BACK TOTOP Browse A-ZSearchBrowse A-ZABCDEFGHIJKLMNOPQRSTUVWXYZ0-9 E-mail FormEmail ResultsName:Email address:Recipients Name:Recipients address:Message: Print-FriendlyBookmarksbookmarks-menuNecrotizing vasculitisNecrotizing vasculitis is a group of disorders that involve inflammation of the blood vessel walls. The size of the affected blood vessels helps to determine the names of these conditions and how the disorder causes disease. Causes Necrotizing vasculitis may be the primary condition such as in people with polyarteritis nodosa or granulomatosis with ANCA associated vasculitis (formerly called Wegener granulomatosis). In other cases, the vasculitis may occur as part of another disorder, such as systemic lupus erythematosus or hepatitis C.Polyarteritis nodosaPolyarteritis nodosa is a serious inflammatory blood vessel disease. The small and medium-sized arteries become swollen and damaged.ImageRead Article Now Book Mark Article Granulomatosis with ANCA associated vas...Granulomatosis with polyangiitis (GPA) is a rare disorder in which blood vessels become inflamed. This leads to damage in major organs of the body. ...ImageRead Article Now Book Mark Article The cause of the inflammation is unknown. It is likely related to autoimmune factors. The wall of the blood vessel may scar and thicken or die (become necrotic). The blood vessel may close, interrupting blood flow to the tissues it supplies. The lack of blood flow will cause the tissues to die. Sometimes the blood vessel may break and bleed (rupture).AutoimmuneAn autoimmune disorder occurs when the body's immune system attacks and destroys healthy body tissue by mistake. There are more than 80 autoimmune d...ImageRead Article Now Book Mark Article NecroticNecrosis is the death of body tissue. It occurs when too little blood flows to the tissue. This can be from injury, radiation, or chemicals. Necro...ImageRead Article Now Book Mark Article Necrotizing vasculitis may affect blood vessels in any part of the body. Therefore, it can cause problems in the skin, brain, lungs, intestines, kidney, brain, joints or any other organ. Symptoms Fever, chills, fatigue, arthritis, or weight loss may be the only symptoms at first. However, symptoms may be in almost any part of the body.ChillsChills refers to feeling cold after being in a cold environment. The word can also refer to an episode of shivering along with paleness and feeling ...ImageRead Article Now Book Mark Article FatigueFatigue is a feeling of weariness, tiredness, or lack of energy.ImageRead Article Now Book Mark Article ArthritisArthritis is inflammation or degeneration of one or more joints. A joint is the area where 2 bones meet. There are more than 100 different types of...ImageRead Article Now Book Mark Article Skin:Red or purple colored bumps on the legs, hands or other parts of the body Bluish color to the fingers and toes Signs of tissue death due to lack of oxygen such as pain, redness, darkening of skin tone, and ulcers that do not healMuscles and joints:Joint pain Joint painJoint pain can affect one or more joints.ImageRead Article Now Book Mark Article Leg pain Muscle weaknessBrain and nervous system:Pain, numbness, tingling in an arm, leg, or other body area NumbnessNumbness and tingling are abnormal sensations that can occur anywhere in your body, but they are often felt in your fingers, hands, feet, arms, or le...ImageRead Article Now Book Mark Article Weakness of an arm, leg, or other body area Pupils that are different sizes Eyelid drooping Swallowing difficulty Swallowing difficultyDifficulty with swallowing is the feeling that food or liquid is stuck in the throat or at any point before the food enters the stomach. This proble...ImageRead Article Now Book Mark Article Speech impairment Speech impairmentSpeech and language impairment may be any of several problems that make it difficult to communicate.Read Article Now Book Mark Article Movement difficultyLungs and respiratory tract:Cough Shortness of breath Shortness of breathBreathing difficulty may involve:Difficult breathing Uncomfortable breathingFeeling like you are not getting enough airImageRead Article Now Book Mark Article Sinus congestion and pain Coughing up blood or bleeding from the noseOther symptoms include:Abdominal pain