How to Treat Refractory Proteinuria for FSGS Patients
How to treat refractory proteinuria for FSGS patients?
It may be a genetic problem that urinary protein can't fall down.
When kidney patients have massive proteinuria, especially those with more than 3.5g/24h proteinuria, steroids are often used to treat them. After a few weeks of steroid application, there is no significant improvement in massive proteinuria, then we call it steroid-resistant Nephrotic Syndrome.
Focal segmental glomerulosclerosis (FSGS) is common in steroid-resistant nephrotic syndrome, a kidney disease closely related to genes.
FSGS is also a kind of refractory nephropathy. There are about 3.6 million FSGS patients in China, of which about 2.1 million can not reduce their urinary protein. Among the 1.5 million people whose urine proteins can drop, half of them relapse frequently. About 80% of patients are refractory proteinuria, and 50% of patients develop uremia 10 years later.
Which FSGS patients need genetic testing?
For FSGS patients with resistance, skin lesions, deafness, neurological abnormalities, eye abnormalities, skeletal abnormalities, young adult onset diabetes mellitus, hepatosplenomegaly, or metabolic acidosis, gene testing is recommended. Especially in patients before 25 years of age, genetic FSGS is more likely. At present, many large hospitals in China can carry out FSGS gene detection.
However, adult FSGS patients without a family history of hereditary nephrotic syndrome, especially those with mixed ethnic groups, are not recommended for genetic testing.
A large cohort study screened 2016 patients with steroid-resistant nephrotic syndrome without family history, and 21% of them inherited in the form of single gene FSGS. Among FSGS patients with family history, the prevalence of single gene FSGS is 30% to 50%. Gene testing in FSGS patients may provide better guidance for clinical treatment, but too many FSGS patients have not been genetically diagnosed.
Genetic FSGS may account for a large proportion of steroid-resistant FSGS patients, especially children.
Gene testing can guide clinical practice and help improve the condition of FSGS.
After the detection of pathogenic genes, some patients who use steroids and immunosuppressive agents ineffective can avoid ineffective treatment and side effects in advance, and can apply targeted therapy based on mutation genes. For example, patients with mutations in COQ6, COQ2 and COQ8B genes may respond to CoQ10 replacement therapy. Patients with WT1, TRPC6 and PLCE1 gene mutations respond to Immunotherapy.
Now you know how to treat refractory proteinuria for FSGS patients. For detailed information on gene testing and FSGS treatment, please leave a message below or contact online doctor.
***Please seek professional medical advise for the diagnosis or treatment of any ailment, disease or medical condition. This article is not intended to be a substitute for the advice of a licensed medical professional.***