What is Sarcoidosis?

Sarcoidosis treatmentSarcoidosis is a rare inflammatory disease characterized by the presence of small nodules (granulomas). Most affected organs are the lungs and lymph nodes, although the lesions can manifest in almost all organs of the body.

These new nodular formations of sarcoidosis are due to a failure in the regulation of the immune system. So far it is not known for sure why this failure triggers sarcoidosis. There may be a relationship between sarcoidosis and hereditary or environmental factors. Sarcoidosis usually develops in the 20s and 40s, and affects men to a lesser extent than women.

Approximately half of all patients with sarcoidosis present with discomfort. These can be as in all inflammatory diseases, general symptoms such as fever, general malaise, fatigue or pain in the extremities. In addition, sarcoidosis can occur with more symptoms since the nodules of the disease can affect each of the organs. Apart from the lungs and lymph nodes, it mainly affects the liver, spleen, heart, eyes, skin, bones and joints, and also the nervous system. In the most common case of sarcoidosis in the lungs, several stages can be distinguished depending on the extent of lung involvement:

  • Stage 0: affects organs outside the chest, without involvement of the lungs
  • Stage I: Inflammation of the lymph nodes between the two lungs, although it goes unnoticed in the lung tissue
  • Stage II: In addition to inflamed lymph nodes, irregular changes can be seen in the lung
  • Stage III: changes in lung tissue without inflammation of the lymph nodes
  • Stage IV: Pulmonary fibrosis

Because of the wide variety of symptoms, sarcoidosis is not easy to diagnose. In this case, a comprehensive diagnosis is required which includes imaging procedures, laboratory tests, functional tests and tissue samples. Sarcoidosis can be both acute and chronic. The prognosis of acute sarcoidosis is more positive. In this case, it can be cured without complications and even without treatment. However, the prognosis of the chronic type is worse. In general, chronic sarcoidosis responds well to corticosteroids, although in some cases (despite treatment) it is not possible to avoid limitations of lung function. Approximately 5% of patients with chronic sarcoidosis die from the disease.


What is the definition of Sarcoidosis?

The term sarcoidosis, by definition, refers to a very rare inflammatory disease characterized by clusters of small nodules (granulomas). For this reason it is also called granulomatosis. These new nodular formations composed of granular tissue are the consequence of a regulatory failure of the immune system.

Sarcoidosis

Depending on how this dysfunction is reached, sarcoidosis produces a series of nodules with different histological structures such as:

  • Infectious granuloma in tuberculosis
  • Foreign body granulomas
  • Rheumatic granuloma

Sarcoidosis mainly affects the lungs, liver, eyes, lymph nodes, heart and brain, although it can attack virtually any organ. This pathology is usually present in both acute and chronic forms and is not contagious.

Incidence of Sarcoidosis

Sarcoidosis usually affects people between the ages of 20 and 40. Each year, 10 to 40 per 100,000 people develop sarcoidosis. Men suffer less than women. In some population groups (eg, African Americans) and countries (eg, Sweden or Iceland), sarcoidosis is relatively common. Among the possible causes of this concentration of cases are hereditary and environmental factors.


Sarcoidosis Causes

At present, the underlying causes of sarcoidosis are not known for sure. There are several factors that play an important role in the onset of this disease.

Pollutants absorbed by respiration

When sarcoidosis occurs, characteristic nodules appear in the lungs, so a possible cause is contaminants that are absorbed by breathing (inhaled harmful agents). It is obvious that these substances activate the immune system to trigger the nodular formations typical of sarcoidosis, since the lungs may be affected both from the beginning of the disease and at the end. It is not yet known what the absorbed substances responsible for triggering sarcoidosis are. Bacteria, fungi or viruses are suspected as well as chemicals, pollen or dust.

Hereditary factors

It is also possible that the causes are certain hereditary (genetic) factors, since 5% of cases occur within the same family. In addition, certain changes in the carrier of the hereditary factor (genetic mutations) raise the risk of suffering from sarcoidosis.


What are the symptoms of sarcoidosis?

