Sarcoidosis

Definition
Sarcoidosis is a disease of unknown cause in which inflammation occurs in the Lymph nodes, lungs, liver, eyes, skin, and/or other tissues.

Causes, incidence, and risk factors

The cause of the disease is unknown. Tissue samples from affected organs show clusters of immune cells (macrophages, lymphocytes, and multinucleated giant cells). These clusters are called granulomas.

Possible causes include a hypersensitive response to some factor in the environment, a genetic predisposition, or an extreme immune response to infection. The incidence varies widely according to race and sex.

The disease is most common among North Americans of African heritage and Northern European Caucasians. Women of African heritage are affected more than men of African heritage. The onset of the disease usually occurs in people between 30 and 50 years old. Sarcoidosis is very rare in young children.

Symptoms

     
  • General discomfort, uneasiness, or ill feeling (malaise)  
  • Fever  
  • Shortness of breath  
  • Cough  
  • Skin lesions  
  • Skin rash  
  • headache  
  • Visual changes  
  • Neurological changes  
  • Enlarged lymph glands (armpit lump)  
  • Enlarged liver  
  • Enlarged spleen  
  • Dry mouth  
  • Fatigue (one of the most common symptoms in the pediatric population)  
  • Weight loss (one of the most common symptoms in the pediatric population)

Additional symptoms that may be associated with this disease:

     
  • Tearing, decreased  
  • Seizures  
  • Nosebleed - symptom  
  • Joint stiffness  
  • Hair loss  
  • Eye burning, itching and discharge  
  • Rales or other abnormal breath sounds

Note: There may be no symptoms.

Signs and tests

     
  • CBC  
  • Chem-7 or chem-20  
  • ACE levels  
  • Chest x-ray to see if the lungs are involved or Lymph nodes are enlarged  
  • Lymph node biopsy  
  • Skin lesion biopsy  
  • Bronchoscopy  
  • Open lung biopsy  
  • Liver biopsy  
  • Kidney biopsy  
  • EKGto see if the heart is involved

This disease may also alter the results of the following tests:

     
  • Quantitative immunoglobulins (nephelometry)  
  • PTH  
  • Serum phosphorus  
  • Nerve biopsy  
  • Mediastinoscopy with biopsy  
  • Lung gallium (Ga.) scan  
  • Immunoelectrophoresis - serum  
  • Calcium; urine  
  • Calcium (ionized)  
  • Serum calcium  
  • Liver function tests

Treatment

Sarcoidosis symptoms often gradually resolve on their own without treatment.

Severely affected patients may require treatment with corticosteroids. Therapy may continue for one or two years, although some of the most severely affected may require life-long therapy.

Immunosuppressive agents, such as methotrexate, azathioprine, and cyclophosphamide, are sometimes used in addition to corticosteroids. Rarely, some individuals with irreversible organ failure require organ transplantation.

Expectations (prognosis)

Many people are not seriously ill, and the disease may resolve without treatment. Thirty to fifty percent of cases resolve without treatment in 3 years. About 20% of those with lung involvement will develop residual lung damage. Death from sarcoidosis is rare.

Complications

     
  • Diffuse interstitial pulmonary fibrosis  
  • Pulmonary hypertension  
  • Anterior uveitis  
  • Glaucoma and Blindness (rare)  
  • Cardiac arrhythmias  
  • Cranial or peripheral nerve palsies  
  • kidney stones  
  • Organ failure, leading to the need for a transplant

Calling your health care provider

Call your health care provider if difficulty breathing, vision changes, palpitations, or other symptoms suggestive of this disorder develop.

Johns Hopkins patient information

Last revised: December 7, 2012
by Sharon M. Smith, M.D.

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