For US Healthcare Professionals

What Are Granulomatosis With Polyangiitis (GPA) and Microscopic Polyangiitis (MPA)?

Granulomatosis with polyangiitis (formerly known as Wegener's Granulomatosis) and microscopic polyangiitis are two types of ANCA-associated vasculitis, or AAV. 

AAV is a form of vasculitis that affects the small blood vessels in your body. In general, AAV affects the sinuses, kidneys, lungs, and skin, but the ways the diseases show themselves vary.

About ANCA-associated vasculitis (AAV)

AAV causes inflammation in the small blood vessels. This inflammation may cause the layers of the blood vessel wall to thicken, which narrows the blood vessel and reduces the amount of blood that can flow through it. This reduction in blood flow can cause serious problems and even damage the body's organs.

Image of normal and inflamed small blood vessel

ANCAs play a role in GPA and MPA

Antibodies are found in the blood and are produced by the immune system. Their job is to fight germs and other outside invaders. But in GPA and MPA, a harmful type of antibody known as an autoantibody (pronounced aw-toh-AN-ti-bod-ee) is produced. 

The autoantibody often involved in GPA and MPA is known as ANCA. ANCAs act against the body’s own healthy tissues and cells. They cause inflammation that targets and attacks the blood vessel walls in different organs in the body.

ANCAs are usually present in GPA and MPA, but there are times when this is not the case. Your doctor will perform a blood test to look for ANCAs.

ANCAs are produced by plasma cells, which develop from B-cells

Depiction of ANCAs causing inflammation in vessel

ANCAs are produced by B-cells. They target a certain type of white blood cell called neutrophils (pronounced NOO-truh-fils). ANCAs cause the neutrophils to stick and clump to the walls of small blood vessels in different tissues and organs of the body. This process leads to inflammation, which means that a part of the body becomes swollen and painful. 

Rituxan is thought to decrease the number of B-cells by targeting those that have a specific marker on their cell surface called CD20. It is thought that interfering with B-cell function can disrupt ANCA production.

GPA and MPA can be marked by periods of flares and remission

In GPA and MPA, there are periods of flares and periods of remission. Flares occur when your symptoms worsen or new symptoms appear. Remission, on the other hand, is a long or short period of time after a flare when symptoms are controlled or go away.

Since GPA and MPA are chronic diseases, they never completely go away. So even when you are in remission, your doctor may continue to prescribe medications to control your symptoms. Everyone's experience is different, so it's hard to predict when a flare may occur.

Keep in Mind

GPA and MPA affect people of all genders, races, and ages. Learn all you can about your disease and available treatment options. Being informed can help you and your doctor choose treatments that are best suited for you.

ANCA, antineutrophil cytoplasmic antibody.

Safety Info

The inflammation caused by GPA and MPA can cause a variety of symptoms

Learn what to look out for

IMPORTANT SIDE EFFECT INFORMATION

What is Rituxan?

Rituxan is a prescription medicine used to treat:

  • Adults with Rheumatoid arthritis (RA): with another prescription medicine called methotrexate, to reduce the signs and symptoms of moderate to severe active RA, after treatment with at least one other medicine called a tumor necrosis factor (TNF) antagonist has been used and did not work well enough. 

  • People with Granulomatosis with Polyangiitis (GPA) (Wegener’s Granulomatosis) and Microscopic Polyangiitis (MPA) ages 2 years and above: with glucocorticoids.

  • Adults with Pemphigus Vulgaris (PV): to treat moderate to severe PV.

RITUXAN is not indicated in children less than 2 years of age with GPA or MPA or in children with conditions other than GPA or MPA.

What is the most important information I should know about Rituxan?

Rituxan can cause serious side effects that can lead to death, including:

  • Infusion-Related Reactions: Infusion-related reactions are very common side effects of Rituxan treatment. Serious infusion-related reactions can happen during your infusion or within 24 hours after your infusion of Rituxan. Your healthcare provider should give you medicines before your infusion of Rituxan to decrease your chance of having a severe infusion-related reaction.

    Tell your healthcare provider or get medical help right away if you get any of these symptoms during or after an infusion of Rituxan:

    • Hives (red itchy welts) or rash
    • Itching
    • Swelling of your lips, tongue, throat, or face
    • Sudden cough
    • Shortness of breath, difficulty breathing, or wheezing
    • Weakness
    • Dizziness or feel faint
    • Palpitations (feel like your heart is racing or fluttering)
    • Chest pain
  • Severe Skin and Mouth Reactions: Tell your healthcare provider or get medical help right away if you get any of these symptoms at any time during your treatment with Rituxan:
    • Painful sores or ulcers on your skin, lips, or in your mouth
    • Blisters
    • Peeling skin
    • Rash
    • Pustules

  • Hepatitis B Virus (HBV) Reactivation: Before you receive your Rituxan treatment, your healthcare provider will do blood tests to check for HBV infection. If you have had hepatitis B or are a carrier of hepatitis B virus, receiving Rituxan could cause the virus to become an active infection again. Hepatitis B reactivation may cause serious liver problems, including liver failure and death. Your healthcare provider will monitor you for hepatitis B infection during and for several months after you stop receiving Rituxan. Tell your healthcare provider right away if you get worsening tiredness, or yellowing of your skin or white part of your eyes during treatment with Rituxan.

