Glomerular Diseases

IgA Nephropathy

Condition

This is the most common cause of glomerular (kidney filter) inflammation in the world. Usually it causes blood (microscopic) and protein (variable amounts from minimal to massive) in the urine. Sometimes it presents during a cold or stomach flu with obvious blood in the urine. It can be associated with swelling in the ankles, high blood pressure and decreases in renal function, found by measuring the creatinine level in blood.  In up to half of patients followed long term (over decades), the kidneys can fail requiring support with dialysis or transplantation.

Diagnosis

This is made by kidney biopsy, demonstrating typical features of IgA deposition in the mesangial portion of the glomerulus.

Treatment: Early treatment is preferred. Multiple treatments are available. These focus on diet, exercise, blood pressure control and use of blood pressure medicines that limit the effect of a hormone called angiotensin II.  Other approaches include fish oil and immunosuppressants such as steroids or cytotoxic medications. There are multiple experimental approaches being tested as well.

Clinical Trials

Rituximab in Progressive IgA Nephropathy

Focal Segmental Glomerulosclerosis (FSGS)

Condition

This is a common cause of heavy protein losses in the urine, disproportionally affecting African Americans, but can affect anyone. This disease can run in families, can be related to other illnesses, but usually occurs without obvious cause.  The urine may be foamy and there can be microscopic blood.  Usually it causes progressive losses in kidney function over a few years, monitored by the blood creatinine.  It is often associated with swelling in the ankles and high blood pressure, sometimes quite difficult to control. 

Diagnosis

This is made by kidney biopsy, demonstrating typical features of scarring of the blood vessel loops within the kidney filter.  Different findings on biopsy can suggest various causes and can be helpful in prognosis.

Treatment

Multiple treatments are available.  These focus on diet, exercise, blood pressure control and use of blood pressure medicines that limit the effect of a hormone called angiotensin II.  Other approaches include immunosuppressants such as steroids, calcineurin inhibitors or cytotoxic medications. There are multiple experimental approaches being tested as well.

Clinical Trials

Membranous Nephropathy

Condition

This is another common cause of heavy protein losses in the urine, disproportionally affecting Caucasians, but can affect anyone. This disease can be related to other illnesses, especially chronic infections and rarely cancers,  but usually occurs without obvious cause.  The urine may be foamy and there can be microscopic blood.  In about half of affected individuals, it causes progressive losses in kidney function over a few years, monitored by the blood creatinine.  In others, the protein losses can stay severe causing there own problems. It is often associated with swelling in the ankles, high cholesterol and high blood pressure, sometimes quite difficult to control.  Blood clots and acute infections can occur as complications of this disease when severe and untreated

Diagnosis

This is made by kidney biopsy, demonstrating typical features of immune complex deposits in the blood vessel walls.  Different findings on biopsy can suggest various causes and can be helpful in prognosis and determining treatment.

Treatment

Multiple treatments are available.  These start with diet, exercise, blood pressure control and use of blood pressure medicines that limit the effect of a hormone called angiotensin II.  Other approaches include immunosuppressants such as steroids combined with either calcineurin inhibitors or cytotoxic medications. There are multiple experimental approaches being tested as well.

Clinical Trials

Glomerular Disease of Systemic Lupus Erythematosus (SLE)

Condition

There is a wide variety in which SLE can affect the glomerulus (kidney filter), ranging from quite minor to very severe.  These have been graded by the World Health Organization as Classes I-VI, generally correlating with the likelihood of SLE to cause kidney failure and symptoms.  These processes are usually recognized by changes in the urine, containing blood, protein or inflammation (white blood cells).   As with other diseases of the glomerulus, swelling from fluid retention, high blood pressure and high cholesterol may result.  Kidney involvement can be the first sign of SLE, or can occur in concert with other complications (arthritis, rashes, fever, other organ involvement). Early treatment may be essential at protecting the kidney from severe, progressive injury.

Diagnosis

This is made by kidney biopsy, demonstrating typical features of immune complex deposits in the blood vessel walls or other parts of the kidney filter.  Different findings on biopsy are extremely helpful in establishing prognosis and determining treatment.

Treatment

Treatment very much depends on class of involvement, including no treatment when mild (Class I or II) or when the kidney is already damaged beyond repair (Class VI).  Treatment includes diet, exercise, blood pressure control and use of blood pressure medicines that limit the effect of a hormone called angiotensin II.  Other approaches include immunosuppression with corticosteroids combined with either calcineurin inhibitors or cytotoxic medications, often using mycophenolate. There are multiple experimental approaches being tested as well, depending on the specific type of glomerular involvement.