nonimmune patient with varicella should receive zoster immunoglobulin therapy if exposed to chickenpox, and acyclovir should be given if the patient develops chickenpox.

Depending on the cause of nephrotic syndrome, a patient may need specialty consultation. For example, an individual with lupus nephritis may benefit from rheumatologic consultation.

The principles for management of adults with acute nephrotic syndrome are similar to those for children. Diuretics will be needed; furosemide, spironolactone, and even metolazone may be used. Volume depletion may occur with diuretic use, which should be monitored by assessment of symptoms, weight, pulse, and blood pressure.

Anticoagulation has been advocated by some for use in preventing thromboembolic complications, but its use in primary prevention is of unproven value.

Hypolipidemic agents may be used, but if the nephrotic syndrome cannot be controlled, the patient will have persistent hyperlipidemia.

In secondary nephrotic syndrome, such as that associated with diabetic nephropathy, angiotensin-converting enzyme (ACE) inhibitors and/or angiotensin II receptor blockers are widely used. These may reduce proteinuria by reducing the systemic blood pressure, by reducing intraglomerular pressure, and also by direct action on podocytes.

Follow-up care in patients with nephrotic syndrome includes immunization, treatment of relapses of steroid-responsive nephrotic syndromes, monitoring for steroid toxicity, and monitoring of diuretic and angiotensin antagonist regimens.

Routine immunizations should be delayed until the patient is free of relapses and has been off immunosuppression for 3 months. Pneumococcal and influenza vaccines are recommended but are not routinely used, because their efficacy is not established. Children who have received immunosuppressive therapy in the preceding 3 months and are not immune to varicella should receive zoster immunoglobulin if they are exposed to chickenpox or shingles. These patients should also receive acyclovir if they develop chickenpox.

Most patients experience relapses; data suggest relapse rates of 76-97%, with frequent relapse occurring at rates of up to 50%. The first 2 relapses are treated in the same manner as the initial presentation; frequent relapses are treated with a maintenance dose of prednisone at 0.1-0.5 mg/kg on alternate days for 3-6 months, with the drug then tapered.

Monitoring for steroid toxicity every 3 months in the outpatient clinic is necessary to help detect adverse effects and to record growth in children. Supplemental calcium and vitamin D may attenuate bone loss. A yearly checkup is necessary to help detect cataracts.

Ongoing use and adjustment of diuretics and angiotensin antagonists are necessary according to the amount of edema and proteinuria that a patient has. This requires periodic monitoring.