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Sarcoidosis


Signs and Symptoms

Physician-developed and -monitored.

Original Date of Publication: 01 Jun 2000
Reviewed by: Stanley J. Swierzewski, III, M.D.
Last Reviewed: 04 Dec 2007

Original Source: http://www.pulmonologychannel.com/sarcoidosis/symptoms.shtml

Home » Sarcoidosis » Signs and Symptoms


Signs and Symptoms

Sarcoidosis is usually asymptomatic, which means there are no outward signs that the person has the disease. This is why so many cases of sarcoidosis are discovered by chance during a routine x-ray. When symptoms appear, the most common are:



A person may experience shortness of breath (dyspnea), a dry cough that persists, skin rashes (either erythema nodosum or other types of rashes), or inflammation in the eye. Sometimes, a person with sarcoidosis complains of "feeling sick" and may experience fever or weight loss. Sarcoidosis can affect nearly any organ, with the possible exception of the adrenal gland.

Respiratory symptoms
In over 90% of patients with sarcoidosis, whether or not they show outward signs of the disease, abnormalities almost always show up on a chest x-ray. These include swelling and inflammation of the lymph nodes (lymphadenopathy) in the chest and, less commonly, a mottled (having colored spots and blotches) appearance to the lungs.

Swollen lymph nodes (adenopathy)
There are two sets of thoracic lymph nodes that are commonly affected: the hilar (where the bronchial tubes enter the lungs) and mediastinal (the region in the center of the chest just outside of the lungs).

Swollen hilar and mediastinal lymph nodes are not exclusive to sarcoidosis. There are several other underlying problems that can lead to lymphadenopathy (e.g., tuberculosis, cancer). There are differences in the type of inflammation that help differentiate the diagnoses of these disorders. Sometimes CT scanning provides a more detailed picture that can help eliminate other possibilities. In most cases, if the lymphadenopathy is bilateral (occuring on both sides of the body) and especially if it is accompanied by characteristic skin or eye lesions, it is fairly certain that the patient has sarcoidosis.

Pulmonary infiltration and fibrosis
The mottled appearance of the lungs that shows up in a chest x-ray is due to what is known as "pulmonary infiltration." Infiltration refers to the abnormal presence of granulomas in the lung tissue. Depending on how severe and extensive the pulmonary infiltration is, it can cause various respiratory symptoms. The two most common are shortness of breath (dyspnea) and a persistent, dry cough. About 25% of sarcoidosis patients experience dyspnea and/or a persistent cough.

Pulmonary infiltration can lead to lung fibrosis (i.e., the hardening and thickening of tissue) that can cause permanent scarring. Fibrosis tends to develop in areas where the infiltration lasts a long time or where it is especially dense. Fibrosis causes the lungs to stiffen, making it difficult to breathe. Unfortunately, fibrosis is irreversible. If extensive, it can cause severe dysfunction and, at its worse, can lead to heart failure.

Although often asymptomatic, granulomas in the lung can cause assorted respiratory problems, including:

  • A persistent, dry cough
  • Chest pain
  • Hemoptysis (spitting up of blood from the lungs)
  • Hoarseness
  • Nasal congestion
  • Pneumothorax (air in the chest outside the lungs, which can lead to a collapsed lung)
  • Shortness of breath, or difficult breathing (dyspnea), a normal result of exercise but in sarcoidosis patients can occur without exercise
  • Wheezing (noisy, difficult breathing)

In the majority of patients, the inflammation and infiltration in the lungs eventually clears up. There is considerable difference among racial groups in the likelihood of the sarcoidosis clearing up without causing any permanent damage. In the United States, nearly 80% of stage 1 cases in Caucasians resolve spontaneously, without intervention, compared to about 33% of stage 1 cases in African Americans. The percentage of cases that resolve decreases as the stage increases. Generally, the older the patient is, the more likely the sarcoidosis will become chronic and lead to fibrosis.



Upper airway involvement
The upper airways, including the sinuses and the larynx, can be affected with sarcoidosis. Sinus involvement can lead to recurrent sinusitis or, rarely, perforation of the nasal septum. Laryngeal involvement can lead to hoarseness and upper airway obstruction.

Endobronchial involvement
Granulomas can occur in the trachea or the bronchial tubes leading to airway obstruction that can cause difficulty breathing and obstructive pneumonia.

