Sarcoidosis


SARCOIDOSIS

Description:

Def: A systemic granulomatous disease of unknown cause, especially involving the lungs with resulting interstitial fibrosis, but also involving lymph nodes, skin, liver, spleen, eyes, phalangeal bones, and parotid glands; granulomas are composed of epithelioid and multinucleated giant cells with little or no necrosis. Syn: Besnier-Boeck-Schaumann disease, Boeck disease, sarcoid, Boeck sarcoid, Besnier-Boeck-Schaumann syndrome, Schaumann syndrome.

The first description of sarcoidosis (as skin lesions) was reported in 1878 by dermatologists, Jonathan Hutchinson, M.D. and Caesar Boeck, M.D. From 1899-1938, sarcoidosis was reported to affect various systems in the body classifying it as a multi-system disorder.

To date, there is no known evidence that sarcoidosis is contagious. Medical researchers speculate several causes for this disease:

  • A viral or bacterial infection.

  • A defect in the body's immune system.

  • Exposure to a toxic substance.

  • An unknown environmental cause.

  • An inherited or genetic cause.

The most common manifestations of sarcoidosis are found in the lungs, skin, joints, eyes, central nervous system, heart, liver, kidneys, lymph glands, and other soft tissue organs. To the medical community, it is known as the “great masquerader” because it mimics other diseases and conditions making it difficult for doctors to diagnose and treat. Definite diagnosis is further hindered because it can dwell in the body in an asymptomatic state (no symptoms).

It is further characterized as persistent inflammation in the form of lymphocytes (a type of overactive blood cell) which causes the formation of what is called “granulomas.” The granulomas appear as lumps or nodules (singular or as clusters) in the body ranging in size from a pea to a fist. As sarcoidosis maneuvers throughout the body, the inflammed granulomas create scar tissue and block the function of vital organs which can cause permanent organ and tissue damage at the rate of 20 to 30 percent of all diagnosed cases.

Sarcoidosis affects all races and age groups primarily targeting young adults between the ages of 20 and 40. In 1994, the National Institutes of Health, National Heart, Lung and Blood Institute reported that in the United States 5 in every 100,000 white people had the disease, but for African Americans the ratio was 40 in every 100,000 people. For reasons unknown, there is no explanation why there is a higher occurrence in the African American community. African American women contract the disease at twice the rate of African American men and have more chronic and debilitating cases. The death rate for African Americans is 15-17 times higher than Whites. For 18% of African Americans diagnosed, 12% have a family member (mother, father, sister, brother) with the disease. It is estimated that over one million people have sarcoidosis in the United States. Sarcoidosis is known to be fatal for 5-10% of diagnosed cases.

The mystery of sarcoidosis is very compelling and its impact in the United States has yet to be realized. Sarcoidosis is a serious health issue and is as important as any of the diseases (AIDs, breast cancer, prostate cancer, diabetes, multiple sclerosis, hypertension, Parkinsons) that are affecting our society today. Many people are dying from this disease. We need your help, and appeal to you to give sarcoidosis awareness paramount status on your advocacy and fund raising agenda.

Sarcoidosis has established itself as a major health concern. If, we do not address the need for sarcoidosis awareness and cure research, this mysterious plague will continue to infiltrate our human community as a silent killer.

Sarcoidosis demands our attention before it reaches epidemic proportions and too many human lives have been sacrificed.

Symptoms:

People with sarcoidosis may have no symptoms, only vague symptoms (such as weight loss, fever and a general "ill feeling"), or symptoms associated with a specific organ(s).

Up to half of sarcoidosis patients have no symptoms when the illness is diagnosed, thus making the diagnosis difficult. Listed below are some signs and symptoms associated with specific organ involvement:

Lungs:

  • Shortness of breath.

  • Wheezing.

  • Hoarseness.

  • Dry cough with phlegm.

  • Chest pain.

  • Tightness in the chest.