Abdominal painAbdominal pain is pain that you feel anywhere between your chest and groin. This is often referred to as the stomach region or belly.ImageRead Article Now Book Mark Article Blood in the urine or stools Blood in the urineBlood in your urine is called hematuria. The amount may be very small and only detected with urine tests or under a microscope. In other cases, the...ImageRead Article Now Book Mark Article StoolsBlack or tarry stools with a foul smell are a sign of a problem in the upper digestive tract. It most often indicates that there is bleeding in the ...ImageRead Article Now Book Mark Article Hoarseness or changing voice Chest pain from damage of the arteries that supply the heart (coronary arteries) Exams and Tests The health care provider will do a complete physical exam. A nervous system (neurological) exam may show signs of nerve damage.Tests that may be done include:Complete blood count Complete blood countA complete blood count (CBC) test measures the following:The number of white blood cells (WBC count)The number of red blood cells (RBC count)The numb...ImageRead Article Now Book Mark Article Comprehensive metabolic panel Chest x-ray Chest x-rayA chest x-ray is an x-ray of the chest, lungs, heart, large arteries, ribs, and diaphragm.ImageRead Article Now Book Mark Article C-reactive protein test Erythrocyte sedimentation rate Erythrocyte sedimentation rateESR stands for erythrocyte sedimentation rate. It is commonly called a "sed rate. "It is a test that indirectly measures the level of certain protei...ImageRead Article Now Book Mark Article Hepatitis blood test HepatitisHepatitis is swelling and inflammation of the liver.ImageRead Article Now Book Mark Article Urinalysis UrinalysisUrinalysis is the physical, chemical, and microscopic examination of urine. It involves a number of tests to detect and measure various compounds th...ImageRead Article Now Book Mark Article Blood test for antibodies against neutrophils (ANCA antibodies) or nuclear antigens (ANA) Blood test for cryoglobulins Blood test for complement levels ComplementComplement is a blood test that measures the activity of certain proteins in the liquid portion of your blood. The complement system is a group of ne...ImageRead Article Now Book Mark Article Imaging studies such as angiogram, ultrasound, computed tomography (CT) scan, or magnetic resonance imaging (MRI) AngiogramAn arteriogram is an imaging test that uses x-rays and a special dye to see inside the arteries. It can be used to view arteries in the heart, brain...ImageRead Article Now Book Mark Article Biopsy of the skin, muscle, organ tissue, or nerveBiopsyA muscle biopsy is the removal of a small piece of muscle tissue for examination.ImageRead Article Now Book Mark Article NerveA nerve biopsy is the removal of a small piece of a nerve for examination.ImageRead Article Now Book Mark Article Treatment Corticosteroids are given in most cases. The dose will depend on how bad the condition is.Other medicines that suppress the immune system may reduce inflammation of the blood vessels. These include azathioprine, methotrexate, and mycophenolate. These medicines are often used along with corticosteroids. This combination makes it possible to control the disease with a lower dose of corticosteroids.For severe disease, cyclophosphamide (Cytoxan) has been used for many years. However, rituximab (Rituxan) is equally effective and is less toxic.Recently, tocilizumab (Actemra) was shown to be effective for giant cell arteritis so the dose of corticosteroids could be reduced. Outlook (Prognosis) Necrotizing vasculitis can be serious and life-threatening disease. The outcome depends on the location of the vasculitis and the severity of tissue damage. Complications may occur from the disease and from the medicines. Most forms of necrotizing vasculitis require long-term follow-up and treatment. Possible Complications Complications may include:Permanent damage to the structure or function of the affected area Secondary infections of necrotic tissues Secondary infectionsA secondary infection is an infection that occurs during or after treatment for another infection. It may be caused by the first treatment or by cha...ImageRead Article Now Book Mark Article Side effects from medicines