GRANULOMATOSIS WITH POLYANGIITIS &MICROSCOPIC POLYANGIITIS GPA & MPA This medical guide is designed for educational purposes to help patients understand GPA & MPA. Please consult your doctor on specific questions and details about your symptoms.
Granulomatosis with Polyangiitis & Microscopic Polyangiitis 02 03 GPA & MPA GRANULOMATOSIS WITH POLYANGIITIS & MICROSCOPIC POLYANGIITIS What is GPA & MPA? Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are different types of a rare disease called vasculitis that cause the small blood vessels in the body to become inflamed. 1-3 Both are serious conditions that can reduce blood supply to vital organs, and if left untreated, can lead to fatal organ damage. 1-3 Contents What is granulomatosis with polyangiitis (GPA) & microscopic polyangiitis (MPA)? What are the symptoms of GPA & MPA? What is the cause of GPA & MPA, and who gets GPA & MPA? How are GPA & MPA diagnosed? How are GPA & MPA treated? Living with GPA & MPA Frequently asked questions NORMAL BLOOD VESSEL Normal blood flow INFLAMED BLOOD VESSEL Decreased blood flow Vasculitis: a group of rare diseases that involves inflammation of the blood vessels (vasculitis can occur in any blood vessel in our body, including arteries, veins, and capillaries) Inflammation: the body s response to foreign and harmful substances, such as bacteria or viruses, or in some cases, self-antigens (symptoms of inflammation include heat, pain, redness, swelling)
Granulomatosis with Polyangiitis & Microscopic Polyangiitis 04 05 What are the symptoms of GPA & MPA? GPA and MPA affect various organs of the body, including the kidneys, lungs, skin, nose, ears, and the nervous system. 1,3,4 This means that they have a wide range of symptoms, such as 1,3,4 Loss of appetite and sudden weight loss Persistent, unexplained fever and a general feeling of illness Fatigue and loss of energy Rashes and skin sores Cough (with or without blood) and shortness of breath Frequent nose bleeds, runny nose, and sinus pain/infections* Inflammation of the ears and eyes that may cause hearing or vision problems* Numbness in the limbs or tingling sensations Joint and muscle pain *These symptoms are more likely to occur in patients with GPA
Granulomatosis with Polyangiitis & Microscopic Polyangiitis 06 07 What is the cause of GPA & MPA, and who gets GPA & MPA? How is GPA & MPA diagnosed? The cause of GPA and MPA is unknown. It is not contagious, does not usually run in families, and is not a form of cancer. While the cause is unknown, it is thought that the abnormal activity of the immune system causes blood vessels to swell. 5,6 Confirming the diagnosis may require a combination of physical examination, laboratory tests, imaging tests (CT or MRI scans), and biopsies. 5,7 Risk factors for GPA & MPA include: 5,6 Diagnosis of GPA & MPA 5,7 1Age Occur in people of all ages Peak age is between 40 and 60 2Gender Affect both genders equally 3Ethnicity Affect all ethnicities Physical exam To check for symptoms and signs of GPA & MPA Blood test To check for specific substances indicative of GPA & MPAs Urine test To check for red blood cells or specific indicators of GPA & MPA in the urine Imaging tests To determine which blood vessels and organs are affected through detailed images of the body with CT or MRI scans Biopsy To examine tissue samples of affected areas to confirm the presence of blood vessel swelling and abnormal cellular features Biopsy: the removal of a small amount of tissue from the body to examine under a microscope CT (computerized tomography) scan: a type of scan that uses a series of X-rays to build up a 3D picture of inside the body MRI (magnetic resonance imaging) scan: a type of scan that uses strong magnetic fields and radio waves to produce detailed images of inside the body
Granulomatosis with Polyangiitis & Microscopic Polyangiitis 08 09 How are GPA & MPA treated? The main goal of GPA & MPA treatment is to alleviate symtoms and turn off disease activity. When signs and symptoms of the disease disappear, the disease is said to be in remission. 3,4 Remission: the disappearance of signs and symptoms of the disease (when a patient is in remission, disease activity cannot be detected in the body) Treatment will vary based on your symptoms, disease activity, organ involvement and lab test results. 