A recent webinar hosted by NephroPlus, the Atypical HUS India Foundation and the Kidney Warriors Foundation, with support from AstraZeneca, brought together leading kidney specialists to discuss atypical hemolytic uremic syndrome (aHUS). This rare and serious condition affects the blood and kidneys and can be difficult to diagnose. Experts including Dr. Vivekanand Jha, Dr. Arvind Bagga, Dr. Nicole Isbel, Dr. Aditi Sinha, Dr. Manisha Sahay, Dr. Raja Ramchandran, Dr. Sreebhushan Raju, Dr. Sayali Thakare, Dr. Sandhya Suresh and Dr. Arun shared their experiences and offered clear guidance on how care can be improved in India.
Why aHUS Is So Hard to Diagnose
One of the biggest challenges with aHUS is that its early symptoms look similar to many other illnesses. Patients often arrive with anemia, low platelet counts, and kidney problems — symptoms also seen in infections, blood pressure emergencies, and pregnancy-related complications. Because these conditions are far more common, aHUS is often not recognized right away.
Certain signs should raise suspicion: kidney injury that seems too severe for the illness, low platelet counts that do not improve, very high LDH (a marker of cell damage), and damaged red blood cells on the blood smear. Early testing to rule out other conditions, especially TTP, is essential. Once these are ruled out, doctors can consider aHUS sooner instead of waiting for symptoms to worsen.
Early Treatment Makes a Major Difference
Early treatment with complement-blocking medicines, most commonly eculizumab, can dramatically improve outcomes. These medicines stop the part of the immune system that causes blood vessel damage in aHUS. Patients treated earlier are much more likely to recover kidney function and avoid long-term dialysis.
Once treatment begins, older approaches such as plasma exchange should usually be stopped because they can reduce the effectiveness of these medicines. Severe symptoms such as confusion or seizures suggest rapidly progressing disease and may require quicker treatment decisions. Similarly, if a woman develops blood vessel damage after childbirth and does not improve within a few days, complement-blocking therapy may be needed.
The Role and Limits of Genetic Testing
Genetic testing can help explain why a person developed aHUS and whether they are at higher risk of relapse. However, a negative genetic test does not rule it out. Many patients have normal results but still have strong signs of complement system overactivity.
Access to high-quality testing remains limited in India, and sample handling errors can give misleading results. Doctors emphasized that treatment should be guided mainly by clinical judgement rather than waiting for test results.
Anti–Factor H Antibody–Related aHUS
Some patients, especially children in South Asia, develop aHUS because their immune system produces harmful antibodies against a protein called factor H. This causes sudden and severe kidney problems. These patients usually need plasma exchange to remove the antibodies, immunosuppressive medicines to control them, and complement-blocking therapy to stop further damage.
Viral infections can trigger relapses even after improvement, so long-term monitoring is important. Ongoing protein loss in urine may indicate that the kidneys still have ongoing injury.
Why Pregnancy Can Trigger aHUS
Pregnancy-related aHUS is especially challenging because it closely resembles complications like preeclampsia or HELLP syndrome. Repeated severe issues across pregnancies, sudden kidney failure, or very low platelet counts may indicate underlying complement problems. In such cases, complement-blocking treatment may help prevent permanent kidney injury.
Long-Term Care and Relapse Prevention
Long-term follow-up after an aHUS episode is essential. Some patients may eventually stop complement-blocking therapy, while others may need longer treatment depending on their condition. Relapses can occur, especially within the first year after stopping treatment. Patients and families should be aware of warning signs and know when to seek immediate help.
Strengthening aHUS Care in India
Experts highlighted several areas where care can be improved: expanded awareness among regional hospitals, easier access to complement and genetic testing, national registries to study patterns of disease, and establishing Centers of Excellence to guide diagnosis and treatment. Better referral pathways and faster access to complement-blocking medicines could make a major difference in outcomes for patients across the country.
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