Sarcoidosis (Besnier-Boeck disease, Besnier-Boeck-Schaumann disease) is a multisystem inflammatory disease of unknown etiology, characterized by non-necrotizing granulomatous inflammation, predominantly in the lungs and intrathoracic lymph nodes.
The presentation of the disease is highly dependent on the extent of damage to the organ. 5% of cases have no outward clinical manifestations making it hard to be diagnosed. There are also cases where the condition can be asymptomatic. However, with the aid of a chest X-ray the condition of the lungs can be clearly seen. The most common complaints are hyperthermia, anorexia and arthralgia; this is accompanied by pulmonary symptoms. A group of symptoms consisting of fever, bilateral hilar lymphadenopathy and polyarthralgia has good prognosis.
Diagnosing sarcoidosis is not only done with one test. Since the disease is quite complex, it requires multiple combination laboratory examinations to come up with a concrete diagnosis. The signs and symptoms of sarcoidosis are very general as well, so the disease needs thorough medical examination. The following are the major laboratory examinations conducted to diagnose the disease.
These are just a few of the most important workups done to identify sarcoidosis. The first step in diagnosing sarcoidosis is to rule out other pulmonary conditions such as tuberculosis or COPD among others. As mentioned, the signs and symptoms are very general, they can occur and mimic that of other diseases; the reason why it’s important to set aside other diseases.
Most patients only require symptomatic treatment with non-steroidal anti-inflammatory drugs (NSAIDs). For extrapulmonary sarcoidosis, corticosteroid therapy is often indicated, although steroid-sparing agents (e.g. methotrexate, azathioprine) are often used as alternatives    .
When sarcoidosis is detected early, the prognosis can be very excellent. However, there are many cases of misdiagnosis because of the nature of its clinical manifestations which is very general. Although the disease can disappear over time, there are cases where it becomes chronic or remissions and relapse may occur. According to studies, half of sarcoidosis cases can be cured within 12 to 36 months while the other half remains chronic or can come and go for years.
The exact etiology of this disease is unknown. However, many physicians suggest that it is actually caused by a mix of environmental factors and genetics. There is also a study suggesting that it is caused by an alteration in the immune system’s normal response to external pathogens. However, what initiates the abnormal response is not known. There are a lot of probable causes mentioned but all these are just speculations that need further investigation.
Although the cause of sarcoidosis is unknown there are several risk factors which can trigger the disease. Age and gender are major considerations. The disease is most common in women and the targeted age group is mostly between the ages of 20 and 40. With regards to race, African Americans are more prone of having this disease. It can also be severe to this specific race than that of others. People who have family members that have sarcoidosis are likely to acquire the disease as well.
The cases of sarcoidosis are widely distributed all over the world. It can happen to anyone regardless of race or ethnicity. People under the age of 50 are more vulnerable with developing the disease; specifically its peak is highly noticeable at the age between 20 to 39 years old. The occurrence of sarcoidosis differs from one place or country to another. Environmental exposures, genetic considerations and predispositions are just a few examples of factors that affect the occurrence of the disease. According to research, the highest number of cases recorded each year came from Northern European countries with an average of 5 to 40 people having sarcoidosis in a sample of 100,000. In Asia, Japan has had 1 to 2 cases with the same sample population.
In the United States, black Americans have the tendency to develop sarcoidosis more than white Americans. Doctors observe that the disease occurs two to three times more in black Americans. Aside from this, the disease is more severe in this group of people; it may even lead to death. Based on gender, sarcoidosis is more prevalent in females. This data is consistent among all races. While there are many articles saying that sarcoidosis is influenced by socioeconomic status, this is actually a myth. The risk factors for this disease are the same in both the rich and the poor.
The development of sarcoidosis starts when an outside pathogen is inhaled. With a foreign body inside the lungs this will then trigger the initiation of the inflammatory process. Immune mediating cells such as giant cells, compose the core, surrounded by an outer rim of T lymphocytes will start migrating to the area in order to help in the normal healing process.
Non-necrotizing granulomas will start to develop and a lesion is formed inside the lungs – this is now called a sarcoid inflammation. A granuloma is a solid formed cell that blocks external pathogens. The core of the latter is composed of epithelioid histiocytes and multinucleated giant cells while the external wall consist of T lymphocytes. T cells play a major role in the occurrence of sarcoidosis because they elicit an exaggerated immune response.
Lymphocytes move tightly together during electrophoresis, they also have limited T-cell receptors and a steady antigen-driven process. The inflammatory process of sarcoidosis is reliant on a consistent initiation of CD4+ T cells. A difference in genetic make-up and T cell receptors which regulates the affinity of antigens is the reason for the development of the disease.
Several studies have been conducted to determine the specific cause of sarcoidosis.
It is still unclear whether it is caused by a single factor or by an alteration in the normal response of the immune system. At present, only identifiable risk factors are known.
The classification for sarcoidosis is identified according to the results of a chest X-ray as well as the body part or organ affected. The following are classified stages according to chest X-ray results.
As mentioned, Sarcoidosis can also be classified according to the affected organ. These are just some of the most common ones.
There are no guidelines for prevention of sarcoidosis.
Sarcoidosis is one of the many diseases that are idiopathic in nature. Considered an inflammatory disease, sarcoidosis is characterized by the presence of granulomas which are mainly composed of accumulated inflammatory cells such as T-lymphocytes. Sarcoidosis can occur at any part and organ in the body. However, it is most common in the lungs and the lymph nodes. This disease may cause a disruption in the physiological function of the organ or affect its overall structure.
An exact cure for the treatment of sarcoidosis has yet to be discovered. Although there is no cure at present, patients of the disease respond well with adjunct therapies.
The occurrence of sarcoidosis is quite unpredictable; it can occur at any time without any trigger and disappear on its own. Its symptoms may also become dormant and resurface after several years or the person can carry it throughout his lifetime. While the prognosis for sarcoidosis is generally good, there are severe cases of the disease where it can lead to organ damage and eventually failure.
Though uncommon, sarcoidosis is not a new disease. It was discovered over 100 years ago by two well-known dermatologists, Dr. Jonathan Hutchinson from England and Dr. Caesar Boeck from Norway. Before the term Sarcoidosis was coined the disease was called Hutchinson's disease or Boeck's disease. It was later changed to sarcoidosis which means flesh-like. The latter is a term used to describe breaks in the skin which are highly evident in people with sarcoidosis.
Once you are diagnosed with sarcoidosis, you have to immediately consult a specialist for your condition. Scheduled check-ups, follow-ups and daily monitoring must be done to observe the progression of the disease. It is also best to religiously undergo laboratory studies as to monitor whether you are getting well or not.
Following treatment regimens and listing unusual symptoms will also help in the developing a care plan. It is also important to remember that once you have sarcoidosis you have to get an annual shot of influenza immunization. Since sarcoidosis is a lung disorder, if smoking is a habit you have to find ways to slowly stop it. Consult an expert and seek for advice, immediately stopping might cause withdrawal symptoms which may be very harmful.
McKinzie BP, Bullington WM, Mazur JE, Judson MA. Efficacy of short-course, low-dose corticosteroid therapy for acute pulmonary sarcoidosis exacerbations. Am J Med Sci. Jan 2010;339(1):1-4.
Pietinalho A, Tukiainen P, Haahtela T, Persson T, Selroos O. Early treatment of stage II sarcoidosis improves 5-year pulmonary function. Chest. Jan 2002;121(1):24-31.