Pulmonary Sarcoidosis - Symptoms

Clinical symptoms of pulmonary sarcoidosis and the degree of manifestation are quite diverse. It is characteristic that most patients can note a completely satisfactory general condition, despite mediastinal lymphadenopathy and fairly extensive lung damage.

M. M. Ilkovich (1998), A. G. Khomenko (1990), I. E. Stepanyan, L. V. Ozerova (1998) describe three variants of the onset of the disease: asymptomatic, gradual, acute.

Asymptomatic onset of sarcoidosis is observed in 10-15% (and according to some data in 40%) of patients and is characterized by the absence of clinical symptoms. Sarcoidosis is detected by chance, as a rule, during preventive fluorographic examination and chest X-ray.

Gradual onset of the disease - observed in approximately 50-60% of patients. In this case, patients complain of such symptoms of pulmonary sarcoidosis as: general weakness, increased fatigue, decreased performance, severe sweating, especially at night. Quite often there is a dry cough or with the separation of a small amount of mucous sputum. Sometimes patients note pain in the chest, mainly in the interscapular region. As the disease progresses, shortness of breath appears during physical exertion, even moderate.

When examining the patient, no characteristic manifestations of the disease are found. In the presence of dyspnea, slight cyanosis of the lips may be noted. Percussion of the lungs may reveal enlarged lung roots (for the technique of percussion of the lung roots, see the chapter "Pneumonia") if there is mediastinal lymphadenopathy. A clear pulmonary sound is determined over the remaining parts of the lungs during percussion. Auscultatory changes in the lungs are usually absent, but in some patients, harsh vesicular breathing and dry wheezing may be heard.

Acute onset of sarcoidosis (acute form) is observed in 10-20% of patients. The following main symptoms are characteristic of the acute form of sarcoidosis:

• short-term increase in body temperature (within 4-6 days);
• pain in the joints (mainly large ones, most often ankles) of a migratory nature;
• dyspnea;
• chest pain;
• dry cough (in 40-45% of patients);
• weight loss;
• enlargement of peripheral lymph nodes (in half of patients), and the lymph nodes are painless and not fused with the skin;
• mediastinal lymphadenopathy (usually bilateral);
• erythema nodosum (according to M. M. Ilkovich - in 66% of patients). Erythema nodosum is an allergic vasculitis. It is localized mainly in the area of the shins, thighs, extensor surface of the forearms, but can appear in any part of the body;
• Löfgren's syndrome - a symptom complex that includes mediastinal lymphadenopathy, increased body temperature, erythema nodosum, arthralgia, and increased ESR. Löfgren's syndrome occurs primarily in women under 30 years of age;
• Heerfordt-Waldenstrom syndrome - a symptom complex that includes mediastinal lymphadenopathy, fever, mumps, anterior uveitis, and facial nerve paresis;
• dry wheezing during auscultation of the lungs (due to damage to the bronchi by the sarcoidosis process). In 70-80% of cases, the acute form of sarcoidosis ends with a reversal of the symptoms of the disease, i.e. recovery occurs.

Subacute onset of sarcoidosis has basically the same signs as acute onset, but the symptoms of pulmonary sarcoidosis are less pronounced and the time of onset of symptoms is more extended in time.

And yet, the most characteristic for pulmonary sarcoidosis is the primary chronic course (in 80-90% of cases). This form can proceed asymptomatically for some time, hidden or manifest only by a mild cough. Over time, shortness of breath appears (with dissemination of the pulmonary process and bronchial damage), as well as extrapulmonary manifestations of sarcoidosis.

When auscultating the lungs, dry, scattered wheezing and harsh breathing are heard. However, with this course of the disease, half of the patients may experience a reversal of symptoms and almost complete recovery.

The most unfavorable prognostic form is the secondary-chronic form of sarcoidosis of the respiratory organs, which develops as a result of the transformation of the acute course of the disease. The secondary-chronic form of sarcoidosis is characterized by extensive symptoms - pulmonary and extrapulmonary manifestations, the development of respiratory failure and complications.

