What Are Symptoms of ANCA Vasculitis?

Vasculitis can cause symptoms such as fever or night sweats, body aches, joint and muscle discomfort, decreased appetite, and weight loss. Joint swelling or redness is uncommon although it can affect several joints and spread from one joint to another.

In addition to these more specific symptoms, antineutrophil cytoplasmic antibody (ANCA) vasculitis can cause a wide range of symptoms, depending on the organ or body part affected, which include the following:

• When the lungs are affected
  • Breathing difficulties or lack of breath
  • Coughing up blood
  • Shortness of breath
  • Coughing (productive or nonproductive)
  • Chest pain that could get worse when inhaling deeply
• When upper airways are affected
  • Cough
  • Hoarse voice
  • Difficulty breathing
  • Shortness of breath
• When the kidneys are affected
  • Blood in the urine
  • Foamy urine (caused by protein in the urine)
  • New or worsened high blood pressure
  • Kidney disease or kidney failure can produce exhaustion, nausea/vomiting, poor appetite, and a metallic taste, but the condition usually does not present many symptoms until it is more advanced or severe.
• When the eyes are affected
  • Red eyes
  • Discomfort in the eyes
  • Hazy eyesight
  • Loss of vision in one (or both) eyes
• When the ears are affected
  • Tinnitus
  • Hearing loss
  • Infection/inflammation in the middle ear
• When nerves are affected
  • Various bodily parts may experience numbness, tingling, or weakening (caused by asymmetric peripheral neuropathy)

Antineutrophilic cytoplasmic antibody (ANCA)-linked vasculitides are a diverse collection of uncommon autoimmune diseases that result in blood vessel inflammation with varying symptoms.

ANCA consists of three disorders:

1. Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis)
2. Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome)
3. Microscopic polyangiitis

Drug-induced vasculitis and renal-limited vasculitis are other conditions linked to ANCA.

Neutrophils are a particular subset of white blood cells that ANCAs primarily target. Their primary target is the cytoplasm of neutrophils. When ANCAs (the autoantibodies) adhere to neutrophils, they attack the body's tiny arteries. This is how this illness causes vasculitis, an inflammation of the blood vessels.

When exposed to ANCAs, neutrophils (a type of white blood cell) assault small blood arteries, causing swelling and inflammation. Most people don't have these ANCAs (autoantibodies that attack small blood vessels). ANCA vasculitis can induce various symptoms depending on the organ or body part affected.

The diagnosis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is based on the presence of ANCA, clinical symptoms, and a biopsy of the frequently affected organs such as the kidneys and nasal mucosa or the lungs (which are infrequently affected).

It is suggested to perform ANCA testing through indirect immunofluorescence and antigen-specific enzyme-linked immunosorbent assays, both of which have excellent sensitivity (about 99 percent) and good specificity (approximately 70 percent).

Patients with Wegener's granulomatosis and microscopic polyangiitis are treated in three stages:

1. Induction of remission
2. Maintenance of remission
3. Therapy of relapse

Cytoxan (cyclophosphamide) and corticosteroids are frequently used as part of induction therapy nowadays. The use of high-dose intravenous methylprednisolone for three days in conjunction with oral or intravenous cyclophosphamide is advised for aggressive disease. 

Prednisone tapering dosages and cyclophosphamide maintenance for 12 to 18 months should be followed.

All medication dosages may be gradually decreased in individuals who have experienced continuous remission for 12 months. Patients who have their symptoms under good control must be closely watched every six months for signs of relapse. While using these drugs, routine complete blood counts and liver function tests should be performed.

Additional therapy regimens that may be beneficial include:

• Methotrexate
• Imuran (azathioprine)
• Bactrim, Septra (trimethoprim-sulfamethoxazole)
• Plasma exchange
• Sandimmune (cyclosporine)
• Intravenous immunoglobulin
• Monoclonal antibodies

H2 blockers or proton pump inhibitors may be administered to patients while they receive potentially ulcerogenic immunosuppressive therapy. In addition to trimethoprim-sulfamethoxazole (480 mg) three times weekly for prophylaxis of pneumocystis carinii, prophylactic treatment with Diflucan (fluconazole) orally for fungus infection may be taken into consideration.

Comorbid disorders such as hypertension, diabetes, hypercholesterolemia, and smoking should be properly treated as they accelerate vascular damage.

When a drug causes vasculitis, it must be stopped. Skin-related arthralgias and myalgias are reduced by antihistamines and nonsteroidal anti-inflammatory drugs. Severe cutaneous diseases may require oral corticosteroid therapy.