Alport Syndrome Medication

Updated: Jul 21, 2015
  • Author: Ramesh Saxena, MD, PhD; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Medication

Medication Summary

Angiotensin-converting enzyme (ACE) inhibitors or angiotensin-receptor blockers (ARBs) should be administered to patients with Alport syndrome who have proteinuria with or without hypertension. Both classes of drugs apparently help to reduce proteinuria by decreasing intraglomerular pressure. Moreover, by inhibiting angiotensin II, a growth factor that is implicated in glomerular sclerosis, these drugs have a potential role in slowing sclerotic progression.

In a randomized study comparing the long-term use of losartan (an ARB) with that of enalapril (an ACE inhibitor) in the treatment of proteinuria in children with Alport syndrome, Webb et al found both drugs to be effective and well tolerated. The investigators studied protein levels and renal function in 27 children with Alport syndrome, following urinary protein ̶ to-creatinine ratios and estimated glomerular filtration rates for up to 3 years. It was found that initial proteinuria decreases in the losartan patients were maintained over the follow-up period, while the enalapril patients not only maintained initial decreases but experienced additional reductions in proteinuria over the 3 years. The incidence of adverse events was low for both sets of patients. [24]

Some small, uncontrolled studies have indicated that cyclosporine may reduce proteinuria and stabilize renal functions in patients with Alport syndrome. Nonetheless, there is also evidence to suggest that patient response to cyclosporine can vary and that the drug may accelerate the development of interstitial fibrosis. [21]

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ACE inhibitors

Class Summary

These agents help to reduce proteinuria by decreasing intraglomerular pressure. Moreover, by inhibiting the conversion of angiotensin I to angiotensin II, a growth factor that is implicated in glomerular sclerosis, these drugs have a potential role in slowing sclerotic progression.

Enalapril (Vasotec)

Enalapril is a competitive inhibitor of ACE. By preventing the conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, it increases levels of plasma renin and reduces aldosterone secretion.

Fosinopril

A competitive inhibitor of ACE, fosinopril reduces angiotensin II levels, decreasing aldosterone secretion.

Lisinopril (Zestril, Prinivil)

A competitive inhibitor of ACE, lisinopril reduces angiotensin II levels, decreasing aldosterone secretion.

Quinapril (Accupril)

A competitive inhibitor of ACE, quinapril reduces angiotensin II levels, decreasing aldosterone secretion.

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Angiotensin-Receptor Blockers (ARBs)

Class Summary

ARBS help to reduce proteinuria by decreasing intraglomerular pressure. As with ACE inhibitors, these drugs inhibit the production of angiotensin II, giving them a potential role in slowing the progression of glomerular sclerosis. Unlike ACE inhibitors, however, ARBs do not activate bradykinin and are less likely to be associated with cough and angioedema.

Losartan (Cozaar)

Losartan can be used in patients who are unable to tolerate ACE inhibitors. It is a nonpeptide angiotensin II receptor antagonist that blocks the vasoconstrictor and aldosterone-secreting effects of angiotensin II. Losartan may induce a more complete inhibition of the renin-angiotensin system than ACE inhibitors do. Unlike ACE inhibitors, it does not affect the response to bradykinin and is less likely to be associated with cough and angioedema.

Candesartan (Atacand)

Candesartan can be used in patients who are unable to tolerate ACE inhibitors. It is a nonpeptide angiotensin II receptor antagonist that blocks the vasoconstrictor and aldosterone-secreting effects of angiotensin II. Candesartan may induce a more complete inhibition of the renin-angiotensin system than ACE inhibitors do. Unlike ACE inhibitors, it does not affect the response to bradykinin and is less likely to be associated with cough and angioedema.

Valsartan (Diovan)

Valsartan is appropriate for patients unable to tolerate ACE inhibitors. It may induce a more complete inhibition of the RAAS than do ACE inhibitors, it does not affect the response to bradykinin, and it is less likely to be associated with cough and angioedema. Compared with ACE inhibitors (eg, captopril, enalapril), losartan is associated with a lower incidence of drug-induced cough, rash, and taste disturbances.

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Immunosuppressive Agents

Class Summary

Cyclosporine may reduce proteinuria and retard the progression of renal disease by inducing afferent arteriolar vasoconstriction, increasing glomerular permselectivity, and inhibiting proinflammatory lymphokines. Efficacy of this treatment in patients was documented only in small series, and further studies are required before this therapy can be recommended on a routine basis. Abstracts suggest that cyclosporine may accelerate the development of interstitial fibrosis. [21, 25] Therefore, such therapy should be approached with caution and close monitoring.

Cyclosporine (Neoral, Sandimmune, Gengraf)

Cyclosporine is a cyclic polypeptide that suppresses some humoral immunity and, to greater extent, cell-mediated immune reactions.

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