Sarcoidosis – lung disease

Sarcoidosis – lung disease

Systemic lung disease, characterized by the formation of granules in the lung tissue, is accompanied by cough, chest pain, weakness, fever.
Sarcoidosis is a more frequent disease than is commonly thought. The incidence of sarcoidosis in the world ranges from 11 to 640 per 100,000 people. The disease develops at any age, even after 70 years, but the peak of sarcoidosis is 40 years. It was believed that women suffer from sarcoidosis more often than men. However, apparently, it is not so: women simply have more pronounced symptoms of the disease, which means they are more likely to see doctors.

The cause of sarcoidosis is unknown, so there is still no exhaustive definition of the disease. The main finding is sarcoid granulomas (epithelioid cell granulomas without caseosal necrosis).

Complaints

Sarcoidosis - lung disease

In more than 90% of cases, the lungs or intra-thoracic lymph nodes are affected, but there are no complaints, at least not at an early stage. Even with extensive lung damage (chest X-ray) there may be no breathlessness or coughing. Dyspnea and other complaints usually occur at an advanced stage of the disease.

The first manifestation of sarcoidosis may be eye damage. Iridocyclitis, chorioiditis, conjunctivitis and lacrimal gland affection with xerophthalmia (dry eyes) are noted in 25% of cases. Approximately 20% of patients have the first sarcoidosis: plaques from orange-pink to brown color. Approximately 10% of patients suffer from the nervous system. Sarcoidosis may be suspected of impaired pituitary function or hypercalcemia (increased calcium levels). In addition, manifestations of sarcoidosis are hepatosplenomegaly (increase in the liver and spleen), bone damage (more often phalanges of the fingers) and symmetrical lesions of the joints. Significant pathology of the heart is rare, but in some cases there are violations of heart rhythm, cardiomyopathy. In 10% of patients, especially those with extensive affection of lymph nodes, there is a fever.

Stages

The stages of sarcoidosis are determined from the data of chest X-ray.
Stage I: bilateral increase in bronchopulmonary lymph nodes.
Stage II: Bilateral enlargement of bronchopulmonary lymph nodes and diffuse lesions of the pulmonary parenchyma, reticular remodeling of the lung pattern, but sometimes multiple focal or biliary shadows.
Stage III: Affection of the pulmonary parenchyma without enlargement of bronchopulmonary lymph nodes.
The probability of spontaneous remission (self-recovery) at stages I, II and III is 80, 50 and 30% respectively.

Diagnostics

Sarcoidosis - lung disease

In 90% of patients the disease is limited to lesions of the intra-thoracic lymph nodes and lungs and often runs asymptomatically. In such cases, radiological changes may be an accidental finding, for example, during a prophylactic examination or examination for another disease. As already mentioned, the first manifestation of sarcoidosis may be lesion of other organs. In any case, before making a diagnosis, the doctor must obtain histological confirmation of sarcoidosis.

Treatment

Despite the fact that glucocorticoids have been used for over 45 years, there is still no consensus on the advisability of their treatment for lung sarcoidosis. Practice shows that glucocorticoid treatment is justified in all patients with stages II and III, if within 6-12 months there are no signs of spontaneous remission or there are signs of deterioration (according to radiography and examination of respiratory function). As a rule, taking 40 mg of prednisolone a day is sufficient.
Although some specialists recommend starting treatment only when there are complaints, studies have shown that shortness of breath appears in the irreversible phase of the disease. Therefore, it is better to prescribe glucocorticoids before complaints occur to prevent the development of irreversible changes.
Most specialists adhere to the following indications for prescription of glucocorticoids: 1) uveitis (begin with local treatment), 2) hypercalcemia, 3) myocardial damage (especially cardiomyopathy), 4) neurological disorders. If glucocorticoids are contraindicated for any reason, the following drugs are recommended: methotrexate, chloroquine, azathioprine and oxyphenbutazone.

Observation

Surveillance of sarcoidosis patients necessarily includes regular chest X-rays and examination of external respiration function (spirometry). The frequency of examination depends on the course of the disease, as well as the preferences of the attending physician. The course of sarcoidosis can be assessed by the activity of angiotensin-converting enzyme in the blood. The frequency of relapse after treatment of patients with stages II and III is about 25%. Therefore, at the end of treatment, patients should be monitored for several years. The probability of relapse is significantly reduced, if for more than a year the condition remains stable and there are no signs of sarcoidosis activity.