Extrathoracic lymph nodes
In addition to the hilar and mediastinal lymph nodes in the chest, sarcoidosis can affect lymph nodes in other parts of the body. Affected glands are typically painless and often not palpable (cannot be felt). Lymph node involvement outside of the thoracic region is more common among African Americans than Caucasians.

Dermatology
Skin lesions, or granulomas that develop on the skin, occur in about 25% of cases. Most skin lesions are harmless, although they can cause scarring and disfigurement. One type, lupus pernio, develops on the face and can leave scars. Another type, erythrema nodosum, develops on the legs and is quite painful.

Erythrema nodosum
Erythrema nodosum lesions are small, hard nodules that develop on the lower parts of the leg and usually disappear without treatment. The bumps appear rather suddenly and are tender and painful. Sometimes, they are accompanied by a mild fever and arthritis in the ankle and other joints.

Although a common symptom of sarcoidosis, the occurence of erythrema nodosum does not necessarily mean that a person has sarcoidosis. Other causes of erythrema nodosum include infections and drug sensitivity.

Lupus pernio
Lupus pernio is a chronic (long term), persistent skin lesion that forms on the face, especially the nose, ears, and cheek. It can leave permanent scarring and nasal disfigurement. It may also affect the hands and feet.

Ophthalmological
Sarcoidosis affects the eyes in about 25% of cases. Its most common manifestation is inflammation, a common disorder that is rarely due to sarcoidosis. Usually patients complain of blurred vision in both eyes, sensitivity to bright light, excessive tearing, or pain. In chronic sarcoidosis, more serious eye problems can develop, such as glaucoma and cataracts. If left untreated, sarcoidosis can lead to blindness.

Gastrointestinal disturbances
Patients may experience dysphagia (difficulty swallowing), abdominal pain, or jaundice (a yellowing of the skin that can be caused by any number of underlying disorders).

Spleen
The spleen is an organ in the upper abdominal cavity that plays an important role in storing red blood cells, as well as filtering out old red blood cells from the circulating blood supply. Although usually asymptomatic, sarcoidosis granulomas are often scattered throughout the spleen.

Liver
Up to three-fourths of patients develop granulomas in the liver that rarely create symptoms. Granulomas in the liver can also be due to cirrhosis, tuberculosis, and Hodgkin's disease.

Neurological symptoms
Sarcoidosis can affect any part of the nervous system. Not more than about 5% of sarcoidosis patients have neurological manifestation. Symptoms can range from short-lived facial nerve palsy (e.g., Bell's palsy), to chronic visual or hearing problems, to symptoms that mimic a tumor. Patients may experience headache, paresthesia (an abnormal prickly or tingling sensation), seizures, or meningitis (inflammation of the brain or spinal cord). Granuloma involvement in the hypothalmus can lead to diabetes insipidus. Sarcoidosis can also lead to obstructive hydrocephalus.

Cardiac symptoms
It is estimated that about 20% to 25% of patients with sarcoidosis have cardiac granulomas. Like many other organs, sarcoidosis granulomas on the heart are usually asymptomatic. Heart problems can arise, however, usually as secondary complications due to granulomas in the lung.

Cardiac symptoms may include syncope (temporary loss of consciousness due to inadequate blood flow to the brain), congestive heart failure (the inability of the heart to adequately pump blood throughout the body causing a back up of fluid into the lungs), cardiac tamponade (compression of the heart due to an accumulation of fluid in the pericardium, the membrane that covers the heart), or dyspnea (difficult breathing). At its worst, sudden cardiac arrest can occur.

Bones, joints, and muscles
Bones, joints, and muscles can be affected by sarcoidosis. The bones are rarely affected and when they are, it is usually in conjunction with lupus pernio (see Lupus pernio). The bones in the hands, feet, and nose may be affected and the joints in the hands and feet may swell. A patient who has swollen lymph nodes and erythema nodosum (see erythema nodosum) may experience pain and tenderness in the muscles that is usually a short-lived myopathic problem (see myopathy) that resolves on its own.

Kidneys
Asymptomatic granulomas invade the kidneys as often as they do the lungs and liver and very rarely do complications result. Nephrolithiasis (the formation of a kidney stone that can cause abdominal pain and blood in the urine) could occur. In severe cases, kidney failure can result.

General ill feelings
Some patients may experience fever, malaise, fatigue, and weight loss.

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