Sarcoidosis – current view on pathogenesis, diagnosis and treatment

Česká verzia

Authors: Kolek V.; Žurková M.; Lošťáková V.
Authors place of work: Klinika plicních nemocí a tuberkulózy LF OU a FN Olomouc
Published in the journal: Kardiol Rev Int Med 2019, 21(3): 145-150

Summary

The papers deals with the current opinions on etiopathogenesis of sarcoidosis, presents modern diagnostic procedures and recommended treatment. The etiology of the disease is still unclear, characterized by the formation of non-caseating granulomas, consisting mainly of T lymphocytes and variously modified macrophages. The importance of Th17+ cells that accumulate at the site of inflammation is emphasized. Involvement of an unknown noxa in a predisposed individual is expected. The incidence of the disease is increasing, as well as the reported mortality. Clinical manifestations may vary greatly; however, about a third of cases are asymptomatic. The initiation process begins in the lungs where alveolitis occurs; the lungs are affected in 95% of cases. The disease is systemic in nature and may affect almost all organs and tissues. Manifestations in the eyes, central nervous system and heart are usually severe. Diagnosis is based on a set of clinical manifestations, radiological changes (high-resolution computed tomography), functional examination, and identification of granuloma. Biopsy is usually performed bronchoscopically. Bronchoalveolar lavage and serum biomarker testing may contribute to the diagnosis. Spontaneous remission occurs in up to half of cases. Administration of corticosteroids usually leads to symptom resolution; however, there are persistent and progressive forms of the disease that threaten patients’ lives. In cases of refractory disease, immunosuppressive agents (methotrexate, azathioprine, leflunomide, and mycophenolate mofetil) may be administred in second line therapy. Furthermore, TNFα inhibitors (infliximab, adalimumab) may be administered in third line therapy, if needed. Improvement of organ function and quality of life should be weighed against the possible side effects when considering the therapy indication.

Keywords:

etiopathogenesis – diagnostics – Sarcoidosis

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