Sarcoidosis can present very varied symptoms, since any organ can be affected by the nodules characteristic of this disease. In addition to the characteristic discomfort for each affected organ, sarcoidosis (like all inflammatory diseases) can manifest itself through general symptoms: fever, general malaise, fatigue or body pain are possible signs of this disease. Approximately 1 in 2 patients does not feel any discomfort.

Sarcoidosis Symptoms

Sarcoidosis most often affects the lungs and lymph nodes in the space between the two lungs (the mediastinum). Other organs commonly affected by sarcoidosis are the liver, spleen, heart, eyes, bones and joints as well as the nervous system. The symptoms of sarcoidosis appear depending on the affected organ. Over time, acute and chronic sarcoidosis can be distinguished.

Acute sarcoidosis

If sarcoidosis is manifested by suddenly appearing symptoms, it is acute sarcoidosis. Most of the time there is acute high fever and a very pronounced general malaise. There are also the discomforts caused by each of the involved organs.

Acute sarcoidosis may occur in the form of Lofgren’s syndrome. In this case, the characteristic symptoms are, in addition to the sudden rise in fever, the following three changes:

  • Erythema nodosum (skin disease)
  • Arthritis (inflammation of the joints)
  • Inflammation of the lymph nodes in the area of ​​entry and exit of the nerves, vessels and bronchi on the side of the lungs, located between the two lungs (mediastinum)

This inflammation of the lymph nodes can only be determined by an x-ray or a computed tomography of the thorax.

Chronic sarcoidosis

If sarcoidosis does not initially occur with clear symptoms or begins with depletion or decreased performance, it is a chronic sarcoidosis. There may be no discomfort related to affected organs. The diagnosis is often shown by chance on a chest x-ray. The prognosis of chronic sarcoidosis is worse than acute type. Patients who have sarcoidosis do not often feel discomfort, treatment is usually delayed.

Depending on the organ that is affected by sarcoidosis, in addition to the symptoms typical of inflammatory diseases (fever, malaise, fatigue and pain in the extremities) may appear the discomfort of each organ involved.

For example, Lofgren’s syndrome is a set of symptoms characteristic of some people with sarcoidosis. Among them, patients usually suffer from fever, enlarged lymph nodes, arthritis and knotty erythema.

At the following, you may see the conditions caused by sarcoidosis in each organ:

  • Lymph nodes (90%): Inflammation
  • Lungs (95%): Cough and shortness of breath (very rare)
  • Liver (50-70%): Liver inflammation (often without discomfort), elevated liver values
  • Spleen (50-70%): Inflammation of the spleen (usually occurs without discomfort), although sometimes appears the syndrome of hypersplenism, which is characterized by a decrease in the number of red blood cells, white blood cells and platelets
  • Heart (20-30%): Heart rhythm disturbances, impaired heart valve function and pericardial effusion
  • Eyes (5-10%): Changes in vision, pain and photophobia
  • Skin (10-25%) Erythema nodosum: reddish and bluish painful skin nodules, almost always found in the lower extremities; Cutaneous sarcoidosis: bright nodules, reddish-blue to grayish, especially on the face
  • Kidney (10%): Increased protein and calcium clearance in the urine
  • Bones (10%): Slight disintegration of the bone structure (osteolysis), mainly in the small bones of the hands and feet
  • Joints (up to 10%): Inflammation of several joints (polyarthritis), mainly in ankles, knees and wrists; Joint effusion, tenosynovitis
  • Nervous system (10%): Paralysis, headache, increased intracranial pressure and epileptic seizures

How is sarcoidosis diagnosed?

Because of the large number of symptoms it presents, it is not easy to establish the diagnosis of sarcoidosis. The essential diagnosis includes, among other tests, imaging procedures, laboratory tests, functional tests and a thorough analysis of tissue samples.

