SINDROM PLUĆNE FIBROZE UDRUŽENE S EMFIZEMOM

Marija Alilović, Tatjana Peroš-Golubičić, Jasna Tekavec Trkanjec, Ana Hećimović, Silvana Smojver-Ježek

Sindrom plućne fibroze udružene s emfizemom (engl. combined pulmonary fibrosis and emphysema – CPFE), novi je pojam čije je obilježje fibroza u donjim plućnim režnjevima uz istodobni emfizem u gornjim plućnim režnjevima. Bolesnici s CPFE-om imaju neočekivano duže vrijeme dobro održane vrijednosti testova plućne ventilacije, ali izrazito snižen difuzijski kapacitet pluća za ugljični monoksid i znatnu plućnu arterijsku hipertenziju. Sve objavljene studije o CPFE-u navode predominaciju bolesti kod starijih muškaraca. Uzroci nastanka bolesti nisu još poznati. Pušenje uz gensku predispoziciju smatra se glavnim čimbenikom nastanka bolesti. Smanjeno preživljenje povezano je sa stupnjem plućne arterijske hipertenzije, a mortalitet je veći nego u bolesnika s izoliranom plućnom fibrozom ili emfizemom. U radu su prikazane osobine 12 bolesnika sa sindromom plućne fibroze udružene s emfizemom. Svi bolesnici bili su muškarci, prosječne životne dobi 68 godina. U određenom razdoblju života svi su bili pušači, a većina je i profesionalno bila izložena onečišćenju zraka. Otežano disanje u naporu bilo je prisutno kod svih bolesnika koji su imali uredne plućne funkcijske testove, no znatno je bio snižen difuzijski kapacitet za ugljični monoksid (prosječno 39%). Plućna arterijska hipertenzija (PAH) prosječno je iznosila 56 mmHg (raspon od 25 do 75 mmHg) i bila je prisutna kod 75% bolesnika. Četiri su bolesnika umrla u razdoblju od 4 mjeseca, tri bolesnika imala su PAH viši od 70 mmHg. Četvrti bolesnik umro je od karcinoma pluća.

CPFE – combined pulmonary fibrosis and emphysema is a new term for a syndrome whose main characteristic is fibrosis in lower pulmonary lobes with simultaneous emphysema in upper pulmonary lobes. CPFE patients have well preserved pulmonary test values for unexpectedly long period, but extremely lowered carbon monoxide diffusion capacity and significant arterial hypertension. All CPFE studies indicate that CPFE occurs predominately in older male population. Smoking is considered main cause in developing CPFE. Reduced survival rate is linked with arterial hypertension extent, and mortality rate is greater than that for patients with isolated pulmonary fibrosis or emphysema. This study is focused on characteristics of twelve CPFE patients. This paper describes cases of 12 patients with the syndrome of pulmonary fibrosis associated with emphysema. All patients were male, mean age of 68 years. At the certain period of life they all were smokers, but most of them were also exposed to air pollution due to their profession. Shortness of breath on exertion was present in all patients. All patients had neat pulmonary function tests with significantly reduced diffusing capacity for carbon monoxide (average 39%). Pulmonary arterial hypertension (PAH) averaged 56 mmHg (range 25–75 mmHg) was present in 75% of patients. Four patients died during the period of four months, of which three patients had PAH greater than 70 mmHg. The fourth patient died of lung cancer.