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PubMed This is a summary of 17 peer-reviewed journal articles Updated
Nephrology

Standard of Care: Protecting Your Kidneys

At a Glance

The standard of care for Alport syndrome focuses on protecting kidney function and delaying disease progression using medications like ACE inhibitors. Current guidelines recommend starting treatment early, often at diagnosis or at the first sign of protein in the urine, to maximize kidney protection.

While there is currently no cure for Alport syndrome, the medical community has a powerful, evidence-based strategy to protect the kidneys. The goal of treatment is to “buy time” by slowing the progression of the disease, often by many years or even decades [1][2].

The Foundation: RAAS Blockade

The cornerstone of Alport care is a group of medications that block the Renin-Angiotensin-Aldosterone System (RAAS). These include ACE inhibitors (like lisinopril) and ARBs (like losartan).

These drugs are not just for high blood pressure. In Alport syndrome, they perform two vital jobs:

  1. Lowering Pressure: They reduce the physical pressure inside the kidney’s filters [3].
  2. Reducing Protein Leakage: They help keep protein in the blood where it belongs. Protein leaking into the urine (proteinuria) acts like a toxin that causes kidney scarring. By stopping the leak, these drugs prevent the damage from spreading [4][5].

When to Start: The 2020 Guidelines

Clinical practice changed significantly in 2020. Experts now recommend starting treatment much earlier than they did in the past. The timing depends on your specific genetic “roadmap” [6][7].

Group Recommended Start Time
Males with XLAS At the time of diagnosis, even if urine is normal [6][2].
Males & Females with ARAS At the time of diagnosis [8].
Females with XLAS At the first sign of microalbuminuria (tiny amounts of protein) [6][9].
Patients with ADAS At the first sign of microalbuminuria [9].

Emerging Therapies: SGLT2 Inhibitors

A newer class of drugs called SGLT2 inhibitors (such as dapagliflozin or empagliflozin) is showing great promise. Originally for diabetes, these drugs have been found to protect the kidneys in many types of chronic kidney disease [10][11]. In Alport syndrome, they are often used as an “add-on” to ACE inhibitors to provide an extra layer of protection and further delay the need for a transplant [12][13]. Note that these may be prescribed under broader chronic kidney disease guidelines rather than having an Alport-specific FDA indication.

Day-to-Day Lifestyle Management

Medications are critical, but daily habits also play a huge role in protecting your kidneys. You can actively assist your treatment by:

  • Restricting Sodium (Salt): High salt intake can raise blood pressure and counteract the protective effects of your ACE inhibitors/ARBs.
  • Monitoring Blood Pressure at Home: Tracking your blood pressure between doctor visits can help ensure your medications are working effectively.
  • Staying Hydrated: Drink adequate water, and discuss with your doctor whether any specific dietary protein modifications are necessary based on your current kidney function stage.

Kidney Transplant and a Unique Risk

If the kidneys eventually fail, a transplant is often the best option. Alport patients generally have excellent results with transplants [14]. However, there is a rare but specific complication to be aware of: De Novo Anti-GBM Disease [15].

Because an Alport patient’s body has never seen “normal” Type IV Collagen, it may occasionally view a new, healthy donor kidney as a foreign object and create antibodies against it [16]. This happens in fewer than 3–5% of Alport transplant cases, but it is a reason why close follow-up with a transplant team is essential [15].

A Proactive Approach

The most important thing to remember is that early treatment works. By starting medications as soon as the guidelines recommend and combining them with healthy lifestyle choices, you are taking the most effective steps possible to preserve kidney function for the long term [17][2].

Common questions in this guide

When should treatment for Alport syndrome begin?
Treatment timing depends on your specific genetic subtype. Males with X-linked Alport syndrome and all patients with autosomal recessive Alport syndrome typically start medications at diagnosis, while others may wait until the first sign of protein in the urine.
How do ACE inhibitors help protect kidneys in Alport syndrome?
ACE inhibitors reduce the physical pressure inside the kidney's filters and help prevent protein from leaking into the urine. Stopping this protein leak reduces scarring and slows the progression of kidney damage.
What are SGLT2 inhibitors, and why are they used for Alport syndrome?
SGLT2 inhibitors are a newer class of medications that provide an extra layer of kidney protection. They are often added to a standard treatment plan alongside ACE inhibitors to help further delay the progression of kidney disease.
Are kidney transplants successful for people with Alport syndrome?
Yes, kidney transplants are generally highly successful for Alport patients and are considered a great option if the kidneys fail. However, there is a small risk of developing a rare antibody complication, requiring close monitoring by your transplant team.
What lifestyle changes can help protect my kidneys?
Restricting your daily sodium intake, staying well-hydrated, and monitoring your blood pressure at home are practical ways to support your kidney function and help your prescribed medications work more effectively.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Based on my (or my child's) genetic subtype and current urine results, do the 2020 guidelines recommend starting an ACE inhibitor now or waiting for microalbuminuria?
  2. 2.Are we at the maximum 'nephroprotective' dose of our current medication, or is there room to increase it safely?
  3. 3.Is an SGLT2 inhibitor a good addition to my treatment plan at this stage?
  4. 4.If we are looking toward a transplant, what is our specific risk for de novo anti-GBM disease, and how is it monitored?
  5. 5.What lifestyle and dietary changes (like sodium restriction) do you recommend I make to protect my kidney function?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (17)
  1. 1

    Angiotensin-converting enzyme inhibitors in patients with Alport syndrome: can all patients benefit?

