IgA Nephropathy – Patient Information

  • How the illness affects people

    IgA Nephropathy is a distinct pattern of disease in which the immune protein immunoglobulin A (IgA) that is normally present in the blood stream gets deposited in the kidneys.

    Symptoms and signs

    Some people with IgA nephropathy do not have any symptoms. For those who do, the common symptoms are:

    • Blood in the urine (haematuria) – if this is visible, the urine may appear red or dark brown in colour (like a cola drink). Visible haematuria may occur at the time of infections such as a common cold or sore throat. In this case it usually lasts a few days and then reduces. Haematuria can be invisible to the naked eye but detected on a simple stick test of the urine (microscopic haematuria)
    • Some patients can develop swelling or puffiness in different parts of the body, especially around the eyes, legs and feet – this is called oedema.
    • Protein in the urine (proteinuria) is an important feature in some patients. If protein losses in the urine are very heavy the urine can look frothy.

       

    Like so many kidney diseases IgA Nephropathy can go unrecognised for a long period until patients notice some of the complications listed below.

    Complications

    Some people with IgA Nephropathy have complications – health problems that happen because of the condition or its treatment. These include:

    • The blood pressure may be too high (hypertension).
    • The kidneys may not work normally so that they are less able to clear waste from the body. This reduction in kidney function may be temporary (Acute Kidney Injury, AKI) and reversible or irreversible (Chronic Kidney Disease (CKD)). The function of the kidney is measured on a blood test (plasma creatinine). A small percentage of people with IgA nephropathy (less than 30%) eventually need kidney replacement therapy with a kidney transplantation or dialysis.
    • Rarely, the condition gets worse more rapidly and the kidney function deteriorates quickly – this is called rapidly progressive glomerulonephritis (RPGN).
  • What can be done about it?

    It is important to make an accurate diagnosis. Often the first step is to recognise that a person has blood in their urine. This in itself does not confirm the diagnosis so a kidney biopsy may be needed.

    A kidney biopsy is very reliable for making the diagnosis. This is because the changes in the kidney are widely spread so a small piece of tissue gives a good indication of what is happening throughout both kidneys. A biopsy is also good at identifying other possible conditions and ruling out IgA.

    However in patients with good kidney function and no complications, and for whom no immediate treatment is needed, a doctor may suspect the diagnosis but delay doing the biopsy.

    Once the diagnosis has been made, treatment will be made based on disease severity.

    The aim of treatment is to try to protect the kidneys from further damage. Crucially this is done by lowering blood pressure. This treatment to lower blood pressure is likely to include life-style measures such as losing weight and reducing salt in the diet as well as taking medicines that lower blood pressure.

    Some people with IgA nephropathy also benefit from tablets to dampen the immune system (called immunosuppressives e.g. steroids).  In some patients the disease comes and goes and there may be fare ups followed by periods of remission. In other patients the damage seems to occur continuously. The kidneys have a lot of reserve capacity however and function is only reduced after extensive damage has occurred.

    Despite treatment, some people with IgA nephropathy eventually require kidney replacement treatment with a kidney transplant or dialysis because their own kidneys can no longer work well enough to keep them well. IgA Nephropathy can come back in a transplant kidney. This seems to happen in around 50% of cases but it does not usually affect the life-span of the transplanted kidney.

    For more information about treatment visit the Clinician Information Page.

  • Other peoples’ experiences

    Janine’s Story

    My name is Janine and I’m 42, married with a daughter 13 and a son 7. I was diagnosed with IgA Nephropathy 2.5 years ago. My first symptoms were obvious blood in my urine. I also experienced fatigue and achy joints. Following a referral to a urologist and two clear cystoscopies, I was referred to a nephrologist who arranged a renal biopsy. Shortly after my 40th birthday, I was diagnosed with IgA Nephropathy and given a 50% chance of progressing to End Stage Renal Failure (ESRF) within 10 years.

    Unfortunately renal failure progressed far more quickly than anticipated and despite a course of cyclophosphamide after a further biopsy confirmed aggressive IgA, I started hemodialysis in December 2014 just 2 years after diagnosis. I found dialysis hard on me and my young family and I think it takes most people a good while to settle into it, getting used to diet and fluid restrictions, the time it takes and the side effects and also to find the right form of dialysis for them. Luckily for me and my family I was offered a cadaver kidney just 3.5 months from joining the transplant list after the same time on dialysis.

