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Jun 08, 2006

Sarcoidosis Treatment

by SirGan

SteadyHealth.com - Health Topics Forum Index -> Articles archive

Sarcoidosis is an immune system disorder which is usually characterized by specific tiny lumps of cells also called non-necrotizing granuloms. Big problem is that virtually any organ can be affected; but - granuloms most often appear in the lungs and surrounding lymph nodes. There is no rule about disease progress because symptoms can occasionally appear suddenly but more often than not appear gradually. These tiny granuloms can grow and clump together, making many large and small groups of lumps. If many granuloms form in an organ, they can affect how the organ works. Rarely, sarcoidosis affects other organs, including thyroid gland, breasts, kidneys, reproductive organs… Sarcoidosis has an active and a non-active phase:
During the active phase, the granuloms form and grow. In this phase, symptoms can develop, and scar tissue can form in the organs where the granulomas occur. In the non-active phase, the inflammation goes down, and the granulomas stay the same size or shrink. But the scars may remain and cause symptoms

Incidence of the condition

Although anyone can develop sarcoidosis, the disease mainly affects adults between the ages of 20 and 40. The prevalence of sarcoidosis in US the adult population ranges from 1 case to 40 cases per 100,000 people population, with an annual age incidence rate of 10.9 per 100,000 for white people and 35.5 per 100,000 in black people. No clear sex predominance exists in childhood sarcoidosis! 

Signs and symptoms of sarcoidosis

The fact is that sometimes sarcoidosis develops gradually and produces signs and symptoms that last for years, but it can also appear suddenly and then disappear just as quickly. Several researches done in the past have shown that there is no rules when we talk about signs and symptoms because they can vary, depending on which organs are affected and how long the patient had the disease.
 
The most common symptoms involve the lungs, skin, eyes and liver.
  • Lungs
It is estimated that the lungs are affected in about 88% of people who have sarcoidosis. The most common symptoms are cough or chest pain. Some people have breathing problems, but most people have few or no problems with breathing!
  • Skin
Very often people with sarcoidosis have some skin symptoms and these problems are mostly rashes or nodules (small bumps on the skin). Small red bumps are located all over the face, arms or buttocks, and a condition more common in blacks than in whites.
  • Eyes
It is proven that about 25% of people who have sarcoidosis have eye symptoms. Although these symptoms can vision a bit hard -it they rarely cause blindness. Eye symptoms usually include dry eyes, but they can also include swelling of the tear gland that makes the eyes water.
  • Liver
Sarcoidosis affects the liver at some point in approximately 20% of people who have the disease. Most common symptoms is hepatomegalia- when the liver can become enlarged.
  • Heart
The most common cardiac abnormalities include conduction abnormalities and dysrhythmias due to granulomatous infiltration of the heart. Half of the patients with cardiac sarcoidosis have electrocardiography abnormalities of rhythm, conduction, and repolarization.
 
Other signs and symptoms of sarcoidosis include:
  • A vague feeling of discomfort and fatigue
  • Fever
  • Weight loss
  • Red, watery eyes
  • Arthritis in your ankles, elbows, wrists and hands, commonly associated with bumps in the skin over your shins  

Possible causes of sarcoidosis

Although many experts are speculating about possible causes of sarcoidosis, no one really know the exact cause of the condition. The most common theory is that it may occur when someone’s immune system overreacts to some unknown toxin, drug or pathogen that enters your body through your airways and lungs. Some experts are saying that this could be some kind of bacteria, especially some non-tuberculosis mycobacterium, a member of the family of bacteria that cause tuberculosis. It is important also to point out that only in a small portion of people; sarcoidosis may have a genetic component so heredity could also play a role!
What is the purpose of immune system? Normally, immune system helps protect body from foreign substances and invading microorganisms such as bacteria and viruses, but in sarcoidosis, T-helper lymphocytes seem to respond too strongly to a perceived threat. This means that the immune response is making more damage than the microorganism it self! This triggers small areas of inflammation called granulomas.

Pathogenesis of sarcoidosis

So, we have came to the conclusion that sarcoidosis is a chronic inflammatory disease characterized by a highly focused exaggerated immune response to an unknown antigen at the target organs. The most common histological signs of the disease - sarcoid granulomas are most likely formed in response to a persistent, poorly degradable, antigenic stimulus.
As a result of these various immunologic interactions, one specific cascade of inflammation occurs which is characterized by changes in tissue permeability, cellular influx, and local cell proliferation, resulting in a granuloma.

