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Is Iga Nephropathy A Chronic Kidney Disease

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MAILBAG! On chronic kidney condition called IgA Nephropathy 3-15-18

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How Is Iga Nephropathy Treated

Researchers have not yet found a specific cure for IgA nephropathy. Once the kidneys are scarred, they cannot be repaired. Therefore, the ultimate goal of IgA nephropathy treatment is to prevent or delay end-stage kidney disease. A health care provider may prescribe medications to

  • control a persons blood pressure and slow the progression of kidney disease
  • remove extra fluid from a persons blood
  • control a persons immune system
  • lower a persons blood cholesterol levels

How Did I Get It

IgA is an autoimmune disease. This is a disease in which a persons own immune system damages their own body. Scientists know that the IgA immune complex causes the inappropriate immune response. But it is not yet known what causes the defective IgA in the first place. It likely has both genetic and environmental components. A person is born with a predisposition for the disease, and then some sort of trigger, for example an infection or food exposure, turns the disease on.

IgA commonly occurs in Caucasians and Asians. It is relatively uncommon in those of African descent. It is twice as common in males as females. Though it potentially affects any age group, IgA is most commonly diagnosed in early and middle adulthood.

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What Are The Symptoms Of Iga Nephropathy

In the early stages of IgA nephropathy, you may not notice any symptoms. You can have the disease for years, or even decades, without knowing it. The first sign of IgA nephropathy can appear when you have a cold, sore throat or other infection. When this happens, you may notice your urine is pink or brown, which is a sign that you have blood in your urine. Some people also have protein in their urine. If you have protein in your urine, you might notice that your urine looks foamy or bubbly and that your hands and feet are swollen.

Your health care provider might notice a problem before you do. A routine urine test at a regular check-up can show signs of IgA nephropathy. If you have tiny amounts of blood in your urine, you may not be able to see it, but it will show up in a urine test. If you have IgA nephropathy, the tiny clumps of blood in your urine will be shaped like tubes, because they form inside the kidneys tube-like filters called glomeruli.

If your urine test shows a problem or if you notice signs of blood or protein in your urine, your health care provider may want you to have blood tests to check your kidney health.

Clinical Presentation And Outcome

Types Of Kidney Disease. Chronic Renal Disease, Iga ...

Macroscopic hematuria is the most frequent IgAN initial presentation in children, followed by the fortuitous finding of microscopic hematuria accompanied or not by proteinuria. In adults, the diagnosis is often made at the stage of chronic kidney disease , as if diagnosis in this belated context were made at an advanced stage after missing a pauci-symptomatic form that previously occurred in childhood. By contrast, the diagnosis of IgAV is, in most cases, made much earlier, revealed by the presence of extra-renal signs.

Indeed, by definition, the IgAV is characterized by the combination of cutaneous , gastrointestinal and articular involvement. More rarely, we can observe a neurological, pulmonary or urological involvement . The long-term prognosis of the disease depends on the presence of renal impairment and its evolution. From a histological point of view, it is not possible to distinguish a glomerulonephritis as part of an IgAV from an IgAN. Renal biopsy shows, in the two cases: on immunofluorescence, predominant IgA1 deposits in the mesangium of all glomeruli , with glomerular deposits of IgG, IgM, C3 and fibrin in variable proportions on light microscopy, mesangial hypercellularity with increased mesangial matrix, endo-capillary hypercellularity, segmental glomerular sclerosis, cellular crescents and tubular atrophy and interstitial fibrosis.

Mesangial and capillary wall IgA deposits .

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What Does It Look Like

First, a quick look at the kidney. Most people have two kidneys, one on each side of their lower back. All of the blood in your body passes through your kidneys many times during the day. Each time blood goes through some of it gets filtered and cleaned by the glomeruli. This cleaning is how your body gets impurities out of the blood . Some of the cleaned blood becomes your urine. Urine isnt red like blood because the red blood cells, which give blood its color, are too big to fit through the filters. A glomerulus is just a tiny bag of blood containers through which blood gets filtered. All of the clean blood runs into tubes which eventually lead to your bladder.

The drawing below shows how this disease affects the kidney. Pictured is a single blood vessel from a kidney filter, shown in cross section. The red disks are red blood cells. They normally should remain inside the vessel itself .The IgA deposits have activated the immune system and damaged the vessel wall. Therefore, red blood cells are spilling out the bottom of the vessel and into the urine.

Mesangial immune deposits

Below is a picture, from the actual kidney biopsy of someone with IgA Nephropathy. Shown is a single filter in which the actual IgA deposits have been stained florescent green.

