What is Sarcoidosis?
Sarcoidosis is a condition characterized by growth of tiny inflammatory cells or granulomas in multiple system of the body most commonly the lungs, lymph nodes and the eyes. The condition of Sarcoidosis has no known etiology and it can develop and disappear suddenly or may develop gradually with symptoms that are recurrent.
The mortality for sarcoidosis is rather slight and death is usually linked to granulomas in the lungs that cause scarring and infection leading to respiratory failure subsequently death. Sarcoidosis is more common in male but female patient has a higher rate for extrapulmonary involvement, morbidity and mortality. The onset usually starts by the age of 25 to 35 years with recurrence in female by the age of 45 to 65 years.
Sarcoidosis is also known as Besnier-Boeck disease and Besnier-Boeck-Schaumann disease and simply called Sarcoid. The condition was first identified simultaneously by two physicians named Dr. Jonathan Hutchinson and Dr. Caesar Boeck and the condition was previously called Hutchinson’s disease or Boeck’s disease. The condition was later named Sarcoidosis fashioned after the characteristic of the condition.
Sarcoidosis is not potentially fatal and is in no relation to cancer or tuberculosis. Most patients get well without a need for treatment as the granulomas usually resolve on its own except for the symptoms that come and go which may need a close monitoring to prevent serious complications.
The condition of Sarcoidosis is usually asymptomatic and can only be identified by chance during the diagnostic test procedure for an unrelated health condition. If symptoms appear, it is often related to the areas or parts of the body where the granulomas developed.
The signs and symptoms of this condition vary and depend on the organ of the body that gets affected by growth of inflammatory cells. The general symptoms are usually vague and may sometimes be mistaken as a manifestation of another condition. The general symptoms however include the following:
- Dyspepsia and cough that would not relieve as among the first symptom
- Skin rashes manifest with sudden onset of granulomas
- Sudden weight loss
- Night sweats
- Onset of fever
- Dry eyes
- Blurry vision
- Pain and swelling of the knees
- General feeling of illness or body weakness
Manifestations in Lungs
The lungs is the most common site for growth of inflammatory cells and the symptoms usually involve the small blood vessels, bronchi and the alveoli.
- Non-stop dry cough
Manifestations in Skin
- Lesions that appear on the nose, ears and cheeks
- Maculopapular eruptions
- Skin nodules
- Changes in skin color over the area affected
Manifestation in Eyes
- Eye pain
- Dry eyes
- Blurry vision
- Light sensitivity
The liver can also get affected with granulomas although the symptom or liver dysfunction is clinically insignificant. The involvement of heart accounts for about a small percent of the overall reported cases of sarcoidosis although the symptoms can range from asymptomatic to fatal. Neurosarcoidosis manifest with optic nerve dysfunction, pituitary abnormalities and peripheral neuropathy to name a few.
Patients can have symptoms that are related to the affected organ of the body or they can only manifest the general symptoms as the condition of Sarcoidosis is generally asymptomatic. The degree of the symptoms also varies depending on how long the granulomas are in existence and the quantity of tissue affected.
Causes of Sarcoidosis
The exact cause of sarcoid remains unknown although theories and other factors are being considered and studied for the involvement in growth of inflammatory cells.
Sarcoidosis is believed to have a link with genetic predisposition although only insignificant numbers confirm the link with sarcoidosis. Significant numbers of infectious agents are also being linked to Sarcoidosis although the associations are not specific enough to have a direct implication with the condition. There have also been reported cases of Sarcoidosis being linked with organ transplant but is vague at the moment and still need more studies to confirm the association with Sarcoidosis.
Increase in Vitamin D production is also being linked with Sarcoidosis that an imbalance in the regulation can affect the hormones. The high level of estradiol and prolactin are theorized to have a link with Sarcoidosis. The high levels of Vitamin D are also believed to have a tie with the development of Sarcoidosis as implicated in the cause of dysfunction in the immune system.
How is Sarcoidosis diagnosed?