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Lymph node involvement in sarcoidosis

The most common lesion is the lesion of the intrathoracic nodes - mediastinal lymphadenopathy - 80-100% of cases. The hilar bronchopulmonary, tracheal, upper and lower tracheobronchial lymph nodes are predominantly enlarged. Less frequently, an increase in the anterior and posterior mediastinal lymph nodes is observed.

In patients with sarcoidosis, peripheral lymph nodes also enlarge (25% of cases) - cervical, supraclavicular, less often - axillary, elbow and inguinal. Enlarged lymph nodes are painless, not fused with each other or with underlying tissues, have a dense elastic consistency, never ulcerate, do not suppurate, do not disintegrate and do not form fistulas.

In rare cases, the lesion of the peripheral lymph nodes is accompanied by the lesion of the tonsils, hard palate, tongue - dense nodules with hyperemia on the periphery appear. The development of sarcoidosis gingivitis with multiple granulomas on the gums is possible.

Bronchopulmonary system involvement in sarcoidosis

The lungs are quite often involved in the pathological process in sarcoidosis (in 70-90% of cases). In the early stages of the disease, changes in the lungs begin with the alveoli - alveolitis develops, alveolar macrophages and lymphocytes accumulate in the lumen of the alveoli, and the interalveolar septa are infiltrated. Later, granulomas are formed in the lung parenchyma, and in the chronic stage, pronounced development of fibrous tissue is noted.

Clinically, the initial stages of lung damage may not manifest themselves in any way. As the pathological process progresses, cough (dry or with a slight release of mucous sputum), chest pain, and shortness of breath appear. Shortness of breath becomes especially pronounced with the development of fibrosis and emphysema of the lungs, accompanied by a significant weakening of vesicular breathing

The bronchi are also affected by sarcoidosis, sarcoid granulomas are located subepithelially. Bronchial involvement is manifested by cough with the separation of a small amount of sputum, scattered dry, less often fine-bubble rales.

Pleural lesions are manifested by the clinical picture of dry or exudative pleurisy (see "Pleurisy"). Often pleurisy is interlobar, parietal and is detected only by X-ray examination. In many patients, pleurisy does not manifest itself clinically and only by X-ray examination of the lungs can local thickening of the pleura (pleural layers), pleural adhesions, interlobar cords be detected - a consequence of past pleurisy. There are usually many lymphocytes in the pleural effusion.

Digestive system damage in sarcoidosis

Liver involvement in the pathological process in sarcoidosis is often observed (according to various data, in 50-90% of patients). In this case, patients are bothered by a feeling of heaviness and fullness in the right hypochondrium, dryness and bitterness in the mouth. Jaundice is usually absent. Palpation of the abdomen reveals an enlarged liver, its consistency may be dense, the surface is smooth. The functional capacity of the liver is usually not impaired. The diagnosis is confirmed by a puncture biopsy of the liver.

Damage to other organs of the digestive system is considered a very rare manifestation of sarcoidosis. There are indications in the literature about the possibility of damage to the stomach, duodenum, ileocecal part of the small intestine, sigmoid colon. The clinical symptoms of damage to these organs do not have any specific signs and it is possible to confidently recognize sarcoidosis of these parts of the digestive system only on the basis of a comprehensive examination and histological examination of biopsy specimens.

A typical manifestation of sarcoidosis is damage to the parotid gland, which is expressed in its enlargement and pain.

Spleen damage in sarcoidosis

Involvement of the spleen in the pathological process in sarcoidosis is observed quite often (in 50-70% of patients). However, significant enlargement of the spleen is not usually observed. Often, an enlarged spleen can be detected by ultrasound examination, sometimes the spleen is palpated. A significant enlargement of the spleen is accompanied by leukopenia, thrombocytopenia, and hemolytic anemia.

