Sarcoidosis

Another disease we RTs see from time to time in the hospital setting is Sarcoidosis. This seems to be a relatively mild disease, although occasionally it can lead to symptoms and a need for a recharge in the hospital requiring the services of a respiratory therapist.

According to the Mayo Clinic it's "characterized by the development and growth of tiny clumps of inflammatory cells in different areas of your body — most commonly the lungs, lymph nodes, eyes and skin." However, it may also effect the liver, brain and heart, thus resulting in more severe complications.

The cause is relatively unknown, although it's believed the body has a normal immune response to something inhaled, such as a bacteria, virus, dust or even chemicals (perhaps some chemical inhaled at work). The immune system triggers a natural response that causes inflammation, and yet this inflammation does not go away.

Instead, as noted at the National Heart Lung and Blood Institute (NHLBI), "some of the immune system cells cluster to form lumps called granulomas in various organs."

Since granulomas in an organ can effect how it works, signs and symptoms will depend on the organ effected, and treatment will depend on the organ effected and the severity of the symptoms.

Most (or about two-thirds) with the condition have no symptoms at all or mild symptoms, and the course of their disease is relatively mild. Yet when symptoms are bothersome, or if an organ is at risk, treatment treatment with anti-inflammatory medication will be needed, according to the Mayo Clinic.

Medlineplus notes that while the course of the disease is generally mild, "it can be severe and do lasting damage." For example, as the Mayo Clinic notes, "Untreated pulmonary sarcoidosis can lead to irreversible damage to the tissue between the air sacs in your lungs, making it difficult to breathe."

In effect, according to the Mayo Clinic, in some rare cases, Sarcoidosis may lead to...

1. Interstitial lung disease (ILD), which is ultimately pulmonary fibrosis.
2. Inflammation of the eyes can cause cataracts, glaucoma or even blindness.
3. Kidney failure as granulomas effect how the body handles calcium
4. Abnormal heart rhythms that can be life threatening because granulomas can effect the electrical conductivity of the heart
5. Facial paralysis due to electrical impulses in the spinal cord and brain
6. Infertility in men if it infests the testes
   

So what is the cause of this disease and who is at risk? According to the Mayo Clinic, NHBLI, and Medline Plus, the cause is relatively unknown, and the gene has yet to be isolated.

However, the Mayo Clinic notes that while anyone can get Sarcoidosis, the risk of getting it is increased in:

• You are between the ages of 20 and 40
• Women slightly more than men (may be more severe in blacks & result in skin problems)
  
• Blacks slightly more than whites
• Families originally from Northern Europe — particularly Scandinavia and Britain. (People with Japanese ancestry are more likely to develop eye or cardiac complications)

Symptoms vary from patient to patient, vary based on the organs effected and length of time the person has had the disease.

As noted above, symptoms are generally mild vary based on the organ and length of time the person has had the disease.

The Mayo Clinic notes that, "Sometimes sarcoidosis develops gradually and produces signs and symptoms that last for years. Or symptoms may appear suddenly and then disappear just as quickly. Many people with sarcoidosis have no symptoms, so the disease may not be discovered until you have a chest X-ray for another reason."

According to the Mayo Clinic, some of the early signs include:

• Fatigue
• Fever
• Swollen lymph nodes
• Weight loss

Most will eventually develop lung problems, and the symptoms will include:

• Persistent dry cough
• Shortness of breath
• Wheezing
• Chest pain

Those with skin involvement may develop a rash, nodules (growths just under the skin), areas of skin may get darker, or disfiguring skin sores on nose, cheeks and ears.

Eye involvement may include blurred vision, eye pain, severe redness and sensitivity to light.

By the time a respiratory therapist is called upon to treat someone with this disease there is lung involvement.

NHLBI notes another classic symptoms is Lofgren's syndrome. This "is a classic set of signs and symptoms that... may cause fever, enlarged lymph nodes, arthritis (usually in the ankles), and/or erythema nodosum... (which is) a rash of red or reddish-purple bumps on... ankles and shins. The rash may be warm and tender to the touch."

Diagnosis usually involves:

• Chest x-ray: will show enlarged lymph nodes or any related lung damage, such as fibrosis of lung tissue or air trapping.
• CT Scan: Provides a more detailed picture
• Lab tests: To check for liver or kidney function (damage)
• Biopsies: Tissue taken from infected areas can be tested for types of granulomas. Lung biopsies are taken from bronchoscopy

Treatment, according to the Mayo Clinic, is usually reserved for those who show symptoms, and includes:

• Corticosteroids: Creams can be applied to affected areas, and inhaled corticosteroids or systemic corticosteroids can be used to treat those with infected lungs to treat inflammation. A first line medicine.
• Anti-rejection meds: Drugs methotrexate (Trexall) or azathioprine (Imuran) reduce inflammation by suppressing your immune system. These are 2nd line therapies, and carry risk of inceasing infections.
• Anti-malarial drugs: Hydroxychloroquine (Plaquenil) may be helpful for skin disease, nervous system involvement and elevated blood-calcium levels. May cause eye problems.
• TNF-alpha inhibitors: Tumor necrosis factor-alpha (TNF-alpha) inhibitors are most commonly used to treat the inflammation associated with rheumatoid arthritis. Some studies have indicated that infliximab (Remicade) is also helpful in treating sarcoidosis. Potential side effects include congestive heart failure, blood disorders and lymphoma.
• Organ transplant: Rare

The Mayo Clinic notes that in most cases where the patient shows symptoms, the symptoms go away with treatment, and usually completely resolves within a year or two. About 50% of cases go into remission within three years..

However, in some cases, permanant damage is done to the organ, such as untreated pulmonary granulomas can result in pulmonary fibrosis, heart granulomas can result in cardiac arrhythmias

Like COPD patients, or those with severe asthma, patients with the severe forms of the disease will need to make lifestyle changes and may require counseling, pulmonary rehabilitation and counseling.