  • Progressive Multifocal Leukoencephalopathy (PML): PML is a rare, serious brain infection caused by a virus that can happen in people who receive Rituxan. People with weakened immune systems can get PML. PML can result in death or severe disability. There is no known treatment, prevention, or cure for PML.

    Tell your healthcare provider right away if you have new or worsening symptoms or if anyone close to you notices these symptoms:

    • confusion
    • dizziness or loss of balance
    • difficulty walking or talking
    • decreased strength or weakness on one side of your body
    • vision problems, such as blurred vision or loss of vision

What should I tell my healthcare provider before receiving Rituxan?

Before receiving Rituxan, tell your healthcare provider if you:

  • have had a severe reaction to Rituxan or a rituximab product
  • have a history of heart problems, irregular heartbeat, or chest pain
  • have lung or kidney problems
  • have had an infection, currently have an infection, or have a weakened immune system
  • have or have had any severe infections including:
    • Hepatitis B virus (HBV)
    • Hepatitis C virus (HCV)
    • Cytomegalovirus (CMV)
    • Herpes simplex virus (HSV)
    • Parvovirus B19
    • Varicella zoster virus (chickenpox or shingles)
    • West Nile virus
  • have had a recent vaccination or are scheduled to receive vaccinations. You should not receive certain vaccines before or during treatment with Rituxan
  • have any other medical conditions
  • are pregnant or plan to become pregnant. Talk to your healthcare provider about the risks to your unborn baby if you receive Rituxan during pregnancy. Females who are able to become pregnant:
    • Your healthcare provider should do a pregnancy test to see if you are pregnant before starting Rituxan.
    • You should use effective birth control (contraception) during treatment with Rituxan and for 12 months after your last dose of Rituxan. Talk to your healthcare provider about effective birth control.
    • Tell your healthcare provider right away if you become pregnant or think that you are pregnant during treatment with Rituxan
  • are breastfeeding or plan to breastfeed. Rituxan may pass into your breast milk. Do not breastfeed during treatment and for 6 months after your last dose of Rituxan
  • are taking any medications, including prescription and over-the-counter medicines, vitamins, and herbal supplements. Especially tell your doctor if you take or have taken:
    • a tumor necrosis factor (TNF) inhibitor medicine
    • a disease modifying anti-rheumatic drug (DMARD)

What are the possible side effects of Rituxan?

Rituxan can cause serious side effects, including:

  • Tumor Lysis Syndrome (TLS): TLS is caused by the fast breakdown of cancer cells. TLS can cause you to have:

    • Kidney failure and the need for dialysis treatment
    • Abnormal heart rhythm

    TLS can happen within 12 to 24 hours after an infusion of Rituxan. Your healthcare provider may do blood tests to check you for TLS. Your healthcare provider may give you medicine to help prevent TLS.

    Tell your healthcare provider right away if you have any of the following signs or symptoms of TLS:

    • nausea
    • vomiting
    • diarrhea
    • lack of energy
  • Serious Infections: Serious infections can happen during and after treatment with Rituxan, and can lead to death. Rituxan can increase your risk of getting infections and can lower the ability of your immune system to fight infections. Types of serious infections that can happen with Rituxan include bacterial, fungal, and viral infections. After receiving Rituxan, some people have developed low levels of certain antibodies in their blood for a long period of time (longer than 11 months). Some of these patients with low antibody levels developed infections. People with serious infections should not receive Rituxan. Tell your healthcare provider right away if you have any symptoms of infection:
    • fever
    • cold symptoms, such as runny nose or sore throat, that do not go away
    • flu symptoms, such as cough, tiredness, and body aches
    • earache or headache
    • pain during urination
    • cold sores in the mouth or throat
    • cuts, scrapes, or incisions that are red, warm, swollen, or painful
  • Heart Problems: Rituxan may cause chest pain, irregular heartbeats, and heart attack. Your healthcare provider may monitor your heart during and after treatment with Rituxan if you have symptoms of heart problems or have a history of heart problems. Tell your healthcare provider right away if you have chest pain or irregular heartbeats during treatment with Rituxan
  • Kidney Problems: especially if you are receiving Rituxan for non–Hodgkin’s lymphoma (NHL). Rituxan can cause severe kidney problems that lead to death. Your healthcare provider should do blood tests to check how well your kidneys are working
  • Stomach and Serious Bowel Problems That Can Sometimes Lead to Death: Bowel problems, including blockage or tears in the bowel, can happen if you receive Rituxan with chemotherapy medicines. Tell your healthcare provider right away if you have any stomach-area (abdomen) pain or repeated vomiting during treatment with Rituxan

Your healthcare provider will stop treatment with Rituxan if you have severe, serious, or life-threatening side effects.

What are the most common side effects during treatment with Rituxan?

  • infusion-related reactions
  • infections (may include fever, chills)
  • body aches
  • tiredness
  • nausea

In adults with GPA or MPA, the most common side effects of Rituxan also include:

  • low white and red blood cells
  • swelling
  • diarrhea
  • muscle spasms

Other side effects include:

  • aching joints during or within hours of receiving an infusion
  • more frequent upper respiratory tract infections

These are not all of the possible side effects with Rituxan.

Call your healthcare provider for medical advice about side effects. You may report side effects to the FDA at (800) FDA‐1088 or www.fda.gov/medwatch. You may also report side effects to Genentech at (888) 835‐2555.

Please see the Rituxan Prescribing Information and Medication Guide including Most Serious Side Effects for additional Important Side Effect Information.