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Sarcoidosis

 

Sarcoidosis is a disease in which inflammation occurs in the lymph nodes, lungs, liver, eyes, skin, or other tissues.

Causes

The exact cause of sarcoidosis is unknown. What is known is that when a person has the disease, tiny clumps of abnormal tissue (granulomas) form in certain organs of the body. Granulomas are clusters of immune cells.

The disease can affect almost any organ. It most commonly affects the lungs.

Doctors think that having certain genes makes it more likely for a person to develop sarcoidosis. Things that may trigger the disease include infections with bacteria or viruses. Contact with dust or chemicals may also be triggers.

The disease is more common in African Americans and Caucasians of Scandinavian heritage. More women than men have the disease.

The disease often begins between ages 20 and 40. Sarcoidosis is rare in young children.

A person with a close blood relative who has sarcoidosis is nearly five times as likely to develop the condition.

Symptoms

 

There may be no symptoms. When symptoms do occur, they can involve almost any body part or organ system.

Almost all people affected by sarcoidosis have lung or chest symptoms:

  • Chest pain (most often behind the breast bone)
  • Dry cough
  • Shortness of breath
  • Coughing up blood (rare, but serious)

Symptoms of general discomfort:

  • Fatigue
  • Fever
  • Joint ache or pain (arthralgia)
  • Weight loss

Skin symptoms:

  • Hair loss
  • Raised, red, firm skin sores (erythema nodosum), almost always on the front part of the lower legs
  • Rash
  • Scars that become raised or inflamed

Nervous system symptoms may include:

  • Headache
  • Seizures
  • Weakness on one side of the face

Eye symptoms include:

  • Burning
  • Discharge from the eye
  • Dry eyes
  • Itching
  • Pain
  • Vision loss

Other symptoms of this disease:

  • Dry mouth
  • Fainting spells, if the heart is involved
  • Nosebleed
  • Swelling in the upper part of the abdomen
  • Liver disease

 

Exams and Tests

 

A physical exam may show the following:

  • Abnormal breath sounds (such as rales)
  • Enlarged liver
  • Enlarged lymph glands
  • Enlarged spleen
  • Rash
  • Eye inflammation (specialized eye exam is needed)

The disease is often found in people without visible physical signs who have an abnormal chest x-ray.

Different imaging tests may help diagnose sarcoidosis:

  • Chest x-ray to see if the lungs are involved or lymph nodes are enlarged
  • CT scan of the chest
  • Lung gallium scan
  • Imaging tests of the brain and liver
  • Echocardiogram of the heart

To diagnose this condition, a biopsy is needed. Biopsy of the lung using bronchoscopy is usually done. Biopsies of other body tissues may also be done.

The following lab tests may be done:

  • Calcium levels (urine, ionized, serum)
  • CBC
  • Immunoelectrophoresis -- serum
  • Liver function tests
  • Quantitative immunoglobulins (nephelometry)
  • Serum phosphorus
  • Angiotensin converting enzyme (ACE)

 

Treatment

 

Sarcoidosis symptoms will often get better without treatment.

If the eyes, heart, nervous system, or lungs are affected, corticosteroid medicine is usually prescribed. This medicine may need to be taken for 1 to 2 years.

Medicines that suppress the immune system (immunosuppressive medicines) are sometimes also needed.

In rare cases, people with very severe heart or lung damage (end-stage disease) may need an organ transplant.

 

Outlook (Prognosis)

 

Many people with sarcoidosis are not seriously ill, and they get better without treatment. Up to half of all people with the disease get better in 3 years without treatment. People whose lungs are affected may develop lung damage.

Overall death rate from sarcoidosis is less than 5%. Causes of death include:

  • Bleeding from the lung tissue
  • Heart damage, leading to heart failure and abnormal heart rhythms
  • Lung scarring (pulmonary fibrosis)

 

Possible Complications

 

  • Fungal lung infections (aspergilloma)
  • Glaucoma and blindness from uveitis (rare)
  • Kidney stones from high calcium levels in blood or urine
  • Osteoporosis and other complications of taking corticosteroids for long periods of time
  • High blood pressure in the arteries of the lungs (pulmonary hypertension)

 

When to Contact a Medical Professional

 

Call your health care provider promptly if you have:

  • Difficulty breathing
  • Palpitations
  • Vision changes
  • Other symptoms of this disorder

 

 

References

Judson MA, Morgenthau AS, Baughman RP. Sarcoidosis. In: Broaddus VC, Mason RJ, Ernst JD, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 6th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 66.

Valeyre D, Prasse A, Nunes H, Uzunhan Y, Brillet PY, Müller-Quernheim J. Sarcoidosis. Lancet. 2014; 383(9923):1155-1167. PMID: 24090799 www.ncbi.nlm.nih.gov/pubmed/24090799.

 
  • Sarcoid, stage I - chest X-ray

    Sarcoid, stage I - chest X-ray - illustration

    Sarcoid is primarily a lung (pulmonary) disease. In the early stages, a chest film may show enlargement of lymph nodes in the center of the chest near the heart (mediastinum).

    Sarcoid, stage I - chest X-ray

    illustration

  • Sarcoid, stage II - chest X-ray

    Sarcoid, stage II - chest X-ray - illustration

    Sarcoid causes damage to the lung tissue that heals by scarring. The film shows a diffuse milky and granular appearance in the normally dark lung areas. This individual has marked decrease in lung function.