Heart damage in sarcoidosis

The frequency of heart damage in sarcoidosis varies from 8 to 60% according to various authors. Heart damage is observed in systemic sarcoidosis. All membranes of the heart may be involved in the pathological process, but most often the myocardium - sarcoid infiltration, granulomatosis, and then fibrous changes are observed. The process may be focal and diffuse. Focal changes may manifest themselves as electrocardiographic signs of transmural myocardial infarction with subsequent formation of a left ventricular aneurysm. Diffuse granulomatosis leads to the development of severe cardiomyopathy with dilation of the heart cavities, which is confirmed by ultrasound examination. If sarcoid granulomas are localized mainly in the papillary muscles, mitral valve insufficiency develops.

Quite often, an ultrasound of the heart reveals effusion in the pericardial cavity.

In most patients with sarcoidosis, heart damage during life is not recognized, as it is usually taken as a manifestation of some other disease.

The main symptoms of heart damage in sarcoidosis are:

• shortness of breath and pain in the heart area during moderate physical exertion;
• sensation of heartbeats and interruptions in the heart area;
• frequent, arrhythmic pulse, decreased pulse volume;
• expansion of the border of the heart to the left;
• muffled heart sounds, often arrhythmia, most often extrasystole, systolic murmur in the region of the apex of the heart;
• the appearance of acrocyanosis, swelling of the legs, enlargement and pain of the liver with the development of circulatory failure (with severe diffuse myocardial damage);
• ECG changes in the form of a decrease in the T wave in many leads, various arrhythmias, most often extrasystole, cases of atrial fibrillation and flutter, various degrees of atrioventricular conduction disturbance, bundle branch block of the His bundle are described; in some cases, ECG signs of myocardial infarction are detected.

To diagnose cardiac damage in sarcoidosis, ECG, echocardiography, cardiac scintigraphy with radioactive gallium or thallium, and in rare cases, even intravital ecdomyocardial biopsy are used. Intravital myocardial biopsy allows for the detection of epithelioid cell granulomas. Cases of detection of extensive scar areas in the myocardium during autopsy in sarcoidosis with cardiac damage have been described.

Heart damage can be fatal (severe heart rhythm disturbances, asystole, circulatory failure).

M. M. Ilkovich (1998) reports on individual observations of occlusion of the femoral artery, superior vena cava, pulmonary artery, as well as the formation of an aortic aneurysm.

Kidney damage in sarcoidosis

Involvement of the kidneys in the pathological process in renal sarcoidosis is a rare situation. Only isolated cases of sarcoid glomerulonephritis have been described. As noted earlier, sarcoidosis is characterized by hypercalcemia, which is accompanied by calciuria and the development of nephrocalcinosis - the deposition of calcium crystals in the renal parenchyma. Nephrocalcinosis may be accompanied by intense proteinuria, a decrease in the renal tubular reabsorption function, which is manifested by a decrease in the relative density of urine. However, nephrocalcinosis rarely develops.

Bone marrow changes in sarcoidosis

This pathology in sarcoidosis has been studied insufficiently. There are indications that bone marrow lesions in sarcoidosis are observed in approximately 20% of cases. The involvement of bone marrow in the pathological process in sarcoidosis is reflected in changes in peripheral blood - anemia, leukopenia, thrombocytopenia.

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Changes in the musculoskeletal system in sarcoidosis

Bone lesions are observed in approximately 5% of patients with sarcoidosis. Clinically, this is manifested by mild bone pain, very often there are no clinical symptoms at all. Much more often, bone lesions are detected by X-ray in the form of multiple foci of bone rarefaction, mainly in the phalanges of the hands and feet, less often - in the bones of the skull, vertebrae, long tubular bones.

Joint damage is observed in 20-50% of patients. Large joints are mainly involved in the pathological process (arthralgia, aseptic arthritis). Joint deformation develops extremely rarely. When such a sign appears, rheumatoid arthritis should be ruled out first.