Diagnosis of sarcoidosis

  • Physical examination
To find out if a patient have sarcoidosis, most doctors will perform a physical exam looking for the common signs!
  • Lab studies
No definitive laboratory test diagnostic of sarcoidosis exists. In the absence of a known etiologic agent, sarcoidosis often remains a diagnosis of exclusion. Doctors usually order a blood test to determine the level of a certain chemical, called angiotensin-converting enzyme, in patient’s blood. ACE is produced by the epithelioid cells of granulomas and, thus, may be elevated in the serum of sarcoid patients. When doctor finds out the level of angiotensin-converting enzyme- it can help him to follow the course of your disease and check your response to treatment.
  • Imaging Studies
The most effective non-invasive test to support the diagnosis of sarcoidosis is chest x-ray which typically shows bilateral lymph node enlargement. It is also very helpful if doctors want to exclude other diseases, including tuberculosis, histoplasmosis or other fungal infection, or lymphoma that can also demonstrate lymph node enlargement similar to sarcoidosis.
High-resolution CT imaging is also considered a very helpful and effective diagnostic tool! Characteristic HRCT scan findings include smooth or nodular interstitial thickening of the structures such as bronchi and surrounding blood vessels!
  • Pulmonary function tests
These tests measure how much air lungs can hold and the flow of air in and out of lungs. They can also measure the amount of gases exchanged across the membrane between your lung wall and capillary membrane.
  • Biopsy
Tissue biopsy findings that confirm the diagnosis of childhood sarcoidosis have been documented in many cases. Biopsy is obtained from the most readily accessible organ with the least invasive method.
  • Histological Findings
The diagnosis of sarcoidosis is confirmed by demonstrating a typical granuloma on a biopsy specimen using the microscope.  An experienced pathologist must review characteristic pathological changes.

Stages of sarcoidosis

  • Stage 0 - Normal findings on chest radiograph
  • Stage I - Bilateral hilar lymphadenopathy, which may be accompanied by adenopathy
  • Stage II - Bilateral hilar adenopathy with pulmonary infiltrates
  • Stage III - infiltrates in the parenchyma without hilar adenopathy
  • Stage IV - Advanced fibrosis with evidence of honey-combing, hilar retraction, bullas, cysts, and emphysema

Risk factors for developing sarcoidosis

Although, literally anyone can develop sarcoidosis there are some factors which can make a person more likely to get the disease:
  • Race
Black Americans are far more likely to develop sarcoidosis than are white Americans.
The real cause of this still remain unknown! And although sarcoidosis affects white men and women about equally, black women get the disease twice as often as black men do.
  • Ethnicity
People of Scandinavian, German or Irish descent have an increased risk.
  • Age
Sarcoidosis normally occurs between the ages of 20 and 40. It rarely affects children, but can occur in adults older than 50.

Sarcoidosis treatment

What are the most important goals of therapy? The goals of treatment are to:
  • Improve how the organs affected by sarcoidosis work
  • Relieve symptoms
  • Shrink the granulomas

Corticosteroids

The main treatment for sarcoidosis is corticosteroid medication called Prednisone.
 Prednisone is very strong anti-inflammatory drug. It almost always relieves symptoms of inflammation. Prednisone is usually given for many months, sometimes for a year or more.
 Low doses of prednisone can often relieve symptoms without causing major side effects.
There are also some other medications mostly immune system suppressants which may also prevent immune system from fighting things like bacteria and viruses.
Most commonly used are:
  • Hydroxychloroquine (Plaquenil)
  • Methotrexate
  • Azathioprine (Imuran)
  • Cyclophosphamide (Cytoxan)

Vitamin D

Disciplined avoidance of sunlight and Vitamin D foods is necessary in patients who are prone to develop hypercalcemia and will help relieve symptoms in all sarcoidosis patients.

Antibiotics

Antibiotic therapy has been reported to be effective for lung, lymph and cutaneous manifestations of sarcoidosis.
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    Article sources
    • www.mayoclinic.com
    • www.emedicine.com
    • www.nhlbi.nih.gov