3,4 Medications that suppress the immune system form the foundation of treatment for GPA and MPA. 5,7 Treatment is divided into two stages: 1. Turning off disease activity (that is, reaching remission) 2. Once disease activity is turned off, keeping it turned off (maintaining remission) 3,4 Medications that suppress the immune system include rituximab*, cyclophosphamide, methotrexate, and azathioprine. These are usually prescribed in combination with corticosteroids. Once disease is improving, your doctor will slowly reduce the corticosteroid dose and eventually hope to discontinue it completely. Most patients will be treated for at least 1-2 years depending on the clinical course. 5,7 * Rituximab, especially, is considered an important advancement in the treatment of GPA and MPA. HIGH DISEASE ACTIVITY NO DISEASE ACTIVITY An effective treatment plan will include a team approach that involves multiple medical specialists according to how many of your organs are affected. 3,4
Living with GPA & MPA Frequently Asked Questions 1. Will I pass on GPA or MPA to my children? 8,9 Even after achieving remission, it is possible for GPA and MPA to recur (often referred to as a relapse ). So, during your treatment course you may go back and forth between feeling better and feeling worse. 3-5,7 To date, there is nothing to suggest that GPA and MPA are hereditary. While it is thought that genes might play a role in the occurrence of GPA and MPA, it is unusual for GPA and MPA to occur in more than one member of the same family. 2. Should I follow a particular diet? 9 The appropriate diet would depend on your individual situation. A well-balanced diet is usually sufficient. If you have high blood pressure, a low-salt diet maybe necessary. If you have renal insufficiency your doctor might put specific restrictions on protein and potassium intake. A consultation with a dietitian who can factor in your specific needs is certainly a good option. It is important that you pay attention to any new symptoms you experience and have regular doctor follow-ups to catch relapses at its earliest and most treatable stage. 3,4 80% Although GPA and MPA are serious illnesses, many patients do extremely well. On average, over 80% of patients survive GPA and MPA for 5 years or more. 5,7 3. If I have a relapse after achieving remission, it is likely that I will have further relapses? 9 Not necessarily. The pattern of relapses that each individual will experience may vary with the degree of disease and health status of a person. Some people may experience no relapses after reaching remission. Whether relapse occurs early on or long after reaching remission may differ for each patient. Additionally, while some experience just one relapse, others may experience multiple relapses. Because of this, it is difficult to see a predictable pattern for the future. You should not feel discouraged after a relapse, as that this does not mean you are always destined to have relapses in the future. Questions
REFERENCES 1. NHS Choices. Granulomatosis with Polyangiitis (Wegener s granulomatosis). Available at: http://www. nhs.uk/conditions/wegeners-granulomatosis/pages/introduction.aspx Accessed March 2017. 2. Genetics Home Reference (U.S. National Library of Medicine). Granulomatosis with polyangiitis. Available at: https://ghr.nlm.nih.gov/condition/granulomatosis-with-polyangiitis Accessed March 2017. 3. Vasculitis Foundation. Microscopic polyangiitis. Available at: http://www.vasculitisfoundation.org/education/forms/microscopic-polyangiitis/ Accesed March 2017. 4. Vasculitis Foundation. Granulomatosis with polyangiitis (GPA/ Wegener s). Available at: http://www. vasculitisfoundation.org/education/forms/granulomatosis-with-polyangiitis-gpa-wegeners/ Accessed March 2017. 5. Cleveland Clinic. Wegener s Granulomatosis (GPA). Available at: http://my.clevelandclinic.org/health/ articles/wegeners-granulomatosis Accessed March 2017. 6. The Johns Hopkins Vasculitis Center. Microscopic polyangiitis. Available at: http://www.hopkinsvasculitis. org/types-vasculitis/microscopic-polyangiitis/ Accessed March 2017. 7. Cleveland Clinic. Microscopic polyangiitis. Available at: http://my.clevelandclinic.org/health/articles/microscopic-polyangitis Accessed March 2017. 8. The Johns Hopkins Vasculitis Center. Frequently Asked Questions About Vasculitis. Available at: https:// www.hopkinsvasculitis.org/resources/vasculitis-faq/#family Accessed March 2017. 9. Vasculitis Foundation. Frequently Asked Questions. Available at: http://www.vasculitisfoundation.org/ education/faqs/ Accessed March 2017. Celltrion Healthcare Co., Ltd. 2017 HCTRU-00 PB-0317/04