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Wegener's Granulomatosis

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Wegener's Granulomatosis

What is Wegener's Granulomatosis?

Wegener's granulomatosis is a disease characterized by inflamed blood vessels. The inflammation damages important organs of the body by limiting blood flow to those organs and destroying normal tissue.

Wegener's granulomatosis mainly affects the respiratory tract (sinuses, nose, trachea, and lungs) and the kidneys. Wegener's granulomatosis can affect people at any age and strikes men and women equally.

Wegener's granulomatosis is very uncommon.

What Causes Wegener's Granulomatosis?

The exact cause of Wegener's granulomatosis is unknown.

What are the Symptoms of Wegener's Granulomatosis?

There are many symptoms of Wegener's granulomatosis. The most common symptoms are:

  • upper respiratory tract symptoms
  • joint pains
  • weakness
  • fatigue
  • hoarseness
  • shortness of breath
  • cough

Complication of Wegener's Granulomatosis

Upper respiratory tract

Symptoms include sinus pain, discolored or bloody fluid from the nose, and, occasionally, nasal ulcers. A common sign of Wegener's granulomatosis is almost constant runny nose or other cold symptoms that do not respond to usual treatment or that become increasingly worse.

Rhinorrhea can result from nasal inflammation or sinus drainage and can cause pain. A hole may develop in the cartilage of the nose, which may lead to collapse (called saddle-nose deformity). The eustachian tubes, which are important for normal ear function, may become blocked, causing chronic ear problems and hearing loss. Bacterial infection can cause Wegener's-related sinusitis (inflammation of the sinuses) with congestion and chronic sinus pain.

Lungs

The lungs are affected in most patients with Wegener's granulomatosis, although no symptoms may be present. If symptoms are present, they include cough, hemoptysis (coughing up blood), shortness of breath, and chest discomfort.

Kidneys

Kidney involvement, which occurs in more than three-fourths of patients, usually does not cause symptoms. If detected by blood and urine tests, the doctor can start proper treatment, preventing long-term damage to the kidneys.

Musculoskeletal system

Pain in the muscles and joints or, occasionally, joint swelling affects two-thirds of patients with Wegener's granulomatosis. Although joint pain can be very uncomfortable, it does not lead to permanent joint damage or deformities.

Eyes

Wegener's granulomatosis can affect the eyes in several ways. Patients may develop:

  • Conjunctivitis (inflammation of the conjunctiva, the inner lining of the eyelid)
  • Scleritis (inflammation of the scleral layer, the white part of the eyeball)
  • Episcleritis (inflammation of the episcleral layer, the outer surface of the sclera)
  • Mass lesion behind the eye globe

Skin lesions

Nearly half of people with Wegener's granulomatosis develop skin lesions. These often have the appearance of small red or purple raised areas or blister-like lesions, ulcers, or nodules that may or may not be painful.

Can Wegener's Granulomatosis be Treated?

Wegener's granulomatosis can be treated. The most common treatment options include corticosteroid medications such as prednisone and chemotherapy drugs such as cyclophosphamide or methotrexate.

The treatments are designed to suppress the immune system and usually induce the complete absence of all signs of Wegener's granulomatosis.

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