Nephrotic Syndrome: Drug Therapy

Therapeutic targets

  • Flushing out edema (water retention) or ascites (abdominal fluid).
  • Avoidance of complications (albumin deficiency, thrombosis / vascular disease in which a blood clot (thrombus) forms in a blood vessel).
  • Effective treatment of concomitant diseases

Therapy recommendations

  • Treatment of the underlying disease (about 70% has glomerular disease: glomerulonephritis, etc.).
  • Diuresis to flush out edema, saline restriction (< 5 g/d); loop diuretics if necessary also potassium-sparing diuretics.
  • Fluid intake depending on the degree of renal insufficiency (kidney weakness) (see below nutritional recommendations in nephrotic syndrome / fiber – water – stimulants).
  • Compensation of electrolyte losses (blood salts) or avoidance of electrolyte disorders (close-meshed control) and fluid balancing.
  • In hypoproteinemia (reduced concentration of total protein in the blood plasma) if necessary albumin infusion; alimentary protein intake depending on the degree of renal insufficiency (1-0.35 g protein/kg/d).
  • ACE inhibitors (antihypertensives), angiotension II receptor antagonists (sartans) in contraindications.
  • Therapy of concomitant diseases:
  • Prednisolone (glucocorticoids: dosage recommendation of the International Study of Kidney Disease in Children (ISKDC)* of 60 mg prednisone/m 2KOF/day, corresponding to approx. 2 mg prednisone/kgKG and day); after 4 weeks, it becomes apparent whether the disease is steroid-sensitive (= response to glucocorticoid therapy) nephrotic syndrome (SSNS) or steroid-resistant nephrotic syndrome (SRNS) or steroid-resistant nephrotic syndrome (SRNS); further treatment depending on the underlying disease:
  • Anticoagulation (inhibition of blood clotting) with heparin/vitamin K antagonists (VKA) (INR 2.0-3.0) in the case of
    • Prolonged or complicated nephrotic syndrome; or
    • Albumin< 20-25 g/l or
    • Drop in AT III below 70%.
  • Infection therapy (for example, in pneumonia (pneumonia), peritonitis (peritonitis) due tolow IgG levels).
  • Vitamin supplementation (including vitamin D, 30 ug), if necessary also osteoporosis therapy.
  • See also under “Further therapy”.

Classification of response to this standard therapy (see above * ).

Classification Definition
Remission (“response”)
  • Proteinuria <4 mg/m 2KOF and h or
  • Albustix urine test strip negative in morning urine or
  • Trace positive on 3 consecutive days; uProt/uKrea < 0.2 g/g
Full remission
Partial remission
  • Reduction of proteinuria by ≥ 50%, uProt/uKrea 0.2 g/g
Recurrence (“relapse”)
  • Recurrence of proteinuria over 40 mg/m 2KOF and h (over 1 g/m 2KOF and day) or
  • Albustix test strip in morning urine ≥100 mg/dl (++) for 3 consecutive days, uProt/uKrea >2 g/g.
Primary steroid-sensitive NS
(“initial responder”)
  • Remission after therapy with prednisone60 mg/m 2KOF and day for 4 weeks.
Primary steroid-resistant NS
(“initial non-responder”)
  • No remission after therapy with prednisone 60 mg/m2KOF and day for 4 weeks.
Secondary steroid-resistant NS
  • Initially steroid-sensitive NS but no response to standard 4-week relapse therapy with prednisone (60 mg/m 2KOF and day) at subsequent relapses
Infrequent relapses (“infrequently relapsing nephrotic syndrome“).
  • Initial steroid-sensitive nephrotic syndrome with a relapse within 6 months of the end of therapy or
  • Up to 3 relapses within 12 months of the end of therapy.
Frequent relapses
(“frequently relapsing nephrotic syndrome”, FRNS).
  • Initial steroid-sensitive NS with ≥2 relapses within the first 6 months after the end of therapy or
  • ≥ 4 recurrences within 12 months of the end of therapy.
Steroid-dependent nephrotic syndrome ( SDNS).
  • At least 2 consecutive relapses on standard relapse therapy with prednisone or
  • Within 2 weeks of the end of therapy

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