    Six months post-transplant and I feel so much better and I’m free from the ties of dialysis and its restrictions. I feel so indebted to my donor and his family. I’ve been told that there’s a high chance the IgA will return in my transplant but in the meantime I’m enjoying feeling well and I’m making the most of the time I have until I need another transplant and enjoying life to the full with my family.

    Jennifer’s Story

    When my son was 11 he noticed one day that his urine was pink. We went to the GP who sent him to the hospital where he was kept in for three nights. He had an ultrasound scan and some blood tests, but was discharged without a diagnosis.

    A week later he started being sick so I took him back to the GP. She phoned the hospital who informed her that a blood test had given a positive result for Strep A and he was given a strong course of antibiotics. The GP suspected IgA Nephropathy and said he needed a hospital referral. I looked for information about the disease and contacted Kidney Research UK who suggested it would be best for him to be seen by a Paediatric Nephrologist. Our GP agreed, he was referred and after a biopsy IgA Nephropathy was confirmed.

    In the first year he was unwell seven times. He kept getting infections which triggered flu like symptoms, plus blood and protein in the urine and sometimes raised creatinine which returned to normal after the infection went.  In the second year he was unwell four times, however in the last year things have really settled down he has only been unwell twice which I would consider normal for any child.  He is now seen by the GP every 6 months for urine and blood pressure checks and has an annual appointment with a Pediatrician. Currently he is not on any medication and it doesn’t impact on his life.

    Karen’ story

    My name is Karen and 13 years ago I was diagnosed with IgA Nephropathy and Cardio Myopathy. To say it was a stressful time is an understatement.

    I had been feeling generally unwell for about two years and high blood pressure was blamed. But then I found I was unable to walk any distance, I was out of breath and found it increasingly difficult to do my job. Around this time I moved from Manchester to Cardiff and a few days later I was rushed to University Hospital of Wales as I literally could not breathe.

    After a great many tests and also a biopsy I was diagnosed. I was very fortunate to have the very best consultants, both renal and heart to look after me and gradually over the next few years I had an assortment of drugs to try to keep me steady.

    Christmas 2010 was very eventful as my consultant told me I needed to start dialysis. By now I was living in Nuneaton and I argued that it really wasn’t convenient as I had all the family coming to stay so would do a deal with him! If he let me wait till the New Year I would have Christmas, tell my family I was a ‘bit unwell’ and then start dialysis in January.

    So it was that January saw me starting dialysis which continued till 15th June 2012 when I had a kidney transplant at University Hospital Coventry. My husband was the living donor.

    I know that IgA may very well return to my new kidney and that I may have to have another transplant but the feeling of being able to have a life without dialysis three times a week, to eat what I want, to be able to spend time with my grandchildren, that is all so wonderful that I really can’t describe it adequately.

    I felt that I had got my life back!

    It wasn’t plain sailing. The kidney didn’t wake up properly for a few days, I had to have some more dialysis, I was rushed back to theatre three times with various problems, I have pills to take for the rest of my life, etc etc but all this is as nothing compared to the probability of not being here.

    On 12th December 2014 I was able to hold the hand of my daughter Nicol at the birth in Leicester Royal of my granddaughter Elizabeth Rose.

    Priceless!

    Margaret‘s story 

    I was diagnosed with IgA Nephropathy on 31st October 2013 when I was 66 years old. I had no obvious symptoms of ill-health, just tiredness and I had had high blood pressure for a number of years which had been treated but not investigated.

    I changed GP in 2011 and during a routine health check I was found to have blood and protein in my urine. I was referred to a urologist and had a cystoscopy and other tests. When they returned negative I was referred to a nephrologist.

    I had had similar tests some 10 years previously when blood was found in my urine during a pre-surgery check. I was referred to as urologist but when those tests came back negative no further tests were ordered.

    The nephrologist referred me for several tests which concluded with a kidney biopsy. In my case the biopsy proved difficult both during the process and afterwards. Unfortunately, the biopsy was a failed test so it had to be repeated. The second biopsy showed I had IgA Nephropathy.

