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Sarcoidosis

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The illustration shows the major signs and symptoms of sarcoidosis and a few organs involved

Sarcoidosis is both an asymptomatic and chronic inflammatory disease that causes inflammation of tissue. It produces granulomas that can accumulate in virtually any organ in the human body. Granulomas are ball-like collection of immune cells that form when the immune system attempts to wall off substances that it perceives as foreign but is unable to eliminate. When these granulomas clump together they form large groups called nodules. That affects the organs and may damage the normal internal structures, ultimately causing the organ to fail. The cause of this disease remains unknown and no cure has been found.

Signs and Symptoms

Reddish-brownish lesions a type of symptom with sarcoidosis

The symptoms of sarcoidosis vary in different people and can range from mild to severe. Often people with Sarcoidosis develop minor symptoms or no symptoms at all, making it difficult to diagnose the disease. Due to its ability to infect a number of organs it further camouflages the symptoms. However, many times these mild symptoms do not progress, but stay at equilibrium. Major symptoms that develop rapidly and pose a serious risk are rare. Generally people develop fevers and night sweats which can periodically reoccur throughout the extent of the illness. Other common symptoms that occur are fatigue, vague chest pain, malaise (a feeling of illness), weight loss, and aching joints. Many peoples’ symptoms develop entirely on what extend and specific organ is affected.[1] [2]

Lungs: Throughout all others organs sarcoidosis can infect, the lungs are the most commonly infected. Lymph nodes become inflamed where the lungs meet the heart and to the right of the trachea (windpipe) enabling them to easily be seen through an x-ray. Inflammation commonly forms in the lung, which through progression can lead to scaring and the eventual formation of cysts, producing a cough and shortness of breath. The cysts can also be colonized by the fungus Aspergillus. When this fungus begins growing it causes bleeding in the lungs increasing the difficulty to breathe.[2]

Skin: The skin is another commonly infected organ. Bumps develop called Erythema Nodosum which appears on shins and ankles. Theses bumps are raised and have a reddish hue. These bumps commonly develop fevers, swelling of ankles and joint pain. Lupus Perenio (disfiguring skin sores) is a serious skin condition that dramatically affect many body regions specifically the face. This condition is very persistent and can reappear after the treatment has been completed. [3]

Liver and Spleen: Liver symptoms are very minimal. Commonly developed symptoms are a fever, fatigue, and pain around the upper right region of the abdomen.[3] Although 70% of people with Sarcoidosis have granulomas in their liver, only 10% have an enlarged liver. Also the spleen can become enlarged. [2]

Eyes: Eyes are another organ accessible to sarcoidosis. Approximately 15% of people with sarcoidosis have eye conditions related to the disease. Granulomas form in the conjunctiva (the membrane over the eyeball and inside the eyelids), limiting the tear gland productivity and resulting in sore, dry, and red eyes. Sarcoidosis can also affect internal eye structures, harming vision and causing the eyes to become red. If left untreated this inflammation will continue and block drainage from the eyes causing glaucoma and if uncured can lead to blindness. [2]

Heart: Cor pulmonale (right-sided heart failure) is started by the lungs being heavily infected with sarcoidosis. This results in the strain on the right side of the heart and eventually heart failure. Heart failure can also occur by the build up of granulomas. These granulomas can also affect the heartbeat if developed too close the electrical conducting system of the heart. [2]

Joints and Bones: When joints are targeted by sarcoidosis it results in inflammation. Although mainly seen in the feet, ankles and hand it can exist throughout the entire body. In addition arthritis can form, which can be either painful or painless and last weeks or months. Bones are dramatically affected by granulomas,they affect bone marrow and even put painless holes in the bones. [3]

Nervous System: The nervous system can be destroyed by the affect of sarcoidosis. The nervous system exhibits numerous symptoms including vision problems, numbness or weakness in a leg or arm, and headaches. This infection can affect the cranial nerves, causing one side of the person’s face to droop as a result of destroyed nerves. If the surrounding organs including the pituitary gland and the bones become affected diabetes insipidus might possibly set in, due to the stop of vasopressin production. [2][3]

High Calcium Levels: The sarcoidosis granulomas cause the accumulation of calcium in the blood and urine. Due to the fact that the granulomas produce activated vitamin D which increases the calcium absorption. Many symptoms both minor and major occur from this high calcium level in the blood. Minor symptoms include loss of appetite, nausea, vomiting, thirst, and excessive urine production, but if left untreated for a long period of time these increased calcium levels result in deposits in the kidney and produce kidney stones. [2]

Diagnosis

Chest x-ray showing the typical nodularity of sarcoidosis in the lungs of a person with sarcoidosis