Lymph nodes:

  • Enlargement Iridocyclitis (inflammation or swelling of the colored part of the eye).

  • Burning or itching

  • Blurred vision.

  • Dry eye.

  • Photophobia (aversion to light).

Skin:

  • Small, raised pink or purplish patches on the face.

  • Erythema nodosum (painful red blotches found mostly on the legs with accompanying joint pain (arthritis) in knees, ankles, elbows and wrist).

  • Lupus pernio (rash that develops slowly on the nose, face and ears).

  • Painful nodules (lumps) under the skin.

Bone:

  • Nodules in the bone.

  • Joint and muscle pain.

  • Sunken lesions on fingers and toes.

Spleen and Liver:

  • Enlargement.

Heart:

  • Irregular heartbeats.

Brain and Nervous System:

  • Loss of sensation.

  • Loss of muscle strength.

  • Headache.

  • Dizziness.

  • Seizure.

  • Meningitis.

Causes and Risk factors:

The cause of sarcoidosis isn't known. More than one factor may play a role in causing the disease.

Some researchers think that sarcoidosis develops when your immune system responds to a trigger, such as bacteria, viruses, dust, or chemicals.

Normally, your immune system defends your body against foreign or harmful substances. For example, it sends special cells to protect organs that are in danger.

These cells release chemicals that recruit other cells to isolate and destroy the harmful substance. Inflammation occurs during this process. Once the harmful substance is destroyed, the cells and the inflammation go away.

In people who have sarcoidosis, the inflammation doesn't go away. Instead, some of the immune system cells cluster to form lumps called granulomas in various organs in your body.

Genetics also may play a role in sarcoidosis. Researchers believe that sarcoidosis occurs if:

    You have a certain gene (or certain genes) that raise your risk for the disease.

  • You're exposed to something that triggers your immune system.Triggers may vary depending on your genetic makeup. Certain genes may influence which organs are affected and how severe your symptoms are.

Sarcoidosis affects people of all ages and races. However, it's more common among African Americans and Northern Europeans. In the United States, the disease affects African Americans somewhat more often and more severely than Whites.

Studies have shown that sarcoidosis tends to vary in different ethnic groups. For example, eye problems due to the disease are more common in Japanese people.

Lofgren's syndrome, a type of sarcoidosis, is more common in people of European descent. Lofgren's syndrome may involve fever, enlarged lymph nodes, arthritis (usually in the ankles), and/or erythema nodosum. Erythema nodosum is a rash of red or reddish-purple bumps on your ankles and shins. The rash may be warm and tender to the touch.

Sarcoidosis is somewhat more common in women than in men. The disease usually develops between the ages of 20 and 50.

People who have certain jobs also may be at higher risk for sarcoidosis. Examples include:

  • Health care workers.

  • People whose jobs expose them to agricultural dust, insecticides, pesticides, or mold.

  • Suppliers of building materials, hardware, or gardening materials.

  • Firefighters.

  • People who have a family history of sarcoidosis also are at higher risk for the disease.

Diagnosis:

In making a diagnosis of sarcoidosis, your doctor will first start by conducting a thorough physical examination, asking about any symptoms you are experiencing, including when they started and how they've progressed or eased over time. Your full medical history will also be recorded.

There is no singular finding that confirms a diagnosis of sarcoidosis. Therefore, the diagnosis is based on multiple factors, including symptoms, abnormalities on a chest X-ray or CT scan, and microscopic examination of one or more specimens from involved tissues or organs.

In addition, you may also have the following tests to help rule out other conditions, such as tuberculosis, which cause similar symptoms as sarcoidosis and to make a definite diagnosis.

Pulmonary Function Testing (PFT) -- This test involves a series of breathing maneuvers that measure the airflow and volume of air in your lungs. This allows your doctor to objectively assess the function of your lungs. PFT is recommended for all patients.

