What is ANCA-associated vasculitis?

ANCA-associated vasculitis (AAV) encompasses rare autoimmune diseases causing inflammation and damage within small- to medium-sized blood vessels. The abbreviation "ANCA", as it applies here, stands for Anti-Neutrophil Cytoplasmic Antibodies—i.e., antibodies which, in their abnormal state, attack the healthy white blood cells of the organism, provoking inflammation of the blood vessels. AAV primarily deals with organs such as the kidneys, lungs, skin, nerves, and sinuses, but can migrate to almost any organ.

Kidney involvement in AAV can rapidly become progressive, culminating with glomerulonephritis in which the glomeruli, the tiny essential filters of the kidneys, become inflamed and subsequently scarred, with impairment of renal function. Without treatment, AAV can lead to either kidney failure or damage to several organs.

Type of ANCA

Three main types of ANCA-associated vasculitides are:-

• Granulomatosis with polyangiitis (GPA) – previously known as Wegener's granulomatosis
• Microscopic polyangiitis (MPA)
• Eosinophilic granulomatosis with polyangiitis (EGPA) – previously called Churg-Strauss syndrome

What are the symptoms of ANCA-associated vasculitis?

The following are the symptoms that may vary depending on which organ systems are affected:-

General Symptoms:

• Fatigue
• Fever
• Weight loss
• Loss of appetite
• Muscles and joint pains.

Kidney Involvement:

• Blood in urine (hematuria).
• Foamy urine (proteinuria).
• Decreased urine output.
• Swelling in the feet, legs, or around the eyes.
• High blood pressure.

Respiratory Involvement:

• Sinus infections or nosebleeds.
• Coughing or shortness of breath.
• Coughing up blood.

Skin/Nerves:

• Rash or purpura (purple spots).
• Numbness or tingling in hands/feet.

What causes of ANCA-associated vasculitis?

The precise cause of ANCA-associated vasculitis is not entirely understood. Still, it is now accepted to be an autoimmune process. Several factors may trigger the immune system to produce ANCAs:-

• Genetic predisposition,
• Environmental factors (e.g. infectious agents, exposure to silica),
• Specific categories of drugs,
• Immune system dysfunction.

Once ANCAs become available after their production, they activate white blood cells, which destroy the endothelium of the blood vessels, thereby triggering inflammation and organ damage.

What is the Diagnosis & Tests in ANCA-associated vasculitis?

Early diagnosis becomes vital to avert any permanent damage to the organs, specifically the kidneys. The approach to diagnosis combines clinical evaluation, laboratory evaluation, and imaging.

Blood Tests

• ANCA Test - Detects antibodies specific to AAV (c-ANCA and p-ANCA).
• Creatinine & BUN - Assess kidney function.
• EGFR (estimated Glomerular Filtration Rate) - Evaluates kidney function.
• CRP and ESR - These are markers of inflammation.

Complete blood count may reveal anaemia or a white blood cell increase.

Urine Tests

• Tests for blood and protein are indicative of kidney damage.

Imaging Studies

• Chest X-ray or CT scan - Looking for nodules, bleeding, or inflammation of the lungs.
• X-rays or scans of the sinuses, if symptoms exist regarding the sinuses.

Biopsy

• The kidney biopsy or the affected tissue is regularly needed to confirm the diagnosis and estimate the level of damage.

How to manage and treat ANCA- associated vasculitis?

ANCA-associated vasculitis is among the rare, serious, but treatable diseases, and management includes:-

1. Induction Therapy

The initial intensive treatment aims at achieving remission of the disease:-

• Corticosteroids (e.g., prednisone) for inflammation.
• Immunosuppressants:
  • Cyclophosphamide – Conventional agent used for severe cases.
  • Rituximab – A targeted therapy used with efficacy for GPA and MPA.
  • Methotrexate – Used for milder cases.

2. Maintenance Therapy

Once remission has occurred, the preventive treatment for relapse continues:-

• Azathioprine, Methotrexate, or Rituximab
• Tapering corticosteroids gradually

3. Supportive Treatment

• Drugs for high blood pressure.
• Dialysis if kidney failure occurs.
• Calcium and vitamin D to protect bones from steroid effects.
• Prevention of infections by vaccination and antibiotics (e.g. against pneumocystis pneumonia).

4. Monitoring

• Regularly monitoring kidney function, ANCA, and medication side effects is critical.
• Regular blood work and urinalysis.

What is the Prognosis for ANCA- associated vasculitis?

Prognosis for AAV has dramatically improved over the last 20 years owing to early diagnosis and efficient treatment.

• Remission is available in 75–90% of patients with proper treatment.
• Relapses, however, are common and occur in 30–50% of patients.
• There may be long-standing effects on the kidneys if the diagnosis is delayed or severe.
• Patients will often need monitoring for life.

If not given treatment, AAV could be life-threatening due to kidney failure, bleeding in the lungs, or damage to other organs.

How to prevent from ANCA- associated vasculitis?

There is no way to prevent AAV entirely; however, the following measures can help reduce complications:-

• Early detection or diagnosis of symptoms and prompt medical evaluation
• Regular checkups if you have a known autoimmune disorder/issue or a family history of autoimmune diseases
• Avoid unfavourable environmental exposure, such as dust or chemicals
• Adherence to medications and follow-up appointments to detect relapses early
• Promptly treat infections, which can trigger flares

Living With

Living with ANCA-associated vasculitis entails living with a changed lifestyle, emotional adjustments, and medical assistance.

Tips to Live Well with AAV:-

• Stay informed - Know about your condition and treatment.
• Follow your treatment plan - Never miss taking your medications.
• Be on a strict kidney-friendly, low-sodium diet if affected.
• Get Physical - Light exercise is tolerable, but maintain strength and mood.
• No Smoking, and Limit Alcohol - Both are bad for blood vessels and kidneys.
• Stress Management - Try meditation, counselling, or support groups.
• Monitor symptoms - Inform your doctor immediately of any new signs.
• Protection from infections - Keep updated with vaccines.

Is ANCA-associated vasculitis curable?

It is not curable, but AAV is treatable. Most patients achieve remission, leading an everyday life with proper medical supervision.

Can ANCA vasculitis involve other organs than the kidneys?

Yes. Depending on the type and severity, it can affect the lungs, skin, nerves, joints, sinuses, and much more.

Is AAV inherited?

No. AAV does not pass directly through inheritance without genetic factors having some minimal role.

Why choose Tender Palm Super Speciality Hospital for ANCA-Associated Vasculitis treatment in Lucknow, India?

Tender Palm Super-Speciality Hospital has the most trusted team of Nephrologists with advanced diagnostic equipment care for ANCA-Associated Vasculitis treatment in Lucknow, India. Our Nephrology department follows international safety standards and has years of experience in successfully managing disease and conditions like ANCA-Associated Vasculitis.

To seek an Expert Consultation for ANCA-Associated Vasculitis treatment in Lucknow, India:

Call us at +91-9076972161
Email at care@tenderpalm.com

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