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Microscopic Polyangiitis

By

Alexandra Villa-Forte

, MD, MPH, Cleveland Clinic

Last full review/revision Sep 2020| Content last modified Sep 2020
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NOTE: This is the Consumer Version. DOCTORS: Click here for the Professional Version
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Topic Resources

Microscopic polyangiitis is inflammation of mainly small blood vessels throughout the body.

  • Symptoms vary depending on which organs are affected.

  • People have a fever, lose weight, and have achy muscles and joints, as well as various other symptoms.

  • Blood and urine tests are done, and biopsy is done to confirm the diagnosis.

  • Treatment depends on disease severity but includes corticosteroids and drugs that suppress the immune system.

Microscopic polyangiitis is rare. It can occur at any age. The cause of microscopic polyangiitis is unknown. People with this disorder usually have abnormal antibodies called antineutrophil cytoplasmic antibodies in their blood.

Symptoms

Most people with microscopic polyangiitis have a fever, feel tired, and lose weight. Muscles and joints often ache.

Various organs may be affected:

  • Kidneys: The kidneys are affected in up to 90% of people. Blood, protein, and red blood cells appear in the urine, but often there is no sign of kidney malfunction until it is severe. Kidney failure may develop rapidly unless diagnosis and treatment are prompt.

  • Respiratory tract: If the lungs are affected, bleeding in the lungs may occur, causing people to cough up blood, feel short of breath, or both. The lungs may fill with fluid, and scar tissue may eventually develop. Fluid buildup and scar tissue cause difficulty breathing. Bleeding in the lungs, which may occur early in the disorder, requires immediate medical attention.

  • Skin: About one third of people have a rash of reddish purple spots and bumps, usually on the legs, feet, or buttocks. The nails may contain thin purplish lines, indicating bleeding (called splinter hemorrhages). Rarely, the blood supply to the fingers and toes is reduced.

  • Digestive tract: Abdominal pain, nausea, vomiting, and diarrhea may occur. Stools may contain blood.

  • Nerves: People may have tingling, numbness, or weakness in a limb.

Other organs, such as the heart, are affected less often.

Diagnosis

  • Doctor's evaluation

  • Blood and urine tests

  • Biopsy

  • Sometimes chest imaging

Doctors suspect microscopic polyangiitis based on symptoms.

Blood and urine tests are done. These tests cannot specifically identify the disorder but can confirm that inflammation is present. Blood tests can also help doctors detect bleeding in the digestive tract. Blood is tested for abnormal antibodies, such as antineutrophil cytoplasmic antibodies (ANCA), which attack certain white blood cells. The erythrocyte sedimentation rate (ESR) and levels of C-reactive protein, white blood cells, and platelets can be very high, indicating active inflammation. The level of red blood cells can be very low, indicating severe anemia due to bleeding in the lungs. A sample of urine is tested for red blood cells and protein. This information can help doctors determine whether the kidneys are affected.

A biopsy of affected tissue (usually the skin, lungs, or kidneys) is done to confirm the diagnosis.

Chest imaging is done in people who have respiratory tract symptoms. Computed tomography (CT) is much more likely than a chest x-ray to reveal small amounts of bleeding in the lungs. If there are signs of bleeding, a flexible viewing tube is inserted through the nose or mouth into the airways to directly view the lungs (bronchoscopy). This procedure can confirm the presence of bleeding (or infection, another possible cause of respiratory tract symptoms).

Treatment

  • For mild symptoms, corticosteroids and methotrexate

  • For severe symptoms, cyclophosphamide or rituximab

  • Sometimes plasma exchange

If symptoms of microscopic polyangiitis are mild, a corticosteroid plus methotrexate, which is another drug that suppresses the immune system (immunosuppressant), are given.

If symptoms are severe and vital organs are affected, cyclophosphamide, a stronger immunosuppressant, or rituximab and high doses of a corticosteroid are given.

To induce remission, which is a period where people have no symptoms, sometimes doctors do plasma exchange (plasmapheresis) or give the corticosteroid methylprednisolone by vein (intravenously). Plasmapheresis is a process in which blood is taken from the person and put through a machine that separates the blood cells from the liquid part of the blood (plasma). The plasma, which contains the disease-causing proteins, is discarded and the blood cells are returned to the person.

More Information

The following English-language resource may be useful. Please note that THE MANUAL is not responsible for the content of this resource.

  • Vasculitis Foundation: Provides information for patients about vasculitis, including how to find a doctor, learn about research studies, and join patient advocacy groups

NOTE: This is the Consumer Version. DOCTORS: Click here for the Professional Version
Click here for the Professional Version
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