Diagnpse of sarcoidosis

As in most cases the lungs are affected by the characteristic nodules of sarcoidosis, an X-ray of the chest is necessary to establish the diagnosis. The extent to which the lungs are affected by sarcoidosis can be divided into several stages. The radiograph shows the stage of lung involvement:

  • Stage 0: affects organs outside the chest, without involvement of the lungs
  • Stage I: Inflammation of the lymph nodes between the two lungs, although it goes unnoticed in the lung tissue.
  • Stage II: In addition to swollen lymph nodes, irregular changes in the lung can be seen
  • Stage III: changes in lung tissue without inflammation of the lymph nodes
  • Stage IV: Pulmonary fibrosis

If sarcoidosis is associated with a lung infection, the doctor also checks for lung function (lung function tests). To confirm the diagnosis of sarcoidosis, bronchoscopy or mediastinoscopy may be performed. Bronchoscopy generally consists of a bronchial lavage wash with a saline solution (bronchoalveolar lavage, LBA). Irrigation fluid is directed to certain subgroups that check for white blood cells classified as T lymphocytes.

Another possibility to ensure the diagnosis of sarcoidosis is to examine tissue samples taken at a bronchoscopy or mediastinoscopy.

Values ​​of the enzyme ACE (angiotensin converting enzyme) are shown to be elevated in a blood test in people with sarcoidosis. In the phases of greater pathological activity, the sedimentation rate of the globules increases; This value indicates inflammatory reactions.

In addition, a high-resolution computed tomography (CT) scan may help in the diagnosis of sarcoidosis. It can detect changes in the lymph nodes, which makes it possible to rule out other lung diseases.

Other tests such as determination of liver values, urinalysis, blood tests, or an electrocardiogram (ECG) are needed to assess the diagnosis of other organ involvement by sarcoidosis. In some patients, calcium levels in the blood increase (hypercalcemia).


How is sarcoidosis treated?

A treatment for sarcoidosis is not always necessary. Acute sarcoidosis cases are cured in many cases without the need for drug treatment, and without any complications. Before a mild sarcoidosis with a favorable prognosis can be expected to begin treatment and perform periodic reviews.

Appropriate treatment against acute relapses presenting discomfort and in the case of Lofgren’s syndrome consists of antipyretic, anti-inflammatory and analgesic drugs such as acetylsalicylic acid or ibuprofen.

Cortisone preparations are used against severe symptoms in stage II sarcoidosis and in cases of affection in the function of the affected organ. Cortisone treatment is usually given in the form of pills. However, in cases of sarcoidosis in the eyes or skin (cutaneous sarcoidosis), you can apply cortisone directly into the affected area in the form of drops or soap. If the lung is affected by sarcoidosis, it can go from an initial treatment with tablets to a spray.

If sarcoidosis is prolonged or corticosteroids are not enough, your doctor may prescribe immunosuppressive drugs such as azathioprine, methotrexate, or chloroquine.

Drug treatment for sarcoidosis lasts approximately three months. After this period, the dose is gradually reduced. During the treatment of sarcoidosis it is advisable to examine the affected organs continuously and check their functionality.


Process of the sarcoidosis

Sarcoidosis can progress acutely or chronically. The prognosis in both cases is favorable. However, in some cases the prognosis and possible complications depend on the organs involved.

Although acute sarcoidosis usually shows a complicated evolution, the prognosis is favorable. In 95% of cases, acute sarcoidosis is cured spontaneously without treatment within four to six weeks. In a few months, the patients heal completely. After a year, you can check on an X-ray that all changes produced in the lungs have been rebuilt.

Even cases of chronic sarcoidosis are usually cured by treatment with cortisone from a single application. If sarcoidosis becomes chronic and continues to reproduce, it is advisable to go to the doctor frequently and be treated. Approximately 5% of patients die from chronic sarcoidosis, especially if it has affected the brain, heart or lungs.

Complications

In the subsequent evolution of sarcoidosis several complications can appear. These depend on the organ that is affected by the nodular formations. Some examples are:

  • Pulmonary fibrosis
  • Renal insufficiency
  • Permanent paralysis
  • Visual Disorders

Sarcoidosis can not be prevented, as the cause of the disease is currently unknown. If you have relatives who have sarcoidosis, the risk of developing sarcoidosis is higher, even slightly higher compared to the rest of the population. In the case of monozygotic twins, it reaches 30 to 50%.

Related Posts
What is Dysthymia?
What causes to hepatomegaly?
What is Nickel Allergy ?
What is Cholera ?

Leave Your Comment

Your Comment*

Your Name*
Your Webpage