High Resolution Computed Tomography (HRCT) -- This is a special type of CT scan that provides your doctor with high-resolution images of your lungs that are extremely valuable in determining which type of ILD you have. Having a HRCT is no different than having a regular CT scan; they both are performed on an open-air table and take only a few minutes. HRCT is recommended for all patients.

Six Minute Walk Testing -- This test is a formal evaluation of the distance you can walk and the oxygen saturations (measured by finger or ear probe) you achieve while walking. This is an important marker of functional status used by your doctor in managing your condition. The six minute walking test is recommended for all patients.

Bronchoscopy -- This test involves the passage of a flexible fiberoptic scope about the diameter of a pencil into the lungs to obtain fluid and tissue samples to aid in diagnosis. This test is an outpatient procedure, which means you do not have to stay overnight in the hospital, performed by your doctor. Bronchoscopy is recommended for some patients.

Once your diagnosis is made, additional tests may be needed to determine the stage and severity of your disease. These may include tests to check for sarcoidosis involving other organs.

Treatment:

Fortunately, many patients with sarcoidosis require no treatment. Symptoms, after all, are usually not disabling and do tend to disappear spontaneously.

When therapy is recommended, the main goal is to keep the lungs and other affected body organs working and to relieve symptoms. The disease is considered inactive once the symptoms fade. After many years of experience with treating the disease, corticosteroid drugs remain the primary treatment for inflammation and granuloma formation. Prednisone is probably the corticosteroid most often prescribed today. There is no treatment at present to reverse the lung scarring (fibrosis) that might be present in advanced sarcoidosis. More than one test is needed to diagnose sarcoidosis.

Tests can also show if patients with sarcoidosis are getting better. Occasionally, a blood test will show a high blood level of calcium accompanying sarcoidosis. The reasons for this are not clear. When it does occur, the patient may be advised to avoid calcium-rich foods, vitamin D, or sunlight, or to take prednisone (this corticosteroid usually quickly reverses the condition).

Because sarcoidosis can disappear even without therapy, even doctors sometimes disagree on when to start the treatment, what dose to prescribe, and how long to continue the medicine. The doctor's decision depends on the organ system involved and how far the inflammation has progressed. If the disease appears to be severe, especially in the lungs, eyes, heart, nervous system, spleen, or kidneys, the doctor may prescribe corticosteroid.

Corticosteroid treatment usually results in improvement. Symptoms often start up again, however, when it is stopped. Treatment, therefore, may be necessary for several years, sometimes for as long as the disease remains active or to prevent relapse.

Frequent checkups are important so that the doctor can monitor the illness and, if necessary, adjust the treatment.

Corticosteroids, for example, can have side effects: mood swings, swelling, and weight gain because the treatment tends to make the body hold on to water; high blood pressure; high blood sugar; and craving for food. Long-term use can affect the stomach, skin, and bones. This situation can bring on stomach pain, an ulcer, or acne or cause the loss of calcium from bones. However, if the corticosteroid is taken in carefully prescribed low doses, the benefits from the treatment are usually far greater than the problems.

Besides corticosteroid, various other drugs have been tried, but their effectiveness has not been established in controlled studies. These drugs include chloroquine (Aralen) and D-penicillamine. Several drugs such as chlorambucil (Leukeran), azathioprine (Imuran), methotrexate (Rheumatrex, Trexall), and cyclophosphamide (Cytoxan), which might suppress alveolitis by killing the cells that produce granulomas, have also been used. None have been evaluated in controlled clinical trials, and the risk of using these drugs must be compared closely with the benefits in preventing organ damage by the disease. They are not to be used by pregnant women.

Cyclosporine, a drug used widely in organ transplants to suppress immune reaction, has been evaluated in one controlled trial. It was found to be unsuccessful. More recently, thalidomide (Thalomid) has been used successfully in a limited number of patients and seemed to improve lung function and heal skin lesions. Infliximab (Remicade) has been reported recently as effective in treating patients with sarcoidosis.