used for treatment When to Contact a Medical Professional Contact your provider if you have symptoms of necrotizing vasculitis.Emergency symptoms include:Problems in more than one part of the body such as stroke, arthritis, severe skin rash, abdominal pain or coughing up blood Changes in pupil size Loss of function of an arm, leg, or other body part Speech problems Swallowing difficulty Weakness Severe abdominal pain Prevention There is no known way to prevent this disorder.Open ReferencesReferencesChung SA, Monach PA. Anti-neutrophil cytoplasmic antibody–associated vasculitis. In: Firestein GS, McInnes IB, Koretzky GA, Mikuls TR, Neogi T, O'Dell JR, eds. Firestein & Kelley's Textbook of Rheumatology. 12th ed. Philadelphia, PA: Elsevier; 2025:chap 90.Dinulos JGH. Hypersensitivity syndromes and vasculitis. In: Dinulos JGH, ed. Habif's Clinical Dermatology. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 18.Free M, Jennette JC, Falk RJ, Jain K. Renal and systemic vasculitis. In: Johnson RJ, Floege J, Tonelli M, eds. Comprehensive Clinical Nephrology. 7th ed. Philadelphia, PA: Elsevier; 2024:chap 26.Rhee RL, Merkel PA. Classification and epidemiology of systemic vasculitis. In: Firestein GS, McInnes IB, Koretzky GA, Mikuls TR, Neogi T, O'Dell JR, eds. Firestein & Kelley's Textbook of Rheumatology. 12th ed. Philadelphia, PA: Elsevier; 2025:chap 88.Stone JH. The systemic vasculitides. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 249.AllVideoImagesTogCirculatory system - illustration Blood used by the body is brought back to the heart and lungs by the veins of the body. Once the blood has gathered more oxygen from the lungs, it is pumped back out to the body through the arteries.Circulatory systemillustrationCirculatory system - illustration Blood used by the body is brought back to the heart and lungs by the veins of the body. Once the blood has gathered more oxygen from the lungs, it is pumped back out to the body through the arteries.Circulatory systemillustrationRelated Information Proliferation(Special Topic)Rheumatoid arthritis(Condition)Systemic lupus erythematosus(Condition)Polyarteritis nodosa(Condition)Scleroderma(Condition)Necrosis(Special Topic)Secondary infections(Special Topic)Rheumatoid arthritis - InDepth(In-Depth)Systemic lupus erythematosus - InDepth(In-Depth)Scleroderma - InDepth(In-Depth) Review Date: 4/1/2025 Reviewed By: Diane M. Horowitz, MD, Rheumatology and Internal Medicine, Northwell Health, Great Neck, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. No warranty of any kind, either expressed or implied, is made as to the accuracy, reliability, timeliness, or correctness of any translations made by a third-party service of the information provided herein into any other language. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited. © 1997- All rights reserved. A.D.A.M. content is best viewed in IE9 or above, Firefox and Google Chrome browser.Content is best viewed in IE9 or above, Firefox and Google Chrome browser.
Necrotizing vasculitisNecrotizing vasculitis is a group of disorders that involve inflammation of the blood vessel walls. The size of the affected blood vessels helps to determine the names of these conditions and how the disorder causes disease. Causes Necrotizing vasculitis may be the primary condition such as in people with polyarteritis nodosa or granulomatosis with ANCA associated vasculitis (formerly called Wegener granulomatosis). In other cases, the vasculitis may occur as part of another disorder, such as systemic lupus erythematosus or hepatitis C.Polyarteritis nodosaPolyarteritis nodosa is a serious inflammatory blood vessel disease. The small and medium-sized arteries become swollen and damaged.ImageRead Article Now Book Mark Article Granulomatosis with ANCA associated vas...Granulomatosis with polyangiitis (GPA) is a rare disorder in which blood vessels become inflamed. This leads to damage in major organs of the body. ...ImageRead Article Now Book Mark Article The cause of the inflammation is unknown. It is likely related to autoimmune factors. The wall of the blood vessel may scar and thicken or die (become necrotic). The blood vessel may close, interrupting blood flow to the tissues it supplies. The lack of blood flow will cause the tissues to