Pulmonary sarcoidosis

Abstract

Sarkoidoza je sustavna granulomatozna upalna bolest još uvijek nerazjašnjene etiologije, a prvu je kliničku sliku bolesti opisao Sir Jonathan Hutchinson još u drugoj polovici 19. stoljeća. Smatra se da je bolest multifaktorijalno uzrokovana; u obzir dolaze razne hipoteze o okolišnim, infektivnim i genetskim uzrocima. Sarkoidoza pluća najčešća je manifestacija bolesti i nalazi se u 90-95% oboljelih od sarkoidoze. Incidencija bolesti se kreće od 10 do 20 oboljelih na 100000 stanovnika. Bolest se tipično javlja u dobi između 20. i 40. godine života, a češća je u pripadnika crne rase kod kojih ima i teži klinički tijek. Potrebno je napomenuti da postoji i drugi vrhunac pojavnosti bolesti u dobi od 50 godina i to češće u ženskog spola. Karakteristična histološka promjena u zahvaćenom tkivu su nekazeozni granulomi. Ove su kompaktne tvorbe građene od epiteloidnih stanica, divovskih multinuklearnih stanica i limfocita bez kazeozne nekroze. Svojim prisustvom one uništavaju normalnu strukturu tkiva pluća i dovode do oštećenja. Bolest u velikom broju slučajeva može biti asimptomatska, ali kod nekih pacijenata uzrokuje opće (slabost, vrućica, umor) i respiratorne simptome (kašalj, zaduha, bol u prsima) te progresivno zatajenje plućne funkcije. Dijagnoza se postavlja na temelju tri elementa koja trebaju biti zadovoljena. To su patohistološki dokaz nekazeoznih granuloma u plućima, kliničko-radiološke značajke plućne bolesti i odsustvo drugih bolesti koje se slično očituju. Liječenje ne zahtijevaju svi pacijenti, već oni s težom kliničkom slikom. Temelj inicijalne terapije su glukokortikoidi, a pacijenti čije stanje to zahtijeva, mogu se liječiti i drugim lijekovima kao što su metotreksat i drugi imunosupresivni lijekovi, te antagonisti TNF-α. U većine pacijenata bolest rezultira spontanom remisijom, ali kod nekih može dovesti i do uznapredovane plućne fibroze i zahtijevati transplantaciju pluća.Sarcoidosis is a systemic granulomatous inflammatory disease of yet unclear etiology and the first known description of its clinical features was published by Sir Jonathan Hutchinson in the second half of the 19th century. Etiology of sarcoidosis is believed to be multifactorial; consideration is given to various hypotheses about environmental, infectious and genetic causes. Pulmonary sarcoidosis is the most common manifestation of the disease and is found in up to 90-95% of sarcoidosis patients. The incidence of the disease ranges from 10 to 20 per 100,000 population. Disease typically occurs in people aged from 20 to 40 years old, and is more frequent among black people with a more severe clinical course. It should be noted that there is another peak in the occurence of the disease at 50 years of age and more often in females. Characteristic histopathological change is the formation of noncaseating granulomas in the affected tissue. Granuloma is a compact nodule that consists of epitheloid cells, giant multinuclear cells and lymphocytes and does not contain elements of caseous necrosis. In many cases, patient may be asymptomatic, but some patients experience general symptoms (e.g. weakness, fever, fatigue) as well as respiratory symptoms (e.g. cough, dyspnea, chest pain) and progressive respiratory failure. Three elements are important when diagnosing a patient with pulmonary sarcoidosis: histopathological evidence of noncaseating granuloma formation, clinicoradiological features of the pulmonary disease and exclusion of other diseases with similar presentation. Only patients with a more severe clinical course require treatment. Glucocorticoids are the basis for initial treatment of the disease, but patients who cannot tolerate glucocorticoids, can be treated with other drugs such as methotrexate and other immunosupressants as well as TNF-α antagonists. Most patients experience spontaneous remission, although some patients may develop advanced pulmonary fibrosis and require lung transplantation