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Granulomatosis with polyangiitis

What is granulomatosis with polyangiitis?

Granulomatosis with polyangiitis is a rare but life-threatening form of vasculitis. There is an immune reaction in which antibodies small damage blood vessel surrounding walls and tissues.

Multiple organs are often involved. The most commonly affected sites are the ears, nose, throat, lungs, eyes, and kidneys. Mild forms can occur without kidney involvement.

Granulomatosis with polyangiitis was previously known as Wegener's granulomatosis.

Who gets granulomatosis with polyangiitis?

Granulomatosis with polyangiitis affects both sexes equally. It occurs in patients of all ages with a mean age of 41 years. It occurs more commonly in white patients than in blacks or Asians.

What causes granulomatosis with polyangiitis?

The cause of granulomatosis with polyangiitis is unknown. It is classified as a autoimmune cytoplasmic cytoplasmic disease and cytoplasm antibody (C-HAUNCH) directed against serine proteinase three antigen (PR3-ANCA) is believed to be pathogen. It can be caused by a bacterial infection.

Genetic mutations may be involved, but granulomatosis with polyangiitis is not inherited.

What are the clinical characteristics of granulomatosis with polyangiitis?

Systemic symptoms

Granulomatosis patients with polyangiitis may complain of several non-specific symptoms, especially:

  • Fever affects 25% –50%
  • Weight loss of> 10% from usual body weight affects 15–35%.

Upper airway disease

Symptoms affecting the ear, nose, and throat are common and affect the 70% of patients at baseline and the 92% of patients during granulomatosis with polyangiitis.

Manifestations of granulomatosis with polyangiitis in the ear, nose, and throat include:

  • Sinusitis, nosebleeds, or nasal deformity
  • Earache or hearing loss (otitis media)
  • Painful mouth ulcersgum disease (strawberry gums),
  • Subglottic stenosis (narrowing of the airways), which occurs with hoarseness, stridor (noisy breathing), or life-threatening airway obstruction.
Lip ulceration in granulomatosis with polyangiitis


Lip ulceration due to granulomatosis with polyangiitis

Lung disease

Pulmonary involvement occurs in 45% of patients with granulomatosis with polyangiitis at presentation and in 87% during disease, which often results in:

  • Cough
  • Hemoptysis (coughing up blood)
  • Chest pain (pleurisy).

Chest x-rays can be abnormal, either showing diffuse disease (67%) or isolated areas (58%).


Renal Involvement occurs in 20% of patients at presentation and in 85% during granulomatosis with polyangiitis. It can manifest as oliguria (decreased urine output) and hematuria (blood in the urine).

Close monitoring of kidney function is required for all patients (blood and urine tests).

Eye disease

Ocular symptoms occur in 28 to 58% of patients with granulomatosis with polyangiitis and are part of the initial presentation in 8 to 16% of patients. The most common problems are:

  • Proptosis (bulging of the eye)
  • Pain (which can be very severe)
  • Loss of vision

Skin illness

The skin may be affected in 40-50% of patients with granulomatosis with polyangiitis. Skin problems include:

  • Ulcers
  • Palpable purple (raised dark red spots due to small vessel vasculitis)
  • Nodules, papules and vesicles (small blisters)
  • Pyoderma gangrenous (rare)
  • Raynaud's phenomenon (white / purple fingers when exposed to cold) is rare.

Muscles and joints

Musculoskeletal problems occur in 30 to 50% of patients at presentation and in 65 to 75% of patients during illness. These include:

  • Muscle pain (myalgia)
  • Aching joints (arthralgia)
  • Arthritis.

Nervous system

Neurological Symptoms are seldom a presenting feature, but may occur in 22-50% of patients during granulomatosis with polyangiitis. Peripheral nerves They are mainly affected, leading to loss or abnormal sensations or weakness of some muscles. Central nervous system compromise (i.e. the brain and spinal cord cable) occur in <10% de los casos.

Gastrointestinal tract

The exact frequency of gastrointestinal symptoms in granulomatosis with polyangiitis is unknown. The symptoms are more frequent:

  • Abdominal pain
  • Diarrhea
  • Bleeding


Heart problems occur in 12-30% of granulomatosis patients with polyangiitis. These include:

  • Inflamed cardiac sac (pericarditis)
  • Angina pectoris (cardiac ischemia)
  • Palpitations (irregular heartbeat).

How is granulomatosis with polyangiitis diagnosed?

Diagnosis of granulomatosis with polyangiitis is not always straightforward.

Blood count abnormalities in granulomatosis with polyangiitis may include:

  • High white blood cell count (leukocytosis)
  • Reduced hemoglobin (normocytic normochromic anemia)
  • High platelet count (thrombocytosis)
  • High erythrocytes sedimentation rate (ESR)

Antineutrophil cytoplasmic antibody (ANCA) is the most useful blood test. One particular type, c-ANCA is highly specific for polyangiitis granulomatosis and affects 70% in patients with the disease. It is recognized by the cytoplasmic staining pattern and is directed against a enzyme, proteinase-3. Protoplasm staining perinuclear neutrophil cytoplasmic antibodiesCalled p-ANCA, it occurs in the 70% of people with granulomatosis with polyangiitis.

A positive test result for rheumatoid factor occurs in 50-60% of patients with polyangiitis granulomatosis and can be misleading because this normally indicates rheumatoid arthritis.

A skin biopsy can be useful if you demonstrate granulomatous vasculitis (inflamed blood vessels with a certain pattern of inflammation) Necrotizing vasculitis, extravascular palisade granulomasand leukocytoclastic Vasculitis can also occur.

What is the treatment for granulomatosis with polyangiitis?

Granulomatosis with polyangiitis is usually treated with glucocorticoids and cyclophosphamide. Other drugs used include:

  • Methotrexate
  • Azathioprine
  • Cyclosporine
  • Antibiotics
  • Intravenous immunoglobulin
  • Plasma to exchange
  • Mycophenolate
  • Rituximab

Skin lesions can be treated symptomatically with current steroids

Surgery may be necessary to repair the tissue after fibrosis or necrosis.

What is the prognosis for granulomatosis with polyangiitis?

Granulomatosis with polyangiitis can be slow or fast. progressive. There is often no way to predict which patients will continue to have limited and milder forms of the disease and which will continue with more severe progressive progression, generalized forms of the disease. Without treatment, severe disease has a high mortality rate due to infection, heart or lung disease, kidney failure, or malignancy.

Disease activity must be regularly monitored. Close monitoring of kidney function is especially important. Many different specialists may need to be involved in patient care. Careful and long-term monitoring is required.