Difuzne bolesti plućnog parenhima

Diffuse parenchymal lung disease (DPLD) is a group of diseases that have the common characteristic of involving the pulmonary parenchyma diffusely. The pulmonary parenchyma consists of all structures distal to the terminal bronchiole. DPLD are numerous, there are some 150 different entities that qualify for the group. Most of these diseases are diagnosed according to common algorithm, but the etiology, prognosis and therapy are quite different. Lung transplantation is the ultimate therapeutic option for some of these diseases, most frequently it is the idiopathic pulmonary fibrosis (IPF).Difuzne bolesti plućnog parenhima su grupa bolesti kojima je zajednička osobina da difuzno zahvaćaju plućni parenhim. Plućni se parenhim sastoji od svih struktura koje se prostiru distalno od terminalnih bronhiola. Ove su bolesti brojne, pa tako čak oko 150 različitih entiteta zadovoljava kriterije uključivanja u ovu skupinu bolesti. Dijagnoza većine ovih bolesti postavlja se prema zajedničkom dijagnostičkom algoritmu, ali su etiologija, prognoza i terapija vrlo različite. Transplantacija pluća krajnja je terapijska opcija za neke od ovih bolesti, a najčešće se zahvat se vrši u bolesnika s idiopatskom plućnom fibrozom

Lung Lavage Cell Profiles in Diffuse Lung Disease

The standard armamentarium of tests that are used by pulmonologist are laboratory tests, pulmonary function tests, different radiological techniques (conventional chest-X rays, HRCT scans, etc) and pathohistological analyses of biopsies. The minimally invasive bronchoalveolar lavage (BAL) procedure, in addition to methods earlier mentioned, is an important diagnostic instrument that can facilitate the diagnosis of various diffuse lung diseases (DLD). BAL fluid white blood cell profiles are analyzed, malignant cells looked for, and in certain circumstances particular stains are performed to detect yet other cell types. Additionally, BAL can play a very important role in the diagnosis of respiratory tract infections. All these analyses are usually readily performed in a moderately equipped cytological laboratory

COMBINED PULMONARY FIBROSIS AND EMPHYSEMA SYNDROME

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

COMBINED PULMONARY FIBROSIS AND EMPHYSEMA SYNDROME

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

Prevalence of Hospitalized Patients with Sarcoidosis in Croatia

The aim of this study was to investigate the prevalence of hospitalized patients of sarcoidosis in the Republic of Croatia, its distribution in relation to sex and age as well as its geographical distribution. The data on sarcoidosis patients hospitalized in Croatia in the last six years, from 1997 to 2002, were analyzed retrospectively. The prevalence of sarcoidosis patients hospitalised in the Republic of Croatia is 4.1/100,000. The prevalence among women is 4.7 and among men 3.5 per 100,000 persons, with a ratio of 1.4:1. The disease more frequently occurs in the regions with a continental climate than in the Mediterranean zone. The ratio of sarcoidosis patients in the continental zone to the Mediterranean zone is 1.5:1. It occurs predominantly among the adults. Over the investigated period, in our country we have not registered any case of sarcoidosis among children. It occurs more frequently at a younger age and therefore 44.5% of the patients with sarcoidosis were between 20 and 39 years of age, 40.1% were between 40 and 59 years of age and 15.3% were more than 60 years old

Učinak dušikova dioksida na broj posjeta hitnim službama

The paper describes an investigation of shortterm effects of NO2 concentrations in the air on the number of emergency room visits caused by respiratory impairments, particularly asthma in adults and children. The data were collected from clinical emergency room records from July 1, 1994 to December 31, 1995. Concurrently, readings of average weekly concentrations of NO2 (µg/m3), average weekly temperature (°C), air pressure (kPa), and relative humidity (%) were registered. Trend and seasonality effects were estimated by the locally weighted regression (LOESS). After standardising for trend, seasonality, and meteorological conditions, the number of cases was regressed on weekly NO2 concentration, including the current and the previous week concentrations and autocorrelated residual. The weekly average NO2 concentrations were significantly associated with the number of emergency asthma cases for children and adults and with the total number of emergency respiratory cases in children, but not in adults. The results suggest that health effects of NO2 on risk groups can be detected even in moderately polluted environments. The effect is more pronounced in children.Analizirani su kratkoročni učinci prosječnih tjednih koncentracija NO2 u zraku na broj posjeta hitnim službama zbog pogoršanja stanja bolesti respiratornog sustava, posebno astme u odraslih i djece. Podaci su prikupljeni iz knjiga hitnih službi u razdoblju od 1. srpnja 1994. do 31. prosinca 1995. Bilježene su prosječne tjedne koncentracije NO2 (µg/m3), prosječne tjedne temperature (°C), tlak zraka (kPa) i relativna vlaga (%). Trend i sezonalnost procijenjeni su lokalnom ponderiranom regresijom (LOESS). Nakon isključivanja učinka trenda, sezonalnosti i meteoroloških uvjeta, broj hitnih slučajeva modeliran je u ovisnosti o tjednim koncentracijama NO2, uključujući koncentracije za tekući i prethodni tjedan te autokorelaciju reziduala. Prosječne tjedne koncentracije NO2 statistički su značajno povezane s brojem hitnih slučajeva astme u djece i odraslih s ukupnim brojem hitnih respiratornih slučajeva u djece, ali ne i u odraslih. Rezultati pokazuju da i pri umjereno visokim koncentracijama NO2 možemo opaziti učinak onečišćenja na zdravlje, pogotovo u osoba iz skupina povišenog rizika. Taj utjecaj na zdravlje osobito je naglašen u djece