Sarcoidosis affects a number of organs, making it difficult to diagnose using a single method. In addition its symptom closely resemble other diseases symptoms further adding to the difficulty of a diagnosis. By both an examination and a medical history review, sarcoidosis can be found relatively fast. [4] To aid in a speedy diagnosis many test and examinations are used to rule out other causes of the symptoms and assess the damaged caused by sarcoidosis. [3] Many beginning tests begin with a blood test and move on to other test based on organ that are thought to be infected. However, a few basic tests are the chest x-ray, pulmonary function tests and a biopsy. [1]

Chest X Ray: Healthcare workers often begin the diagnosis with a chest x-ray and over 95% of people with sarcoidosis show an abnormal chest x-ray. This is often used due to the suspicions of pneumonia. If granulomas or enlarged lymph nodes are shown the diagnosis is then highly positive to be sarcoidosis. However, to verify this diagnosis other tests are used in conjunction with a chest x-ray, including a CT scan. [2]

Lung Function Tests: Sometimes called pulmonary function (breathing) tests are used to evaluate the condition of the lungs. This test is often used when the sarcoidosis has affected the lung and to conform a chest x-ray. [4] These test measure the size of one’s lungs and how much air one can breathe in and out. It also evaluates the efficiency of the lungs to deliver oxygen to the blood. [1]

Biopsy: This test is often used to rule out any other symptom related to sarcoidosis. These biopsies are dependant on which body organ is affected by this disease. This test is often used to confirm the diagnosis of the health care worker. This biopsy is done by taking a small amount of tissue from the area said to be infected, then it is tested for the disease. While most of the time the biopsy takes place around the surface of the body, the healthcare provider might deem it necessary to do a biopsy in the lungs. [1] This biopsy is often referred to a bronchoscopy. While being under local anesthesia a small tube with a light on the end is passed down the trachea and into the bronchial tubes of the lungs. This specific biopsy is used to inspect the bronchial tubes of the lungs and withdraw a sample for testing and examination for inflammation. [3]

Treatment

Although sarcoidosis has no cure, many of the symptoms can be treated. The therapy used is very diverse and based on the organ affected. Due to the very minimal symptoms produced by sarcoidosis, many people do not need any treatment, but just let the disease run its course. These symptoms are usually not disabling and reverse rapidly. When the symptoms are not prevalent anymore the disease is said to be inactive [5] Based on the stage of sarcoidosis there are two different treatment. One is good health practices and the other is drug treatment. However, when the disease begins to affect the functionality of the organ medications are used to control the degeneration and inflammation. One of the main drugs that are used in the treatment is corticosteroids. The main corticosteroid used is prednisone. While it relives the symptoms of sarcoidosis it still has some minor side effects, these include difficulty sleeping, heartburn, acne, glaucoma, cataracts, weight gain, diabetes, high blood pressure, mood swings, depression, osteoporosis, adrenal glad insufficiency, and aseptic or avascular necrosis. [3] There is still some controversy in using corticosteroid and their optimal doses. While the common doses are 60 to 80mg of prednisone, new studies are showing a reduced initial dose to 30mg is equally affective as the larger dose. The goal is to have at least 10-15mg of prednisone over a period of six months and as disease conditions improve, a slow reduction in dosing is used, until the medication is completely discontinued. [6] When one becomes unable to take prednisone due to either lack of immunity or tolerance, other medication can take its place. In addition to corticosteroid, there are medications used to treat a specific organ or used in place of corticosteroids. These medications are taken orally or applied topically too affected areas and includes. [3] [7]

  • Hydroxychloroquine: This is often prescribed to people suffering from a skin and brain disorder caused by sarcoidosis. Also it is prescribed when high levels of calcium are found in the blood.[1]
  • Methotrexate: Treats multiple organs suffering from sarcoidosis including the lungs, eyes, skin and joint of the body. [1]
  • Azathioprine: Due to its lack of effectiveness it is often not prescribed, it only works in about half of people with sarcoidosis. In addition it takes about six months to become effective and begin to reduce the symptoms.[3][7]
  • Cyclophosphamide: Is rarely given to patients with sarcoidosis, due to it highly toxic nature. Although it works, it is only prescribed to people with neurosarcoidosis. Its side effects can be deadly to the body and include a reduction in white cell count, nausea and a risk of cancer.[3][7]
Six bottles of different types of cancer drugs including Cytoxan (cyclophosphamide), an alternative medication for sarcoidosis.