die. Sometimes the blood vessel may break and bleed (rupture).AutoimmuneAn autoimmune disorder occurs when the body's immune system attacks and destroys healthy body tissue by mistake. There are more than 80 autoimmune d...ImageRead Article Now Book Mark Article NecroticNecrosis is the death of body tissue. It occurs when too little blood flows to the tissue. This can be from injury, radiation, or chemicals. Necro...ImageRead Article Now Book Mark Article Necrotizing vasculitis may affect blood vessels in any part of the body. Therefore, it can cause problems in the skin, brain, lungs, intestines, kidney, brain, joints or any other organ. Symptoms Fever, chills, fatigue, arthritis, or weight loss may be the only symptoms at first. However, symptoms may be in almost any part of the body.ChillsChills refers to feeling cold after being in a cold environment. The word can also refer to an episode of shivering along with paleness and feeling ...ImageRead Article Now Book Mark Article FatigueFatigue is a feeling of weariness, tiredness, or lack of energy.ImageRead Article Now Book Mark Article ArthritisArthritis is inflammation or degeneration of one or more joints. A joint is the area where 2 bones meet. There are more than 100 different types of...ImageRead Article Now Book Mark Article Skin:Red or purple colored bumps on the legs, hands or other parts of the body Bluish color to the fingers and toes Signs of tissue death due to lack of oxygen such as pain, redness, darkening of skin tone, and ulcers that do not healMuscles and joints:Joint pain Joint painJoint pain can affect one or more joints.ImageRead Article Now Book Mark Article Leg pain Muscle weaknessBrain and nervous system:Pain, numbness, tingling in an arm, leg, or other body area NumbnessNumbness and tingling are abnormal sensations that can occur anywhere in your body, but they are often felt in your fingers, hands, feet, arms, or le...ImageRead Article Now Book Mark Article Weakness of an arm, leg, or other body area Pupils that are different sizes Eyelid drooping Swallowing difficulty Swallowing difficultyDifficulty with swallowing is the feeling that food or liquid is stuck in the throat or at any point before the food enters the stomach. This proble...ImageRead Article Now Book Mark Article Speech impairment Speech impairmentSpeech and language impairment may be any of several problems that make it difficult to communicate.Read Article Now Book Mark Article Movement difficultyLungs and respiratory tract:Cough Shortness of breath Shortness of breathBreathing difficulty may involve:Difficult breathing Uncomfortable breathingFeeling like you are not getting enough airImageRead Article Now Book Mark Article Sinus congestion and pain Coughing up blood or bleeding from the noseOther symptoms include:Abdominal pain Abdominal painAbdominal pain is pain that you feel anywhere between your chest and groin. This is often referred to as the stomach region or belly.ImageRead Article Now Book Mark Article Blood in the urine or stools Blood in the urineBlood in your urine is called hematuria. The amount may be very small and only detected with urine tests or under a microscope. In other cases, the...ImageRead Article Now Book Mark Article StoolsBlack or tarry stools with a foul smell are a sign of a problem in the upper digestive tract. It most often indicates that there is bleeding in the ...ImageRead Article Now Book Mark Article Hoarseness or changing voice Chest pain from damage of the arteries that supply the heart (coronary arteries) Exams and Tests The health care provider will do a complete physical exam. A nervous system (neurological) exam may show signs of nerve damage.Tests that may be done include:Complete blood count Complete blood countA complete blood count (CBC) test measures the following:The number of white blood cells (WBC count)The number of red blood cells (RBC count)The numb...ImageRead Article Now Book Mark Article Comprehensive metabolic panel Chest x-ray Chest x-rayA chest x-ray is an x-ray of the chest, lungs, heart, large arteries, ribs, and diaphragm.ImageRead Article Now Book Mark Article C-reactive protein test Erythrocyte sedimentation rate Erythrocyte sedimentation rateESR stands for erythrocyte sedimentation rate. It is commonly