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Skeletal muscle involvement in sarcoidosis

Muscle involvement in the pathological process is rare and manifests itself mainly in pain. There are usually no objective changes in skeletal muscles or significant reduction in muscle tone and strength. Severe myopathy, resembling polymyositis in its clinical course, is very rare.

Endocrine system damage in sarcoidosis

There are usually no significant endocrine system disorders in sarcoidosis. Thyroid enlargement with hyperthyroidism symptoms, decreased sexual function in men, and menstrual irregularities in women have been described. Adrenal cortex insufficiency is extremely rare. There is an opinion that pregnancy can lead to a reduction in the symptoms of pulmonary sarcoidosis and even recovery. However, after childbirth, the clinical picture of sarcoidosis may recur.

Nervous system damage in sarcoidosis

The most common is peripheral neuropathy, which manifests itself as decreased sensitivity in the feet and shins, decreased tendon reflexes, a feeling of paresthesia, and decreased muscle strength. Mononeuritis of individual nerves may also occur.

A rare but severe complication of sarcoidosis is damage to the central nervous system. Sarcoidosis meningitis is observed, manifested by headaches, rigidity of the muscles of the back of the head, a positive Kernig's sign. The diagnosis of meningitis is confirmed by a study of the cerebrospinal fluid - an increase in the content of protein, glucose, and lymphocytes is characteristic. It should be remembered that in many patients, sarcoidosis meningitis almost does not give clinical manifestations and diagnosis is possible only with the help of an analysis of the cerebrospinal fluid.

In some cases, spinal cord damage is observed with the development of paresis of the motor muscles. Damage to the optic nerves with decreased visual acuity and limitation of the visual fields is also described.

Skin lesions in sarcoidosis

Skin changes in sarcoidosis are observed in 25-30% of patients. Acute sarcoidosis is characterized by the development of erythema nodosum. It is an allergic vasculitis, localized mainly in the shins, less often - thighs, extensor surfaces of the forearms. Erythema nodosum is characterized by painful, reddish, never ulcerating nodes of varying sizes. They occur in the subcutaneous tissue and involve the skin. Erythema nodosum is characterized by a gradual change in skin color over the nodes - from red or red-violet to greenish, then yellowish. Erythema nodosum disappears spontaneously after 2-4 weeks. For a long time, erythema nodosum was considered a manifestation of tuberculosis. It is now considered a non-specific reaction, observed most often in sarcoidosis, as well as in tuberculosis, rheumatism, drug allergies, streptococcal infections, and sometimes in malignant tumors.

In addition to erythema nodosum, true sarcoidosis of the skin may also be observed - granulomatous sarcoidosis of the skin. A characteristic sign is small- or large-focal erythematous plaques, sometimes these are hyperpigmented papules. Telangiectasias may be present on the surface of the plaques. The most common localization of sarcoidosis lesions is the skin of the dorsal surfaces of the hands, feet, face and the area of old scars. In the active phase of sarcoidosis, skin manifestations are more pronounced and extensive, the lesions bulge above the surface of the skin.

Very rarely, sarcoidosis can cause dense, painless, spherical nodes of 1 to 3 cm in diameter to appear in the subcutaneous tissue - Darier-Rousseau sarcoidosis. Unlike erythema nodosum, the appearance of nodes is not accompanied by changes in skin color, and the nodes are also painless. Histological examination of the nodes is characterized by changes typical of sarcoidosis.

Eye damage in sarcoidosis

Eye damage in sarcoidosis is observed in 1/3 of all patients and is manifested by anterior and posterior uveitis (the most common type of pathology), conjunctivitis, corneal opacity, development of cataracts, changes in the iris, development of glaucoma, lacrimation, photophobia, and decreased visual acuity. Sometimes eye damage produces minor symptoms of pulmonary sarcoidosis. All patients with sarcoidosis must undergo an ophthalmological examination.