    When I was diagnosed my kidney function was at 29%. It had dropped from 65% the previous year, so by over 30% in 12 months. I was told that without treatment or if I didn’t respond to treatment, I was looking at dialysis within six months. My consultant and I agreed we would start a fairly aggressive treatment immediately.

    My blood pressure medication was increased. I went on to 40m prednisolone and 1500mg Mycophenolate Mofitel a day. I gradually reduced the steroids to the 5mg dose I am on now and I have increased the immunosuppressants to 2000 mg a day.

    My eGFR has stabilised between 30% and 35%, my blood pressure is controlled and it is very rare for there to be any sign of blood or protein in my urine. I feel very lucky that those initial urine tests were pursued and my condition diagnosed.

    I try and keep my exercise level up. I play golf, I am in a walking group and I play racket ball. I would be happier if I could come off steroids altogether but my consultant says the impact of that is unpredictable. So, I am feeling fairly positive at the moment, I am just trying to get on with my life and hope I maintain this current level of stability.

    Phil’s Story

    I was diagnosed with IgA Nephropathy six years ago, when I was 54 years old. I was feeling absolutely fine, but was called into my local GP’s surgery for a quick health check (they do this for people in the mid-50s). I was found to have very high blood pressure and although I was in a high stress profession, there seemed no real reason for this. This was followed by a blood test which showed I had severely impaired kidney function with an eGFR of 27 and was diagnosed with IgA Nephropathy after a renal biopsy at the local hospital. It seems likely that I have had the condition for many years undiagnosed.

    In terms of medication, I take Candesartan to control my blood pressure, which seems to work well and have a low dose statin to keep my cholesterol low, which it is. I’m also currently part of the year-long HARP 111 drug trial (HARP stands for heart and renal protection) which is part of the research looking for a cure for CKD. Since being diagnosed, I have attempted to be pro-active with my health. I have lost 5 kilos (which has been really hard) eat a low salt and potassium diet and try to keep myself fit – I am an enthusiastic attender of my local gym. I see my consultant every six months and have an e-consultancy via the internet following a blood test also every six months. My eGFR has been falling at about 1% a year and stands at about 20% currently, but I still have no real symptoms. I have concerns about the future and sometimes worry about how things will be if I ever need dialysis and a transplant, but hopefully, if that ever does happen, it will be a long time from now and I will be well prepared. My wife and family are incredibly supportive. Keeping positive and forward looking is important for me.

  • Patient Support Group

    A UK IgA Nephropathy Patient Support Group has been established, which coordinates the UK IgAN Patient Information Day. Details of the group can be found on the IgAN Support UK Facebook Forum which is available to all UK patients with IgAN.

  • How the disease works

    Immunoglobulin A is a protein produced by the immune system to fight infections in the body. In IgA nephropathy the IgA molecule is abnormally formed and this helps explain why it becomes embedded in the microscopic blood vessels of the kidney (glomeruli). This process usually takes place over a long period of time and causes damage to the kidneys. It is not known why the bone marrow cells start to produce the abnormally formed IgA.

    Sometimes IgA nephropathy occurs in the condition called Henoch–Schönlein purpura (HSP).

  • What’s new? Opportunities for research and development

    Historically there has been a lack of high-quality clinical trials in IgA nephropathy and an absence of effective therapies. Over the past few years this has changed. There are now a number of clinical trials in IgA Nephropathy, most of which are being conducted in the UK. One of the best places to find out about clinical trials is the ClinicalTrials.gov website.

    The IgA Nephropathy Rare Disease Group (RDG) is working with international partners with the aim of finding new and improved treatments, and to empower patients. A first step is to compare the symptoms and genetic markers of IgA Nephropathy. To do this the RDG is registering patients with this condition in the National Renal Rare Disease Registry (RaDaR). The registry will be used to find suitable participants for future research trials into the effectiveness of new treatments. If you are interested in finding out more about the registry RaDaR or the activity of the RDG please visit the IgA Nephropathy RDG page.

  • Further Information

    A paediatric information sheet on IgA Nephropathy is available from Info Kid. A printable version can be downloaded here.

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IgA Nephropathy Patient Information Version 4 updated September 2017
Written by the IgA Nephropathy Rare Disease Group