The condition of Sarcoidosis is rather difficult to diagnose especially during the early stage of the progression. Only a few signs and symptoms are produced during the early stage of the condition and the symptoms are rather vague.
The diagnosis is initially done with physical examination and medical history taking of the patients. The process of diagnosing Sarcoidosis is usually through elimination of other disease that has similar symptoms with Sarcoidosis. The elimination is important in differentiating Sarcoidosis with another condition that resulted in the enlargement of lymph nodes and shadowing revelation in x-ray results.
The process of elimination of diagnosing the condition is not a direct confirmation of Sarcoidosis. Doctors may also request for other diagnostic tests that have significance with the symptoms being manifested.
Chest x-rays are recommended to check for evidence of any lung damage and evidence of lymph node enlargement. PET or MRI procedures are suggested if Sarcoidosis is suspected to have affected the heart and the central nervous system.
Blood and serum tests are being done to evaluate elevations of levels of enzymes, proteins, amyloids and others that are suspected to have a link with the development of Sarcoidosis.
Pulmonary function test is utilized to monitor air trapping that is common in Sarcoidosis as supported by imaging tests. The chest radiograph is the imaging test that is central and basis for the evaluation of Sarcoidosis.
Tissue samples can also be collected for biopsy to check for evidence of granulomas. Tissue samples are usually taken from the affected area. The skin and eye tissues are the most common area for biopsy as it can be easily taken and is the commonest site for granulomas. A tissue sample in the lungs is obtained through a bronchoscopy procedure and is recommended for Sarcoidosis suspected to develop in the lungs.
Sarcoidosis has no exact cure and treatment is often directed towards relief of symptoms and if the development of Sarcoidosis is threatening the function of the organ involved.
Steroid treatment is given to pacify the inflammatory process and corticosteroid is the most commonly prescribed. It is usually the first-line of treatment that can be taken either orally or applied directly depending on what area or organ of the body is affected.
Non-steroid treatment is given to those who have contraindications to steroid treatment. This is not the most commonly used treatment for Sarcoidosis but it is being tried in patient with intolerance and has adverse effect of steroid treatment. It is also prescribed for those with steroid resistant disease and to those patients who do not wish to take steroid treatments.
Other medications are also being prescribed to relieve the symptoms of the condition. Anti-rejection medications are given to diminish inflammation by suppressing the immune system. Antimalarial medications are especially helpful to Sarcoidosis with skin involvement. Sarcoidosis with nervous system involvement and with elevations in blood calcium is also beneficial with anti-malarial medications.
Tumor necrosis factor alpha or TNF-alpha inhibitors are prescribed to patients who are unresponsive to other treatment for Sarcoidosis. This medication is commonly used to treat inflammation associated with rheumatoid arthritis.
Lung transplantation is indicated for patients with severe cases of sarcoidosis that is threatening the function of the lungs. This is strongly recommended for patients especially to those with advance stage of sarcoidosis. High mortality is expected in this situation as most cases of death in Sarcoidosis are related to respiratory organ failure. Other organs affected with Sarcoidosis and with functions that are threatened can also undergo organ transplantation if possible.
Sarcoidosis usually resolves on its own without the need of medical treatment. The condition however, can remit and may become chronic with exacerbation. Organ damage will have some degree of extent and can be fatal especially if Sarcoidosis is untreated.
Severe and progressive cases of Sarcoidosis are beneficial to medical treatment and are initially given at higher dosages. The dosage can only be reduced if the condition has been pacified. Sarcoidosis requires close monitoring regardless of the mode of treatment. This is to monitor whether the condition is progressing or responding well to the treatment.
Is Sarcoidosis Contagious?
Sarcoidosis is not a contagious condition but a self-limiting disease that can occur to anyone regardless of racial and gender classification. The mean onset is usually at the age of 20 to 35 years with remission occurring at the age of 45 years. There is no exact cause identified with the onset of the disease and neither a report for being contagious.