Potential New Medications

Due to the increased need of alternative sarcoidosis medication, researchers have been particularly studying the immune system and testing alternate medications and their potentials in treating the disease. Further testing is being done on the effectiveness of using antibiotics in the treatment of sarcoidosis. Researches have even begun using medications that would normally treat other diseases and ailments and have begun to be use them in the treatment of sarcoidosis.[1] [3]

  • Etanercept: While it is an immune suppressant that is injected into a person’s arm to treat rheumatoid arthritis, it has also shown through studies that it can be used to treat sarcoidosis.[3]
  • Pentoxifylline: This medication while an immune suppressant can help reduce the amount of prednisone if taken in conjunction with this medication.[3]
  • Thalidomide: Scientist have be studying the usefulness of this medication. While it is a immune suppressant, it is being studied in reducing and effectively stopping the granulomas of the skin.[3]
  • Tetracycline: This antibiotic is commonly used to treat lyme disease, forms of pneumonia, and acne. During multiple evaluations it is suggested that it can help with sarcoidosis in the skin. [3]

Epidemiology

Sarcoidosis affects a number of people. While it can happen in anyone, many factor can increase or decrees this risk. These factors include geographic location, race, ethnicity, age, and gender. [8] However, females have a higher risk of developing the disease then males. Based on certain continents the disease prevalence increases or decrease by race. For example in the United States the disease is more prevalent in blacks with a 10:1 to 17:1 ratio. While in Europe it is higher in whites then black, with an even higher prevalence in Sweden and Iris females. [5] One of the highest incident rates of sarcoidosis is in Scandinavia, which has a rate of 50-60 cases of sarcoidosis per 100,000 population. [6] These increases risk are often a result of a lack of proper medical centers with the equipment necessary for diagnosing this disease, rather then differences in human genetics. [8] Generally people between the ages of 20 to 40 have a higher risk of contracting the disease, with a peak in females over the age of 50. [9] Children between the ages of 9 to 15 more frequently develop the disease, while children under the age of 4 to 1 can contract the disease, but it is very rare. [5]

Lifestyle

Living with sarcoidosis can be very challenging. Since there is not a cure for it, people have to alter their lifestyles. One of the most important steps in managing you sarcoidosis is by routine visits to a doctor. These visits will help control the worsening of the disease. Doctors will often perform tests each visit to monitor the disease and stop the potential spreading to other organs. They will also provide a plan for controlling the symptoms. In addition to seeing a regular practitioner it is often advisable that one visits a specialist. This specialist can either specialize in sarcoidosis or the organs affected by the disease and aid in helping one’s general doctor. [1] In addition to routine checkup, healthy lifestyles help control many of the symptoms. These healthier lifestyles include quitting smoking, eating healthier, getting more exercise, and avoiding harmful substances like chemical and dust. If completed these basic changes can help reduce the risk of reoccurring sarcoidosis. [10]


Many symptoms of sarcoidosis can become overwhelming. If one is living with chronic sarcoidosis it can add unnecessary stress. This stress can often lead to depression, but through one’s health care professional these can be eliminated. They can appoint counselors and other professional to help you get through these struggles. Drugs are used to help control these emotional issue regarding the disease. Many people join local support groups that help provide emotional guidance. [1] Pregnancy can pose another serious issue with females suffering from sarcoidosis. While corticosteroids are safe to take during pregnancy, most of the other medications are not recommended to take during pregnancy. People with severs symptoms must find alternate methods to control the symptoms. Many females have given birth while having sarcoidosis, since it often does not affect the disease, but some cases hove shown that the symptoms have increased after giving birth. [10]

Gallery

References

  1. 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 1.8 Sarcoidosis Information National Heart Lung and Blood Institute, February 2009.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 Sarcoidosis Merck Manual Home Edition,Talmadge E. King, Jr., MD, March 2008.
  3. 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 3.12 3.13 3.14 3.15 Defining Sarcoidosis Thomas C. Weiss, Disabled world, January 2009.
  4. 4.0 4.1 Sarcoidosis WebMD, Accessed 12/8/10.
  5. 5.0 5.1 5.2 Sarcoidosis e-radiography.net, Accesses December 2010.
  6. 6.0 6.1 Cardiac Sarcoidosis Doughan, A.; Williams, B, BMJ Publishing Group and British Cardiovascular Society, February 2006.
  7. 7.0 7.1 7.2 Sarcoidosis Treatment University of California San Francisco, last updated December 2010.
  8. 8.0 8.1 Sarcoidosis Epidemiology The Cleveland Clinic Center,Daniel A. Culver, Accessed 12/9/10.
  9. Sarcoidosis Orphanet J Rare Dis, BioMed Central Ltd, November 2007.
  10. 10.0 10.1 living with Sarcoidosis by www.emedtv.com, Arthur Schoenstadt, MD, May 21, 2010.

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