called a "sed rate. "It is a test that indirectly measures the level of certain protei...ImageRead Article Now Book Mark Article Hepatitis blood test HepatitisHepatitis is swelling and inflammation of the liver.ImageRead Article Now Book Mark Article Urinalysis UrinalysisUrinalysis is the physical, chemical, and microscopic examination of urine. It involves a number of tests to detect and measure various compounds th...ImageRead Article Now Book Mark Article Blood test for antibodies against neutrophils (ANCA antibodies) or nuclear antigens (ANA) Blood test for cryoglobulins Blood test for complement levels ComplementComplement is a blood test that measures the activity of certain proteins in the liquid portion of your blood. The complement system is a group of ne...ImageRead Article Now Book Mark Article Imaging studies such as angiogram, ultrasound, computed tomography (CT) scan, or magnetic resonance imaging (MRI) AngiogramAn arteriogram is an imaging test that uses x-rays and a special dye to see inside the arteries. It can be used to view arteries in the heart, brain...ImageRead Article Now Book Mark Article Biopsy of the skin, muscle, organ tissue, or nerveBiopsyA muscle biopsy is the removal of a small piece of muscle tissue for examination.ImageRead Article Now Book Mark Article NerveA nerve biopsy is the removal of a small piece of a nerve for examination.ImageRead Article Now Book Mark Article Treatment Corticosteroids are given in most cases. The dose will depend on how bad the condition is.Other medicines that suppress the immune system may reduce inflammation of the blood vessels. These include azathioprine, methotrexate, and mycophenolate. These medicines are often used along with corticosteroids. This combination makes it possible to control the disease with a lower dose of corticosteroids.For severe disease, cyclophosphamide (Cytoxan) has been used for many years. However, rituximab (Rituxan) is equally effective and is less toxic.Recently, tocilizumab (Actemra) was shown to be effective for giant cell arteritis so the dose of corticosteroids could be reduced. Outlook (Prognosis) Necrotizing vasculitis can be serious and life-threatening disease. The outcome depends on the location of the vasculitis and the severity of tissue damage. Complications may occur from the disease and from the medicines. Most forms of necrotizing vasculitis require long-term follow-up and treatment. Possible Complications Complications may include:Permanent damage to the structure or function of the affected area Secondary infections of necrotic tissues Secondary infectionsA secondary infection is an infection that occurs during or after treatment for another infection. It may be caused by the first treatment or by cha...ImageRead Article Now Book Mark Article Side effects from medicines used for treatment When to Contact a Medical Professional Contact your provider if you have symptoms of necrotizing vasculitis.Emergency symptoms include:Problems in more than one part of the body such as stroke, arthritis, severe skin rash, abdominal pain or coughing up blood Changes in pupil size Loss of function of an arm, leg, or other body part Speech problems Swallowing difficulty Weakness Severe abdominal pain Prevention There is no known way to prevent this disorder.Open ReferencesReferencesChung SA, Monach PA. Anti-neutrophil cytoplasmic antibody–associated vasculitis. In: Firestein GS, McInnes IB, Koretzky GA, Mikuls TR, Neogi T, O'Dell JR, eds. Firestein & Kelley's Textbook of Rheumatology. 12th ed. Philadelphia, PA: Elsevier; 2025:chap 90.Dinulos JGH. Hypersensitivity syndromes and vasculitis. In: Dinulos JGH, ed. Habif's Clinical Dermatology. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 18.Free M, Jennette JC, Falk RJ, Jain K. Renal and systemic vasculitis. In: Johnson RJ, Floege J, Tonelli M, eds. Comprehensive Clinical Nephrology. 7th ed. Philadelphia, PA: Elsevier; 2024:chap 26.Rhee RL, Merkel PA. Classification and epidemiology of systemic vasculitis. In: Firestein GS, McInnes IB, Koretzky GA, Mikuls TR, Neogi T, O'Dell JR, eds. Firestein & Kelley's Textbook of Rheumatology. 12th ed. Philadelphia, PA: Elsevier; 2025:chap 88.Stone JH. The systemic vasculitides. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 249.