    Sarcoid, stage II - chest X-ray

    illustration

  • Sarcoid, stage IV - chest X-ray

    Sarcoid, stage IV - chest X-ray - illustration

    This film shows advanced sarcoid, scarring of the lungs (the light streaking), and cavity formation (the dark areas in the upper right side of the picture).

    Sarcoid, stage IV - chest X-ray

    illustration

  • Sarcoid - close-up of the skin lesions

    Sarcoid - close-up of the skin lesions - illustration

    Sarcoid - close-up of the skin lesions: 20 to 25 percent of individuals with sarcoidosis have skin manifestations as seen in this picture. The extent of the skin manifestations is difficult to predict, but the most common are red papules that are translucent as seen here.

    Sarcoid - close-up of the skin lesions

    illustration

  • Erythema nodosum associated with sarcoidosis

    Erythema nodosum associated with sarcoidosis - illustration

    This picture shows reddish-purple, hard (indurated), painful nodules (Erythema nodosum) that occur most commonly on the shins. These lesions may be anywhere on the body and may be associated with tuberculosis (TB), sarcoidosis, coccidioidomycosis, systemic lupus erythematosis (SLE), fungal infections, or in response to medications.

    Erythema nodosum associated with sarcoidosis

    illustration

  • Sarcoidosis - close-up

    Sarcoidosis - close-up - illustration

    Typical sarcoid lesions consist of red, raised lesions (papules) and patches (plaques) with minimal surrounding skin change.

    Sarcoidosis - close-up

    illustration

  • Sarcoidosis on the elbow

    Sarcoidosis on the elbow - illustration

    These lesions of sarcoidosis are located on the elbow and are red, elevated patches (plaques). The cause of sarcoidosis remains unknown.

    Sarcoidosis on the elbow

    illustration

  • Sarcoidosis on the nose and forehead

    Sarcoidosis on the nose and forehead - illustration

    These are sarcoid lesions on the face. These lesions often appear in scars, as is seen in this photograph.

    Sarcoidosis on the nose and forehead

    illustration

  • Respiratory system

    Respiratory system - illustration

    Air is breathed in through the nasal passageways, travels through the trachea and bronchi to the lungs.

    Respiratory system

    illustration

    • Sarcoid, stage I - chest X-ray

      Sarcoid, stage I - chest X-ray - illustration

      Sarcoid is primarily a lung (pulmonary) disease. In the early stages, a chest film may show enlargement of lymph nodes in the center of the chest near the heart (mediastinum).

      Sarcoid, stage I - chest X-ray

      illustration

    • Sarcoid, stage II - chest X-ray

      Sarcoid, stage II - chest X-ray - illustration

      Sarcoid causes damage to the lung tissue that heals by scarring. The film shows a diffuse milky and granular appearance in the normally dark lung areas. This individual has marked decrease in lung function.

      Sarcoid, stage II - chest X-ray

      illustration

    • Sarcoid, stage IV - chest X-ray

      Sarcoid, stage IV - chest X-ray - illustration

      This film shows advanced sarcoid, scarring of the lungs (the light streaking), and cavity formation (the dark areas in the upper right side of the picture).

      Sarcoid, stage IV - chest X-ray

      illustration

    • Sarcoid - close-up of the skin lesions

      Sarcoid - close-up of the skin lesions - illustration

      Sarcoid - close-up of the skin lesions: 20 to 25 percent of individuals with sarcoidosis have skin manifestations as seen in this picture. The extent of the skin manifestations is difficult to predict, but the most common are red papules that are translucent as seen here.

      Sarcoid - close-up of the skin lesions

      illustration

    • Erythema nodosum associated with sarcoidosis

      Erythema nodosum associated with sarcoidosis - illustration

      This picture shows reddish-purple, hard (indurated), painful nodules (Erythema nodosum) that occur most commonly on the shins. These lesions may be anywhere on the body and may be associated with tuberculosis (TB), sarcoidosis, coccidioidomycosis, systemic lupus erythematosis (SLE), fungal infections, or in response to medications.

      Erythema nodosum associated with sarcoidosis

      illustration

    • Sarcoidosis - close-up

      Sarcoidosis - close-up - illustration

      Typical sarcoid lesions consist of red, raised lesions (papules) and patches (plaques) with minimal surrounding skin change.

      Sarcoidosis - close-up

      illustration

    • Sarcoidosis on the elbow

      Sarcoidosis on the elbow - illustration

      These lesions of sarcoidosis are located on the elbow and are red, elevated patches (plaques). The cause of sarcoidosis remains unknown.

      Sarcoidosis on the elbow

      illustration

    • Sarcoidosis on the nose and forehead

      Sarcoidosis on the nose and forehead - illustration

      These are sarcoid lesions on the face. These lesions often appear in scars, as is seen in this photograph.

      Sarcoidosis on the nose and forehead

      illustration

    • Respiratory system

      Respiratory system - illustration

      Air is breathed in through the nasal passageways, travels through the trachea and bronchi to the lungs.

      Respiratory system

      illustration

    Tests for Sarcoidosis

     

       

      Review Date: 6/22/2015

      Reviewed By: Denis Hadjiliadis, MD, MHS, Associate Professor of Medicine, Pulmonary, Allergy, and Critical Care, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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