There are many unanswered questions about sarcoidosis. Identifying the agent that causes the illness, along with the inflammatory mechanisms that set the stage for the alveolitis, granuloma formation, and fibrosis that characterized the disease is the major aim of researchers of sarcoidosis. Development of reliable methods of diagnosis, treatment, and eventually, the prevention of sarcoidosis is the ultimate goal.

Originally, scientists thought that sarcoidosis was caused by an acquired state of immunological inertness (anergy). This notion was revised when the technique of bronchoalveolar lavage provided access to a vast array of cells and cell-derived mediators operating in the lungs of sarcoidosis patients. Sarcoidosis is now believed to be associated with a complex mix of immunological disturbances involving simultaneous activation, as well as depression, of certain immunological functions.

Immunological studies on sarcoidosis patients show that many of the immune functions associated with thymus-derived white blood cells, called T-lymphocytes or T-cells, are depressed. The depression of this cellular component of systemic immune response is expressed in the inability of the patients to evoke a delayed hypersensitivity skin reaction (a positive skin test), when tested by the appropriate foreign substances, or antigen, underneath the skin.

In addition, the blood of sarcoidosis patients contains a reduced number of T-cells. These T-cells do not seem capable of responding normally when treated with substances known to stimulate the growth of laboratory-cultured T-cells. Neither do they produce their normal complement of immunological mediators, cytokines, through which the cells modify the behavior of other cells.

In contrast to the depression of the cellular immune response, humoral immune response of sarcoidosis patients is elevated. The humoral immune response is reflected by the production of circulating antibodies against a variety of exogenous antigens, including common viruses. This humoral component of systemic immune response is mediated by another class of lymphocytes known as B-lymphocytes, or B-cells, because they originate in the bone marrow.

In another indication of heightened humoral response, sarcoidosis patients seem prone to develop autoantibodies (antibodies against endogenous antigens) similar to rheumatoid factors.

With access to the cells and cell products in the lung tissue compartments through the bronchoalveolar technique, it also has become possible for researchers to complement the above investigations at the blood level with analysis of local inflammatory and immune events in the lungs. In contrast to what is seen at the systemic level, the cellular immune response in the lungs seems to be heightened rather than depressed.

The heightened cellular immune response in the diseased tissue is characterized by significant increases in activated T-lymphocytes with certain characteristic cell-surface antigens, as well as in activated alveolar macrophage. This pronounced, localized cellular response is also accompanied by the appearance in the lung of an array of mediators that are thought to contribute to the disease process; these include interleukin-1, interleukin-2, B-cell growth factor, B-cell differentiation factor, fibroblast growth factor, and fibronectin. Because a number of lung diseases follow respiratory tract infections, ascertaining whether a virus can be implicated in the events leading to sarcoidosis remains an important area of research.

Some recent observations seem to provide suggestive leads on this question. In these studies, the genes of cytomegalovirus (CMV), a common disease-causing virus, were introduced into lymphocytes, and the expression of the viral genes was studied. It was found that the viral genes were expressed both during acute infection of the cells and when the virus was not replicating in the cells. However, this expression seemed to take place only when the T-cells were activated by some injurious event. In addition, the product of a CMV gene was found capable of activating the gene in alveolar macrophage responsible for the production of interleukin-1. Since interleukin-1 levels are found to increase in alveolar macrophage from patients with sarcoidosis, this suggests that certain viral genes can enhance the production of inflammatory components associated with sarcoidosis. Whether these findings implicate viral infections in the disease process in sarcoidosis is unclear.

Currently, thalidomide is being studied as a treatment for sarcoidosis. Future research with viral models may provide clues to the molecular mechanisms that trigger alterations in white blood cell (lymphocyte and macrophage) regulation leading to sarcoidosis.

For difficult to treat (refractory) sarcoidosis and sarcoidosis involving the nervous system (neurosarcoidosis), recent research using biologic medications that inhibit tumor necrosis factor (TNF-blockers) has been beneficial. The TNF-blockers used were adalimumab (Humira) and infliximab.

