Sarcoidosis

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Sarcoidosis

Sarcoidosis is a systemic granulomatous disease of unknown origin, characterized by the formation of epithelioid tubercles in various tissues and organs: lungs, lymph nodes, skin, liver, spleen, bones, within the ear salivary glands. The frequency is 10-40 cases per 100,000 population (Europe). The predominant age is 20-40 years. The predominant gender is female. Pathomorphology A typical sarcoid granuloma is a dense structure consisting of aggregates of mononuclear phagocytes, surrounded by a rim of T-lymphocytes (helpers, inducers) and, to a lesser extent, B-lymphocytes. Giant multinucleated cells in the granuloma are often cambial subendothelial Langhans cells.

Clinical picture

    • Lung involvement (90%): shortness of breath, dry cough, dry wheezing, pleural effusion (1-5%)
    • Lymphadenopathy (75-90%) – ulceration is uncommon
    • The defeat of the intrathoracic lymph nodes – para-tracheal, radical
    • Peripheral lymphadenopathy involving cervical, axillary, inguinal lymph nodes
    • Skin lesions (25%): erythema nodosum, more often on the legs; maculopapular rash on the face, back, limbs; lupus pernio – dense purple-blue shiny nodules on the face, fingers, legs
    • Eye damage (25%): anterior and posterior uveitis, conjunctivitis, reti-

nit, retinal periphlebitis

    • Liver involvement (60-90%): cholestasis, portal hypertension (uncommon)
    • Fever is often associated with liver damage
    • Kidney damage (1-2%): nephrocalcinosis, nephrolithiasis
    • Nervous system involvement (less than 5%): facial paralysis, meningoencephalitis, peripheral neuropathy
    • Bone involvement (5%): cysts in the bones of the hands and feet
    • Joint damage: acute transient symmetrical polyarthritis of the knee and ankle joints, more often as part of Löfgren’s syndrome; chronic persistent polyarthritis with damage to the knee, ankle joints, less often the joints of the upper extremities
    • Heart damage: arrhythmias, atrioventricular block of various degrees, pericarditis, cor pulmonale on the background of pulmonary fibrosis (not often)
    • Endocrine pathology: diabetes insipidus, adrenal insufficiency
    • Mineral metabolism disorders: hypercalcemia, hypercalciuria against the background of an abnormally high level of 1,25-dihydroxyvitamin D3 produced by granuloma macrophages
    • Damage to the exocrine glands: within the ear glands (within 10% of all patients with sarcoidosis) – the glands are enlarged, compacted, xerostomia is possible; probably damage to the lacrimal glands (less often)
    • Löfgren’s syndrome: erythema nodosum, bilateral hilar lymphadenopathy, fever, polyarthritis
    • Heerfordt-Waldenström syndrome: anterior uveitis, facial paralysis, fever, lesions within the ear salivary glands
    • Asymptomatic form of the disease (found incidentally on chest X-ray) – 10-20% of patients with sarcoidosis.

Laboratory research

    • Leukopenia, lymphopenia, eosinophilia
    • ESR increase
    • Hypergammaglobulinemia
    • Hypercalcemia
    • An increase in the concentration of ACE in the blood serum and washing fluid (the sign is nonspecific, may occur with hyperthyroidism and diabetes mellitus)
    • An increase in the concentration of alkaline phosphatase in the blood serum in cholestasis. Special Studies
    • X-ray examination
    • Bilateral enlargement of lymph nodes – radical, paratracheal
    • Reticulonodular changes in the lung parenchyma
    • Infiltrative changes – rounded, cluster-like infiltrates
    • Pleural effusion (less than 5%)
    • Lung atelectasis (within 1%)
    • The stages of the disease are classified according to Scuding. Stage 0 is the norm.
    • Stage 1 – hilar lymphadenopathy
    • Stage 2 – hilar lymphadenopathy and parenchymal infiltrates
    • Stage 3 – only infiltrates in the lung parenchyma
    • Stage 4 – pulmonary fibrosis.
    • Examination of the washing fluid – the predominance of lymphocytes, especially T-helpers; similar changes are observed in pneumonia.
    • The pleural fluid is an exudate, the cellular composition is dominated by lymphocytes.
    • Biopsy of the lung, liver, skin, lymph nodes – sarcoid granulomas.
    • ATS – restrictive changes, the diffusion capacity of the lungs is reduced.
    • Scanning with 67Ga – accumulation of the isotope in the hilar lymph nodes, liver, spleen, within the ear glands.
    • The Kveim test is the development of a granulomatous reaction in the skin at the site of injection of an extract of the spleen or lymph node affected by sarcoidosis 4-6 weeks after the injection. The sensitivity of the test is 80%, the specificity is over 90%.
    • Ophthalmic research.

Differential Diagnosis

    • Tuberculosis
    • Fungal infection of the lungs
    • Granulomatous reaction to the introduction of a foreign body
    • Lymphoma
    • Metastatic lesion of the lymph nodes of the roots of the lungs
    • Beryllium.

Treatment:

General tactics. Due to the high frequency of spontaneous cure, the question of indications for treatment has not been resolved.

    • Indications for immunosuppressive therapy: severe dyspnea, fever, arrhythmias, liver failure, hypercalcemia, lesions of the nervous system or eyes
    • X-ray changes in the lungs are not considered an indication for the appointment of immunosuppressive therapy (does not prevent the development of pulmonary fibrosis).

Immunosuppressive Therapy

    • Glucocorticoids: prednisolone at a starting dose of 15-20 mg/day, the dose is gradually reduced to a maintenance dose of 5 mg/day. The duration of treatment is several months for Löfgren’s syndrome, several years for chronic sarcoidosis.
    • Cytostatic immunosuppressants in the absence of the effect of glucocorticoids or the presence of contraindications to their use (peptic ulcer, diabetes mellitus, severe arterial hypertension)
    • Methotrexate 10 mg/week
    • Mercaptopurine 4 mg/day
    • Plaquenil (hydroxychloroquine) 100-200 mg/day – for correction of skin lesions only
    • Vitamin E (tocopherol) 200 mg 3 r / day for 3-4 months (efficacy has not been proven).

Observation

    • When using immunosuppressants, it will be necessary to periodically conduct general blood and urine tests, liver function tests
    • In the treatment of glucocorticoids – determination of the concentration of blood plasma glucose, blood pressure, regular FEGDS
    • After the cessation of immunosuppressive treatment, the clinical and laboratory examination is repeated after 2-3 months, because. possible relapses
    • If immunosuppressive therapy has not been performed, X-ray control is carried out for 3-4 years. Complications
    • With lung involvement: respiratory failure, chronic cor pulmonale, atelectasis
    • With cardiovascular disease: congestive heart failure, sudden death
    • If the eyes are affected, blindness.

Course and forecast

    • In 80% of patients, the disease resolves spontaneously.
    • Erythema nodosum is an indicator of good prognosis
    • 10% of patients develop pulmonary fibrosis
    • Chronization – 10%.

Synonyms

    • bekadisease
    • Vecasarcoid
    • Besnier-Weck-Schaumann syndrome
    • Besnier-Boeck-Schaumann disease
    • schaumann syndrome
    • Granulomatosis benign
    • Lymphogranulomatosis benign
    • Reticuloendotheliosis epithelioid cell chronic
    • Schaumann benign lymphogranulomatosis See also Tuberculosis, Pleural effusion, Diffuse interstitial lung disease. D86 Sarcoidosis