    Rheault MN

    Kidney international 2020; (98(6)):1400-1402 doi:10.1016/j.kint.2020.07.011.

    PMID: 33276866
  2. 2

    Lifelong effect of therapy in young patients with the COL4A5 Alport missense variant p.(Gly624Asp): a prospective cohort study.

    Boeckhaus J, Hoefele J, Riedhammer KM, et al.

    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 2022; (37(12)):2496-2504 doi:10.1093/ndt/gfac006.

    PMID: 35022790
  3. 3

    Renal, auricular, and ocular outcomes of Alport syndrome and their current management.

    Zhang Y, Ding J

    Pediatric nephrology (Berlin, Germany) 2018; (33(8)):1309-1316 doi:10.1007/s00467-017-3784-3.

    PMID: 28864840
  4. 4

    Monotherapy of RAAS blockers and mobilization of aldosterone: A mechanistic perspective study in kidney disease.

    Gupta G, Dahiya R, Singh Y, et al.

    Chemico-biological interactions 2020; (317()):108975 doi:10.1016/j.cbi.2020.108975.

    PMID: 32032593
  5. 5

    RAAS inhibition and the course of Alport syndrome.

    Savva I, Pierides A, Deltas C

    Pharmacological research 2016; (107()):205-210 doi:10.1016/j.phrs.2016.03.017.

    PMID: 26995302
  6. 6

    Clinical practice recommendations for the diagnosis and management of Alport syndrome in children, adolescents, and young adults-an update for 2020.

    Kashtan CE, Gross O

    Pediatric nephrology (Berlin, Germany) 2021; (36(3)):711-719 doi:10.1007/s00467-020-04819-6.

    PMID: 33159213
  7. 7

    Genotype-phenotype correlations influence the response to angiotensin-targeting drugs in Japanese patients with male X-linked Alport syndrome.

    Yamamura T, Horinouchi T, Nagano C, et al.

    Kidney international 2020; (98(6)):1605-1614 doi:10.1016/j.kint.2020.06.038.

    PMID: 32712167
  8. 8

    Alport Syndrome in Women and Girls.

    Savige J, Colville D, Rheault M, et al.

    Clinical journal of the American Society of Nephrology : CJASN 2016; (11(9)):1713-1720 doi:10.2215/CJN.00580116.

    PMID: 27287265
  9. 9

    Alport Syndrome: Clinical Utility of Early Genetic Diagnosis in Children.

    Christodoulaki V, Kosma K, Marinakis NM, et al.

    Genes 2024; (15(8)) doi:10.3390/genes15081016.

    PMID: 39202375
  10. 10

    Impact of primary kidney disease on the effects of empagliflozin in patients with chronic kidney disease: secondary analyses of the EMPA-KIDNEY trial.

    The lancet. Diabetes & endocrinology 2024; (12(1)):51-60 doi:10.1016/S2213-8587(23)00322-4.

    PMID: 38061372
  11. 11

    SGLT2 inhibitors for non-diabetic kidney disease: drugs to treat CKD that also improve glycaemia.

    Fernandez-Fernandez B, Sarafidis P, Kanbay M, et al.

    Clinical kidney journal 2020; (13(5)):728-733 doi:10.1093/ckj/sfaa198.

    PMID: 33123352
  12. 12

    SGLT2 inhibitors - a potential treatment for Alport syndrome.

    Mabillard H, Sayer JA

    Clinical science (London, England : 1979) 2020; (134(4)):379-388 doi:10.1042/CS20191276.

    PMID: 32064497
  13. 13

    Current and Future Therapeutical Options in Alport Syndrome.

    Reiterová J, Tesař V

    International journal of molecular sciences 2023; (24(6)) doi:10.3390/ijms24065522.

    PMID: 36982595
  14. 14

    [Kidney allotransplantation from alive related donor in patients with Alport syndrome].

    Goriaĭnov VA, Kaabak MM, Babenko NN, et al.

    Khirurgiia 2016; 50-54 doi:10.17116/hirurgia2016150-54.

    PMID: 26977611
  15. 15

    Anti-Glomerular Basement Membrane Disease.

    McAdoo SP, Pusey CD

    Clinical journal of the American Society of Nephrology : CJASN 2017; (12(7)):1162-1172 doi:10.2215/CJN.01380217.

    PMID: 28515156
  16. 16

    Anti-glomerular basement membrane vasculitis.

    Ponticelli C, Calatroni M, Moroni G

    Autoimmunity reviews 2023; (22(1)):103212 doi:10.1016/j.autrev.2022.103212.

    PMID: 36252931
  17. 17

    Long-term ACE inhibition in Alport syndrome: are the benefits worth the risks?

    Rheault MN, Smoyer WE

    Kidney international 2020; (97(6)):1104-1106 doi:10.1016/j.kint.2020.01.030.

    PMID: 32444091

This page provides educational information about standard treatments for Alport syndrome. Always consult your nephrologist before starting new medications, changing your dose, or making significant dietary adjustments.

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