Sarcoidosis, or Boeck’s disease, is a rare disorder that is primarily manifested by inflammatory granulomas (small nodules). Although all organs of the human body can be affected by sarcoidosis, the lymph nodes and lungs are more commonly affected. The exact cause of Boeck’s disease is not yet sufficiently known, but various environmental factors are thought to be involved.
What is sarcoidosis?
Sarcoidosis, also known as Boeck’s disease in medical circles, is one of the diseases that primarily affects soft tissue structures due to its course and the predominantly affected organ systems. In addition to the lung tissue and structures, sarcoidosis can affect the skin, the eyes, the bone system, other organs such as the spleen and the liver, as well as the vital heart. Some courses of the disease have shown that sarcoidosis also extends to the nerves and pancreas. Sarcoidosis is characterized by the development of nodule-like tissue balloons that are bordered by foci. These collections of tissue and cells are equally known as granulomas because histologically they have a misshapen surface. Sarcoidosis may represent a specific immune system response that manifests itself in a concentration of cellular defense elements. Sarcoidosis disease is predominantly seen between the ages of 20 and 40.
Causes
Initially, the causes of sarcoidosis, also called granulomatosis, are based only on conjecture, because the disease itself and the functioning of the human immune system have not yet been adequately studied. In addition to the body’s immune-driven activities, it is believed that sarcoidosis is genetic. It is possible that different environmental factors also contribute to the development of sarcoidosis.
Symptoms, complaints, and signs
The symptomatology of sarcoidosis depends on the organ system affected, and often affected individuals remain completely symptom-free. As a rule, the disease appears in the chronic form, which begins insidiously: its nonspecific signs include general malaise, joint pain, and weight loss; body temperature may be slightly elevated. Symptoms of the common pulmonary sarcoidosis may include exertional dyspnea and chronic irritable cough; swelling of the lymph nodes in the chest is often discovered only by chance during an X-ray examination. If Boeck’s disease affects the eyes, inflammation of the iris typically develops, accompanied by photophobia and eye pain. Involvement of the skin is manifested by characteristic skin changes: Typical of nodular erysipelas is the severely painful nodule formation in the subcutaneous fatty tissue associated with skin redness, predominantly on the lower legs. Bluish-purple skin discolorations (lupus pernio) on the face also occur. A pronounced affection of the heart often results in cardiac arrhythmias or cardiac insufficiency requiring treatment. In the kidneys, sarcoidosis favors the excretion of calcium and thus the formation of urinary stones. Rarely, neurosarcoidosis appears in the form of meningitis or paralysis of the facial muscle (facial paresis). Acute sarcoidosis is characterized by a sudden onset with fever and severe fatigue, typically accompanied by skin changes described as nodular rosacea, joint inflammation in the ankle, and lymph node swelling in the lungs. Bluish-purple skin discolorations (lupus pernio) on the face also occur. A pronounced affection of the heart often results in cardiac arrhythmias or cardiac insufficiency requiring treatment. In the kidneys, sarcoidosis favors the excretion of calcium and thus the formation of urinary stones. Rarely, neurosarcoidosis appears in the form of meningitis or paralysis of the facial muscle (facial paresis).
Course of the disease
Acute sarcoidosis may be initiated by the sudden onset of fever and the general symptoms of infection. These general signs of illness include fatigue, severe lassitude, loss of appetite, and aching limbs. The final diagnosis of granulomatosis can only be made by specific clinical pictures that appear on the affected organs.Cardiac and respiratory problems, impaired digestion and immune defense as well as changes in the blood count and altered liver values indicate the existence of sarcoidosis. In addition to the acute form of progression, chronic sarcoidosis tends to be recognized by its gradual progression. Some patients suffer from sarcoidosis and do not show any symptoms. In the context of a possibly occurring restriction of the respiratory and pulmonary function, an accumulation of granulomas can often be diagnosed in the evaluation of an X-ray. This is a clear indication of sarcoidosis. The nodules are sometimes so small that they can be seen individually only under microscopic magnification. They are sometimes also found in the lymph nodes. In addition, joint pain of the ankle, hematoma-like patches on the lower legs, and inflammation of the iris occur as definite signs.