Lifestyle And Home Remedies

To help keep your kidneys healthier:

  • Take steps to reduce your blood pressure. Keeping your blood pressure levels near normal can help slow kidney damage from IgA nephropathy. Healthy changes in your lifestyle â including limiting your salt intake, losing excess weight, being physically active, using alcohol in moderation and taking your blood pressure medications as prescribed â are ways to keep your blood pressure under control.
  • Monitor your blood pressure at home. Note each reading and bring this record with you to your doctor’s appointments.
  • Eat less protein. Reducing the amount of protein you eat and taking steps to decrease your cholesterol levels may help slow the progression of IgA nephropathy and protect your kidneys.

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Innovative Iga Nephropathy Therapy Receives Accelerated Fda Approval

  • Last Update: 2022-01-11

A few days ago, Calliditas Therapeutics announced that the US FDA approved budesonide sustained-release capsules for the treatment of adult patients with primary immunoglobulin A nephropathy who are at risk of rapid disease progression . Budesonide is a corticosteroid immunosuppressant that can reduce proteinuria levels in such patients, but it has not been determined whether it can slow down the decline in renal function . It is worth mentioning that Everest Medicines reached a cooperation with Calliditas in 2019 and has the exclusive rights to develop and commercialize the drug in Greater China . The press release stated that this is the first FDA-approved therapeutic drug to reduce proteinuria in patients with IgA nephropathy .

IgA nephropathy is the main cause of chronic kidney disease and renal failure. It is a chronic and progressive autoimmune disease related to progressive kidney injury . IgA is an immunoglobulin that is produced in large amounts in the body and is closely related to mucosal immunity . The main feature of patients with IgA nephropathy is the presence of IgA deposits in the glomeruli . The progression of IgA nephropathy will be accompanied by glomerulosclerosis, renal interstitial fibrosis, renal insufficiency, proteinuria and hypertension . Among them, 50% of patients with IgA nephropathy will develop end-stage renal disease within 30 years, which seriously affects the quality of life of patients .

Lifestyle And Risk Of Progression To End Stage Renal Disease

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In a study by Huang et al in Chinese subjects with IgA nephropathy, which compared cases that progressed to end-stage renal failure with controls that did not, the proportion of patients who were males, smokers, alcohol drinkers, and physically inactive was signficantly higher in cases than in controls. Alcohol use proved to be an independent risk factor for progression to ESRF, while regular exercise was associated with decreased risk males who did not exercise seemed especially likely to progress to ESRF. These authors concluded that physical exercise should be encouraged in IgA nephropathy patients, especially in males, for a better renal outcome, and cessation of alcohol use might help prevent disease progression.

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Proteomic Studies In Case Of Fsgs Mgn And Igan

Modern technologies such as proteomics have led to new opportunities in searching for biomarkers of renal diseases especially in bio-fluids, mostly in urine, serum, and plasma, which are much less invasive for the patients. Urine sample prepared by a non-invasive collecting method offers a valuable source of biomarkers, reflecting physiological state of the system. Except for the urine itself, urinary microvesicles or exosomes are also rich in biomarkers. However, some considerations must be taken into account such as time of sampling, as urine chemical content varies during different times of a day.

Membranous glomerulonephritis

Table 1 Proteomic studies in the case of FSGS, MGN and IgAN

Focal segmental Glomerulosclerosis

IgA nephropathy

What Are Clinical Trials And Are They Right For You

Clinical trials are part of clinical research and at the heart of all medical advances. Clinical trials look at new ways to prevent, detect, or treat disease. Researchers also use clinical trials to look at other aspects of care, such as improving the quality of life for people with chronic illnesses. Find out if clinical trials are right for you.

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How Can A Person Prevent Iga Nephropathy

Researchers have not found a way to prevent IgA nephropathy. People with a family history of IgA nephropathy should talk with their health care provider to find out what steps they can take to keep their kidneys healthy, such as controlling their blood pressure and keeping their blood cholesterol at healthy levels.

What Are The Signs And Symptoms Of Iga Nephropathy

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In its early stages, IgA nephropathy may have no symptoms it can be silent for years or even decades. Once symptoms appear, the most common one is hematuria, or blood in the urine. Hematuria can be a sign of damaged glomeruli. Blood in the urine may appear during or soon after a cold, sore throat, or other respiratory infection. The amount of blood may be

  • visible with the naked eye. The urine may turn pink or the color of tea or cola. Sometimes a person may have dark or bloody urine.
  • so small that it can only be detected using special medical tests.

Another symptom of IgA nephropathy is albuminuriawhen a persons urine contains an increased amount of albumin, a protein typically found in the blood, or large amounts of protein in the urine. Albumin is the main protein in the blood. Healthy kidneys keep most proteins in the blood from leaking into the urine. However, when the glomeruli are damaged, large amounts of protein leak out of the blood into the urine.