Medicine and medications:

Sarcoidosis Drugs - Effectiveness & Side Effects

Medications are used to treat sarcoidosis. Most sarcoidosis medications are used to suppress the immune system. By suppressing the immune system, the body's defenses are lowered and a person has a greater risk of infection from other diseases. Sarcoidosis medications are usually strong and can have bad side effects.

Types of medication

Prednisone: Prednisone is a drug most often used to treat sarcoidosis. A corticosteroid, prednisone is used to relieve tissue inflammation (a common symptom of sarcoidosis). People with sarcoidosis normally respond to the effects of prednisone. Prednisone treatment can last several months to many years - the longer the duration of treatment the greater the chance that symptoms will not return.

However, prednisone can have bad side effects and the doctor and person with sarcoidosis must 'weigh up' the benefits from using this drug against possible side effects. Low doses of prednisone are often prescribed to relieve symptoms without causing significant side effects.

Prednisone side effects can include:

    High blood pressure.

  • Diabetes.

  • Heartburn.

  • Acne.

  • Weight gain.

  • Mood swings.

  • Sleep disturbance.

Long term treatment using prednisone can lead to other side effects:

    Osteoporosis - thinning of the bones & skin.

  • Cataracts.

  • Glaucoma.

Hydroxychloroquine: Hydroxychloroquine (brand name: Plaquenil) is another drug with anti-inflammatory properties. It is used to treat a number of other diseases including malaria, rheumatoid arthritis and lupus erythematosus.

Hydroxychloroquine is more effective at treating sarcoidosis of the skin and when blood calcium levels are high. There are fewer side effects with hydroxychloroquine compared to Prednisone. However, the drug is effective in only about a third of persons with sarcoidosis.

Hydroxychloroquine side effects include

  • Stomach irritation.

  • Eye problems - it is recommended that people who take hydroxychloroquine have their eyes tested every 6 months.

Methotrexate:

Methotrexate is another sarcoidosis medication which is also used to treat other diseases. These include cancer, psoriasis and rheumatoid arthritis. The drug works in a high percentage of people with sarcoidosis (about 60 to 80 percent). However, it takes up to 6 months to relieve symptoms and methotrexate has side effects which can be dangerous or life-threatening.

Methotrexate side effects include:

    Nausea.

  • Mouth sores.

  • An allergic reaction - can affect the lungs (rare).

  • Boils or acne.

  • Joint pain

Methotrexate can also kill white bloods cells which are used by the immune system to fight off infection. People taking this drug should have regular blood tests to check white blood cell levels.

Long term use of methotrexate can lead to serious liver problems. If methotrexate is taken for more than 2 years a biopsy should be carried out on the liver to see if there is damage & if this treatment can still be used.

When methotrexate is taken in small doses side effects are usually limited.

Folic acid can be taken to reduce the chances of side effects.

Azathioprine: Azathioprine (brand name: Imuran) is another multi-use drug & is used to treat a number of diseases including rheumatoid arthritis - it is also used with organ transplants. Azathioprine treatment lasts for 6 months or more. Azathioprine can improve the symptoms in about 50 percent of people with sarcoidosis.

Azathioprine side effects include:

    Nausea

  • Itchy rashes.

  • Sore mouth, throat and/or ulcers

  • Skin becomes sensitive to ultraviolet light.

  • A lowering of white blood cells - regular blood tests should be used to monitor white blood cell levels.

Cyclophosphamide: Cyclophosphamide (brand name: Cytoxan) is a powerful drug. Due to its potentially life threatening side effects it is used only in people with severe forms of the disease (such as neurosarcoidosis).

Cyclophosphamide side effects include:

    Irritation of the bladder.

  • Lowering of white blood cells.

  • Nausea.

  • Loss of appetite or weight.

  • Bladder cancer - found in some people who have been taking cyclophosphamide for more than 2 years.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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