Complications
Chronic sarcoidosis can cause various complications as the disease progresses. The specific symptoms that occur depend on which organ is affected. In the lungs, Boeck disease can cause pulmonary fibrosis, during the course of which changes occur in the heart and eventually right heart failure. Nodule formation in the kidneys leads to renal insufficiency and associated damage to the kidney tissue, cardiac arrhythmias and disturbances in bone metabolism. If the eyes are affected, visual disturbances occur in the course of sarcoidosis and complete blindness may result. Furthermore, the chronic form of the disease can cause permanent paralysis, splenic disorders, skin damage and other complications. Acute sarcoidosis usually does not result in long-term symptoms. Side effects are conceivable with treatment of both forms. For example, the cortisone preparations prescribed for infestation of the eyes, liver, skin, heart, and nerves can cause nerve damage and gastrointestinal discomfort. Anti-inflammatory and painkilling drugs also harbor corresponding risks. Physically weakened people and patients already taking other medications are particularly at risk. However, serious complications do not usually occur.
When should you go to the doctor?
Symptoms such as muscle and limb pain, fever, and a general feeling of illness may indicate sarcoidosis. A visit to the doctor is indicated if the symptoms persist for several days or even become more intense. If the typical nodules appear on the mucous membrane, a doctor must be consulted. If the complaints occur after inhalation of chemicals, fine dust, bacteria, fungi or allergy-causing substances, it is best to consult the family doctor immediately. People with lung diseases also belong to the risk groups and should consult the doctor if the described symptoms occur. In addition to the family doctor, the complaints can be taken to a lung specialist or a dermatologist, depending on the symptom picture and the localization of the nodules. Children should be presented to the pediatrician immediately if respiratory problems or skin and mucous membrane changes occur. Boeck’s disease requires comprehensive treatment and close monitoring. The physician must be informed of symptoms and complaints, as well as any side effects triggered by the prescribed medication, to rule out complications.
Treatment and therapy
Medications represent the basis of treatment for sarcoidosis. These are needed mainly in the chronic course; acute sarcoidosis usually requires little therapy. In addition to drugs for lowering fever temperatures, conventional physicians have at their disposal substances that prevent inflammatory processes in the body. Since granulomatosis is associated with painful impairments, which in the case of the joints predominantly resemble the conditions of arthritis, painkillers are administered. So-called non-steroidal substances have proven effective in this context. These are known as acetylsalicylic acid and ibuprofen. The therapy always depends on the individual manifestation of the disease and on the occurring impairments of other organs. If the granulomas occur in the eyes, nerves and kidneys, then the highly effective cortisone is used as a remedy. The good effect of the cortisone is that the strong immunological activities are slowed down.If cortisone is poorly tolerated or not tolerated at all, then the doctor may also prescribe methotextran or azathioprim.
Follow-up
The decisive factors for the follow-up of sarcoidosis (Boeck’s disease) are its course (acute or chronic) and the severity of the symptoms. In the case of the acute form of the disease (30 percent of cases), the aftercare includes the continuation of the measures started therapeutically until the disease has healed. Sarcoidosis heals completely in about 95 percent of acute cases. The nodules regress spontaneously within a few months. Until the acute disease has healed, regular visits to the general practitioner or lung specialist may be necessary. In the remaining five percent of cases of acute sarcoidosis, the affected individuals die. The focus of follow-up care is then the therapeutic treatment of the bereaved. To cope with the grief, psychotherapeutic counseling is generally recommended by physicians for the bereaved. In the chronic course of sarcoidosis (70 percent of cases), the function of the lungs remains permanently impaired in 20 to 30 percent of patients, depending on the severity of the disease. In this case, follow-up care focuses on the damage to the lungs. Regular lung function tests are therefore urgently needed, also in order to detect a possible deterioration in lung function at an early stage and also to be able to treat it in time. In addition, appropriate rehabilitation measures and endurance sports can improve lung function in individual cases. For rehabilitation measures, clinics located by the sea are recommended.
What you can do yourself
Although the cause of the disease could not be completely clarified to this day, based on the results of various studies and courses of the disease, the first indications show that an involvement of the immune system takes place. Therefore, in the field of self-help, a strengthening of the body’s own defense system should take place. Sufficient movement, a healthy and vitamin-rich nutrition as well as a good sleep hygiene promote the activity of the organism. The possibilities of movement are to be examined in such a way that no situation of overexertion occurs. Eating fresh foods and avoiding unhealthy meals can do much to improve well-being. The consumption of alcohol and nicotine should be generally refrained from. Stays in fresh air and adequate rest are also advisable. If complaints occur, rest periods should be taken. Sleep hygiene should be checked at regular intervals and optimized if possible. Attention should be paid to an appropriate room temperature, optimal sleeping utensils and the avoidance of disturbing noises. In everyday life, stress and hectic activity should be kept to a minimum. Long-term studies have shown that they weaken the human organism and thus the immune system. When reading, writing or watching television, attention should be paid to suitable lighting conditions. Many patients complain of eye pain and exposure to light that is too bright.