When albumin leaks into the urine, the blood loses its capacity to absorb extra fluid from the body. Too much fluid in the body may cause edema, or swelling, usually in the legs, feet, or ankles and less often in the hands or face. Foamy urine is another sign of albuminuria. Some people with IgA nephropathy have both hematuria and albuminuria.

After 10 to 20 years with IgA nephropathy, about 20 to 40 percent of adults develop end-stage kidney disease.5 Signs and symptoms of end-stage kidney disease may include

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What Causes Iga Nephropathy

Scientists think that IgA nephropathy is an autoimmune kidney disease, meaning that the disease is due to the bodys immune system harming the kidneys.

People with IgA nephropathy have an increased blood level of IgA that contains less of a special sugar, galactose, than normal. This galactose-deficient IgA is considered foreign by other antibodies circulating in the blood. As a result, these other antibodies attach to the galactose-deficient IgA and form a clump. This clump is also called an immune complex. Some of the clumps become stuck in the glomerulus of the nephron and cause inflammation and damage.

For some people, IgA nephropathy runs in families. Scientists have recently found several genetic markers that may play a role in the development of the disease. IgA nephropathy may also be related to respiratory or intestinal infections and the immune systems response to these infections.

How Common Is Iga Nephropathy And Who Is More Likely To Get The Disease

IgA nephropathy is one of the most common kidney diseases, other than those caused by diabetes or high blood pressure.1

IgA nephropathy can occur at any age, although the first evidence of kidney disease most frequently appears when people are in their teens to late 30s.2 IgA nephropathy in the United States is twice as likely to appear in men than in women.3 While found in people all over the world, IgA nephropathy is more common among Asians and Caucasians.4

A person may be more likely to develop IgA nephropathy if

  • he or she has a family history of IgA nephropathy or IgA vasculitisa disease that causes small blood vessels in the body to become inflamed and leak
  • he is a male in his teens to late 30s
  • he or she is Asian or Caucasian

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How Is Iga Nephropathy Diagnosed

Currently, health care providers do not use blood or urine tests as reliable ways to diagnose IgA nephropathy therefore, the diagnosis of IgA nephropathy requires a kidney biopsy.

A kidney biopsy is a procedure that involves taking a small piece of kidney tissue for examination with a microscope. A health care provider performs a kidney biopsy in a hospital or an outpatient center with light sedation and a local anesthetic. The health care provider uses imaging techniques such as ultrasound or a computerized tomography scan to guide the biopsy needle into the kidney. A pathologista doctor who specializes in examining tissues to diagnose diseasesexamines the kidney tissue with a microscope. Only a biopsy can show the IgA deposits in the glomeruli. The biopsy can also show how much kidney damage has already occurred. The biopsy results can help the health care provider determine the best course of treatment.

Discussion And Future Research

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After reporting all those studies, can we say that IgA Nephropathy and IgA Vasculitis are two clinical entities of the same disease?

Clinical studies showed that they differentiate clearly in terms of clinical presentation and age at onset.

Concerning outcome, studies are conflicting, but tend to show that if patients are stratified on age and genetic background, IgAN and IgAV have the same renal prognosis. The presence of clinically speaking extra-renal disease makes the diagnosis of IgAV easy at an early stage, whereas in patients whose disease is limited to the kidney, the diagnosis is inevitably belated and therefore more advanced. It is not said, moreover, that these patients had, some years before, some unnoticed purpuric lesions. Therefore, the real question is: Why do some patients with IgAV have no renal involvement, and why do patients with IgAN have no skin lesions?

Physiopathological mechanisms and their related biomarkers are similar, each time they have been evaluated in the same study, none of which have been identified, to date, as having a strong prognostic value, either in IgAN and IgAV. It is thus most essential to identify early diagnostic and prognostic markers, which could be able to detect patients who will not spontaneously heal and require specific treatment . Working together to set up new clinical studies appears necessary. It will be crucial for those future trials:

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What Symptoms Occur In Iga Nephropathy

IgA nephropathy does not trigger symptoms in its initial stages generally, due to which one can live with this disease unknowingly for years. Routine tests can help detect the abnormal functioning of glomeruli and microscopic hematuria .

Signs and symptoms associated with IgA nephropathy are stated below:

  • The recurrent reddish or brownish appearance of the urine is caused by the presence of red blood cells in the urine.
  • The bubbly or foamy appearance of the urine is caused due to protein leakage in the urine by the kidneys tiny filters. This condition is known as proteinuria.
  • Side or back pain.

How Do People Know They Have Iga Nephropathy

In the early stages, IgA often has no symptoms. This disease can be silent for years, even decades. The first sign that the patient may notice is blood in the urine. Visible blood may appear in the urine if a person has a cold, sore throat, or other infection. At other times, blood in the urine can only be detected by a urinalysis .

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What Clinical Trials Are Open

Clinical trials that are currently open and are recruiting can be viewed at www.ClinicalTrials.gov.

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