Midline granulomatosis

Alternative names
Wegener’s granulomatosis

Definition
Wegener’s Granulomatosis is a rare disorder which causes inflammation of blood vessels (vasculitis) in the upper respiratory tract (nose, sinuses, ears), lungs, and kidneys. Many other areas of the body may also be affected, with arthritis (joint inflammation) occurring in almost half of all cases. The eyes and skin may also be affected.

Causes, incidence, and risk factors

The cause is unknown, but Wegener’s Granulomatosis is thought to be an autoimmune disorder and is often classified as one of the rheumatic diseases. Destructive lesions develop in the upper and lower respiratory tract and the kidney. In the kidney, these lesions cause glomerulonephritis that may result in hematuria (blood in the urine) and kidney failure.

It occurs most often between the ages of 30 and 50, and men are affected twice as often as women. It is rare in children, but has been seen in infants as young as 3 months old.

The kidney disease can progress rapidly, with kidney failure occurring within months of the initial diagnosis. If untreated, kidney failure and death occur in more than 90% of all patients with Wegener’s granulomatosis.

Symptoms

Early symptoms may include fatigue, malaise (an ill feeling), fever, and a sense of discomfort around the nose and sinuses. Upper respiratory infections such as sinusitis or ear infections frequently precede the diagnosis of Wegener’s Granulomatosis. Other upper respiratory symptoms include nose bleeds, pain, and sores around the opening of the nose.

Persistent fever without an obvious cause (fever of undetermined origin - FUO) may be an initial symptom. Night sweats may accompany the fever. Loss of appetite and weight loss are common. Skin lesions are common, but there is no one characteristic lesion associated with the disease.

Kidney disease is necessary to make the definitive diagnosis of Wegener’s Granulomatosis. The urine may be bloody, which often first appears as red or smoky urine. There may be no symptoms, but is easily diagnosed with laboratory studies.

Eye problems develop in a significant number of patients and may range from a mild conjunctivitis to severe inflammation of the eyeball and the tissues around the eyeball. Additional symptoms include:

     
  • weakness  
  • loss of appetite  
  • weight loss  
  • bloody discharge from the nose  
  • pain over the sinuses  
  • sinusitis  
  • lesions (ulcers, sores, and crusting) in and around opening of the nose  
  • cough  
  • coughing up blood  
  • bloody sputum  
  • shortness of breath  
  • wheezing  
  • chest pain  
  • blood in the urine  
  • rashes  
  • joint pains

Signs and tests

     
  • biopsy of abnormal tissue, which may include:       o open lung biopsy       o upper airway biopsy       o nasal mucosal biopsy       o bronchoscopy with transtracheal biopsy       o kidney biopsy  
  • urinalysis, protein and blood in the urine (proteinuria and hematuria)  
  • chest X-ray (cavity formation, pulmonary infiltrates)  
  • bone marrow aspiration (may be done)  
  • blood tests to look for the presence of autoantibodies (antibodies that the body makes against its own tissue)

Treatment
Treatment with corticosteroids, cyclophosphamide, methotrexate, or azathioprine produces long-term remission in over 90% of affected people.

Support Groups

Support groups with others who suffer from similar diseases may help patients and their families learn about their diseases and adjust to the changes associated with the treatment.

Expectations (prognosis)
With treatment, most people recover within months, although chronic renal failure may develop. Without treatment, the disease is usually fatal within a few months of diagnosis.

Complications

     
  • chronic kidney failure  
  • hemoptysis (coughing up blood)  
  • respiratory failure  
  • inflammation of the eyes  
  • nasal septum perforation (hole)  
  • rash  
  • side effects of medications used to treat the disease

Note: Complications usually result from lack of treatment.

Calling your health care provider
Call your health care provider if chest pain, coughing up blood, blood in the urine, or other symptoms of this disorder are present.

Prevention
No preventive measures are known.

Johns Hopkins patient information

Last revised: December 3, 2012
by Levon Ter-Markosyan, D.M.D.

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