Can Berger’s disease that affects the kidneys have an autoimmune origin? – breaking latest news

I suffer from Berger’s disease, discovered when I was 20 years old after an episode of gross haematuria (presence of blood in the urine), a consequence of chickenpox. Is Berger’s an autoimmune disease? Since birth I have suffered from atypical dermatitis and various allergies. I never read news about progress in science on this nephropathy, is it possible that in 2023 a cure has not yet been invented that can free the kidneys from this inflammation?

He answers Arrigo SchieppatiClinical Research Center for Rare Diseases, Mario Negri Institute for Pharmacological Research IRCCS, Bergamo (VAI AL FORUM)

Berger’s disease, whose more appropriate name is «IgA mesangial deposit nephropathy” In short IgA nephropathy (NIgA) can be traced back to the family of autoimmune kidney disease, as an alteration of the immune system plays a role in the mechanism that damages the kidney. This disease occurs when class A immunoglobulins (IgA), which are antibodies produced by the immune system to protect the body from foreign substances such as bacteria or viruses, for an abnormality are deposited in the kidney tissuespecifically in the renal glomeruli, causing a more or less marked inflammatory reaction which results in damage to kidney function. By what mechanism these IgA (which are normally present in everyone) develop an anomaly that determines their ability to do damage has been studied for a long time and much knowledge has been accumulated, although research to precisely define this mechanism is still in progress. course.

Here it is not possible to summarize what has been known up to now and what are the future research projects, which however are concentrated on finding curative therapy for this condition, because just as you say, unlike other autoimmune diseases, there is still no NIgA. To date, attempts to deal with the disease with drugs that directly interfere with the immune system (as occurs in other diseases such as lupus or vasculitis), such as cortisone or other immunosuppressantshave not always guaranteed satisfactory and long-lasting results and have had to deal with significant toxic effects. In the field of immunosuppressive therapy, the most promising clinical research results have so far come from the study of a drug, budesonidewhich is a cortisone that acts in the intestine and is poorly absorbedreducing the risk of side effects.

This drug has been approved by the European Medicines Agency (Ema) and also the Italian one has resolved its approval for use in our country, but for the moment – as far as I know – to date it has placed it in the so-called «band C», you dedicated drugs not yet evaluated for reimbursement purposes, waiting to have more information on efficacy and risk profile. This drug is however indicated in cases of rapidly progressing NIgA with proteinura PC/R >1.5. Meanwhile, the therapy of NIgA can benefit from drugs that are capable of reduce proteinuria (and in this way slow down the evolution of the disease), without interfering with the immune system. These drugs are the ACE inhibitors and angiotensin receptor blockers, which have been in use for many years. Recently, drugs developed for the therapy of the diabeteswhich belong to the category of SGLT2 inhibitors: are able to slow down the evolution of renal insufficiency even in patients who do not have diabetes. I’m already on the market and can be prescribed with a treatment planif the clinical conditions that indicate its usefulness are present (conditions that must obviously be evaluated on a case-by-case basis by the specialist).

More recently, the result of a study on the drug was published sparsentan which belongs to a different category from the previous ones, specifically it is an endothelin antagonist. The study concluded that this drug is effective in reducing proteinuria; the development of research will tell us if it is also able to prevent the evolution of nephropathy. This clarification is important, because the NIgA is often a disease with slow or very slow evolution; indeed it is well known that in some patients renal function is preserved in the long or very long term. Therefore, to verify the effectiveness of a therapy in a clinical study that cannot last years and years, parameters that are easy and quick to measure over time are first evaluated (the loss of protein in the urine) and then in the long run it evaluates efficacy on renal function.

I have tried to provide an overview of the current situation of NIgA therapy. If I can sum it up in a few words, I’d say what’s next an effective treatment (which perhaps contemplates the use of multiple drugs combined) appear more concrete today than in the past and other drugs, in addition to those I have mentioned (the closest to being available), are being studied: the hopes of making a breakthrough in the treatment of NIgA are by no means unfounded.