Anti-Neutrophil Cytoplasmic Autoantibody (ANCA) Vasculitis: Causes, Symptoms, Treatment

Kidney Disorders- The following article discusses about a medical condition called Anti-Neutrophil Cytoplasmic Autoantibody (ANCA) Vasculitis. This condition causes renal dysfunction. In this article, we will discuss about various causes, symptoms, and treatment rendered for Anti-Neutrophil Cytoplasmic Autoantibody (ANCA) Vasculitis.

• Autoimmune Disease- Anti-Neutrophil Cytoplasmic Autoantibody (ANCA) Vasculitis is an autoimmune disease that results in vascular tissue inflammation and systemic vasculitis.
• Antibodies- Autoimmune response by the body is triggered by autoantibodies. Antibodies react against the antigen present in the wall of neutrophils granulocytes and blood vessels.¹
• Immune System- Antibodies are protein molecule that circulates in the blood. Antibodies are produced by the immune system of the body to fight and terminate bacteria and viruses. Protein molecules located over wall of bacteria and viruses are treated as antigen.
• Autoimmune Disease- Sometimes antibodies react against normal cells, which are part of tissue and organs. In other words antibodies start attacking own cells and triggers autoimmune disease. These antibodies are called as autoantibodies. The autoantibodies react against antigen or protein molecules of cellular wall of cells of blood vessels and neutrophils.¹
• ANCA Against Neutrophils- Anti-Neutrophil Cytoplasmic Autoantibody or ANCA are autoantibodies, which attacks certain type of white blood cells namely neutrophils (neutrophil granulocytes) and activates the white blood cells.
• The antigen and antibody reaction causes inflammatory reaction of blood vessels also known as vasculitis. Vasculitis changes the function of the blood vessel. Vasculitis causes irregular exchange of electrolytes, water and waste product in kidney.
• Anti-Neutrophil Cytoplasmic Autoantibody induces vasculitis of renal blood vessels and causes excessive leakage of blood, minerals, water and protein into urine collected by renal tubule. The disease may cause end stage renal disease and kidney failure.

• The cause of autoimmune reaction and antibody secretion is not known.
• Research has not confirmed but hypothesis is the disease is triggered by gene mutation. The malfunction of gene controlling immune system is probable cause of beginning of autoimmune antibody secretion.
• Anti-Neutrophil Cytoplasmic Autoantibody Vasculitis is caused as a result of the autoantibodies causing inflammatory vasculitis and inflammatory edema of blood vessels
• The antibody reaction with antigen of capillaries and small arteriole activates neutrophils to attack the capillaries and blood vessels resulting in vasculitis. Vasculitis of kidney vessels and glomeruli results in a medical condition called glomerulonephritis.²

Anti-Neutrophil Cytoplasmic Autoantibody Vasculitis can attack different organ structures. The different forms of it affecting the kidneys are:

• Renal Limited Vasculitis or ANCA Glomerulonephritis: In this, swelling of the blood vessels causes capillary swelling. Apart from the kidneys, no other organs are affected in this form of vasculitis.
• Microscopic Polyangiitis: In this, swelling causes injury/damage to blood vessels in numerous tissues at once. This form is usually seen in the kidneys, nerves, and lungs.

• Anti-Neutrophil Cytoplasmic Autoantibody Vasculitis is an extremely rare condition.
• It generally strikes people who are above 55 years of age.
• There is no gender bias with this disorder but research has shown it usually affects the whites more.

• Flu-like Symptoms
• Fever
• Body and muscle pains
• Fatigue and malaise
• Decreased appetite
• Unintentional weight loss.

Generalized vasculitis can cause specific symptoms related to organ involved. Most common organ involved is kidney.

Renal Vasculitis- Renal vasculitis may result in Crescent Glomerulonephritis.

• Polyuria
• Proteinuria
• Hypokalemia
• Lesion

Wegener’s Granulomatosis- Affects blood vessels of kidney and lungs. Symptoms are same as above and additional symptoms caused by lung vasculitis are as follows-

• Cough
• Rhinorrhea
• Epistaxis
• Sinusitis
• Hemoptysis
• Dyspnea
• Life-threatening pulmonary hemorrhage.

• Non-Specific symptoms
• Skin rash
• Cough
• Diarrhea
• Gastrointestinal bleeding
• Bleeding in lung
• Abdominal severe pain
• Numbness in hands and feet.

• Vasculitis of the blood vessels of the lungs
• Cough
• Hemoptysis- Coughing blood
• Dyspnea- Short of breath

• Bleeding in lungs
• Symptoms and signs of renal vasculitis.

• Physician conducts a detailed physical examination

Urine examination may show following abnormalities.

• Abnormal Creatinine Clearance
• Blood in urine
• Proteinuria
• Check and estimate excretion of Urine Total Protein
• Uric acid in urine
• Abnormal Urine concentration test
• Abnormal Specific Gravity of urine

The Following Blood Tests Are Abnormal In Case Of Glomerulonephritis:

• Blood BUN and Creatinine is increased.
• Positive serum Anti-Glomerular Basement Membrane Antibody are observed,
• Positive Antineutrophil Cytoplasmic Antibodies
• Antinuclear Antibodies is increased.
• Complement Levels are increased.
• Anti-Neutrophil Cytoplasmic Antibody (ANCA) Blood Test is increased.
• Tests to look for specific infections such as hepatitis B or hepatitis C.

Some of the radiological studies that may be done to confirm the diagnosis of vasculitis of lung, gastrointestinal system and kidney are as follows:

• CT scan of the kidney, lung and abdomen
• MRI of the kidney, lung and abdomen
• Intravenous Pyelogram

• Ultrasound study is performed to examine the kidney, lung and gastrointestinal organs to diagnose shape and external surface of the organ and hematoma (blood clots).

• Kidney biopsy and Microscopic examination is performed to confirm diagnosis of glomerulonephritis.

• Rarely bronchoscopy is performed to check the trachea, bronchi and lungs.
• The secretions of lungs are collected during bronchoscopy for lab and microscopic study.

Referral to Specialist- Referral depends on organs affected by vasculitis.

• Vasculitis of Kidney- Nephrologist
• Vasculitis of Lungs- Pulmonologist
• Vasculitis of Gastrointestinal (GI) System- GI Specialist

A steroid helps in controlling swelling and slows down the autoimmune response.⁵

• Medications like cyclophosphamide and cyclosporine prevents the immune system’s to attack itself.⁵

• These medications treat the infections that are caused as a result of vasculitis,
• Antibiotics may remove triggering bacteria and viruses from system, which are initiating Anti-Neutrophil Cytoplasmic Autoantibody formations.

• Unfortunately, there are some patients who even with definite treatment do not improve and end up having renal failure. In such cases, kidney transplant becomes an option.

When it comes to kidney transplant as a result of Anti-Neutrophil Cytoplasmic Autoantibody Vasculitis, patients may have the following queries which have been answered below:

There are instances in about 15% of people where the disease has recurred after the transplant; hence, it is imperative to make sure that Anti-Neutrophil Cytoplasmic Autoantibody disease is completely inactive prior to undergoing a kidney transplant. This significantly reduces the chances of disease recurrence after the transplant. Usually, Anti-Neutrophil Cytoplasmic Autoantibody Vasculitis symptoms may return after about five years after the kidney transplant.⁶

The treatment remains the same even after a kidney transplant, but in this case the medications may be different in order to prevent kidney rejection.

It is very rare that Anti-Neutrophil Cytoplasmic Autoantibody Vasculitis causes loss of the transplant. Research suggests that only about 7% of people lose their kidneys after transplant after approximately 10 years post transplantation due to it.

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