Anti-Glomerular Basement Membrane disease (anti-GBM disease) is a disease that occurs as a result of injury to small blood vessels (capillaries) in the kidneys and/or lungs. Autoantibodies are antibodies directed toward the body itself (rather than towards something foreign such as bacteria or viruses). In anti-GBM disease, these autoantibodies are targeted to the basement membrane, which is part of the wall of these capillary blood vessels in the kidneys and lungs. The name anti-GBM disease reflects the fact that this disease is caused by autoantibodies targeting and causing damage to (anti-) the glomerular basement membrane (GBM).
Anti-GBM disease can affect just the kidneys or both the lungs and kidneys (it is uncommon for the only the lungs to be affected). The reason these organs are affected is because the basement membrane that is targeted or attacked in this disease is only accessible to antibodies in the kidneys and lungs (not in other organs). About half of people with anti-GBM disease have lung involvement. Anti-GBM disease that only affects the kidneys is called anti-GBM glomerulonephritis. This is because there is inflammation (-itis), occurring in the glomeruli (filters) of the kidney. Anti-GBM disease affecting both the kidneys and lungs is often called Goodpasture’s syndrome or Goodpasture’s disease (though anti-GBM disease is the preferred term now).
Glomerulonephritis due to anti-GBM antibody disease is very rare. It occurs in less than 1 in a million people. It most commonly affects young, white men (aged 15-35). After young men, it occurs next most commonly in older adults (late 50s and beyond, women more than men). It is very rare in children.
Once the diagnosis is confirmed, treatment includes a few different parts. Anti-GBM disease can be life threatening due to bleeding in the lungs, and kidney failure can occur quickly, so starting treatment as soon as possible is important.
- If the lungs are involved, providing oxygen as needed to keep oxygen levels up. Sometimes, a breathing tube may be needed temporarily.
- If the kidneys have a lot of damage, they may not be able to function well enough to do what they need to, and dialysis may be needed to help do the kidneys’ job. This includes getting rid of fluid from the body, balancing electrolytes and acid-base levels in the blood, and removing toxins/wastes from the body.
- Plasmapheresis. This is a procedure that removes the anti-GBM antibody from the bloodstream. It can also be called plasma exchange or PLEX. A person’s blood is run through a machine that removes antibodies from the blood, then the blood is returned to the body (without the antibodies). Treatments last a couple hours and are usually done every 1-2 days for about 2 weeks.
- Immunosuppressive treatment(medicines to suppress the immune system) are given to decrease/prevent the immune system
When there is lung involvement, symptoms can include:
- Coughing up blood
- Cough (without blood)
- Shortness of breath/difficulty breathing (and even respiratory failure)
- Chest pain
When the kidneys are affected, this can cause:
- Low kidney function (kidney injury) or kidney failure, which can cause symptoms including fatigue, nausea/vomiting, poor appetite or weight loss, metallic taste in mouth, and confusion or decreased alertness. However, most people with kidney injury or damage do not have a lot of symptoms until they are in kidney failure.
- Blood in the urine – this may or may not be visible
- Foamy urine (from protein in the urine)
- Creatinine (blood test) can be tested to evaluate kidney function
- CBC (blood test) may show anemia
- Anti-GBM antibodies can be checked (bloodwork) – this is an important part of making the diagnosis.
- Corticosteroids– usually this is given as a high-dose infusion of a medication called methylprednisolone (Solumedrol) daily for 3 days. Treatment with an oral (by mouth) medication such as prednisone is typically continued after the infusion and tapered off over a period of 3-9 months. This medication decreases the inflammation caused by the immune system that contributes to kidney and lung damage.
- Cyclophosphamide (Cytoxan)– this is an immune suppressing medication that can be given as a monthly infusion or as a daily oral (by mouth) dose. This is generally continued for at least 2-3 months but up to 6 months. This medication helps to prevent the immune system from making more anti-GBM autoantibodies.
- Rituximab (Rituxan)- this is an immune suppressing medication that is given as an IV infusion, generally in 2-4 doses spaced over 2-4 weeks. This medication has not been evaluated in trials, but has been used in some patients – if cyclophosphamide is not a good option or in some patients in addition to cyclophosphamide.
- Other – other immunosuppressive medications such as mycophenolate mofetil or azathioprine have sometimes been used in this disease after treatment with cyclophosphamide or in combination with cyclophosphamide or other immunosuppressive medications. These medications are used much less commonly and in selected circumstances. They should not be the primary medication used to treat the disease as cyclophosphamide is the most effective medication from the evidence we have.
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