Korelacija scintigrafije pluća Ga-67 s RTG pluća i testovima plućne funkcije u oboljelih od sarkoidoze

In order to assess the usefulness of Ga-67 scintigraphy in the evaluation of sarcoidosis activity, 44 patients including 14, 20 and 10 patients with radiologic stage I, II and III, respectively, were examined. Control group consisted of 22 subjects with healthy lungs who underwent Ga-67 scintigraphy for extrathoracic disease. An objective method of quantitative computer analysis was used on scintigram interpretation. Results were expressed as mean impulse count per pixel of the given area in the left and right hilar region, left and right lung, and in the area of focal accumulation if present. In sarcoidosis patients, the mean impulse count was statistically significantly higher in all regions than in the control group (p<0.0001 for left hilum, right hilum and right lung; and p<0.001 for left lung). The mean impulse count showed correlation with pathologic finding. Pathologic accumulation of Ga-67 in the hilar region was evident in 37 (84.1%) patients, and in the region of pulmonary parenchyma in 31 (70.5%) patients, and showed statistically significant correlation with radiologic staging of sarcoidosis (p<0.01). However, scintigraphy supplemented radiologic staging by providing additional information on the disease extent. In 35.7% of patients with X-ray stage I sarcoidosis, scintigraphy revealed pathologic accumulation of Ga-67 also in pulmonary parenchyma, whereas in as many as 70% of patients with X-ray stage III sarcoidosis the pathologic accumulation of Ga-67pointed to the disease activity in the lymph nodes of the hilum. Only three (6.7%) patients were free from any form of pathologic Ga-67 accumulation. The patients with pathologic Ga-67 accumulation in the lung parenchyma (n=31) had a more severe form of the disease with lower values of pulmonary function tests, i.e. forced vital capacity (FVC), forced expiratory volume in first second (FEV 1), diffusion capacity for carbon monoxide (DLCO) and arterial blood partial oxygen pressure (pO 2). Forced vital capacity as an indicator of ventilation restrictive impairments was statistically significantly lower in the group of patients with pathologic accumulation of Ga-67 in pulmonary parenchyma as compared with the group of patients without it (p<0.01). The decrease in diffusion capacity correlated statistically significantly with the increase in the pathologic accumulation of Ga-67 in pulmonary parenchyma (p<0.01). Thus, lung Ga-67scintigraphy was found to be a highly sensitive marker of the extent and activity of sarcoidosis, however, due to the low specificity of the method its use in good clinical practice should be reserved for diagnostically and therapeutically vague cases, with due consideration of the irradiation dose and cost.Radi procjene značenja scintigrafije pluća s Ga-67 u oboljelih od sarkoidoze ispitana su 44 bolesnika, i to 14 u I., 20 u II. i 10 u III. radiološkom stadiju bolesti. Kontrolnu skupinu činile su 22 osobe sa zdravim plućima u kojih je scintigrafija s Ga-67 rađena zbog ekstratorakalne bolesti. Pri tumačenju scintigrama rabljena je objektivna metoda kvantitativne kompjutorske analize gdje se rezultat izražava kao prosječan broj impulsa po kvadrantu zadane regije u području lijevog i desnog hilusa, lijevog i desnog pluća, te u području žarišnog nakupljanja ako je ono izraženo. U oboljelih od sarkoidoze prosječan broj impulsa za svaku regiju bio je statistički značajno viši od onoga u kontrolnoj skupini (p<0,0001 za lijevi hilus, desni hilus i desno pluće, te p<0,001 za lijevo pluće). Prosječan broj impulsa pratio je radiološki nalaz. Patološko nakupljanje Ga-67 u području hilusa bilo je očito u 37(84,1%) bolesnika, a u području plućnog parenhima u 31 (70,5%) bolesnika, uza statistički značajnu korelaciju s radiološkim stadijem sarkoidoze (p<0,01). Međutim, scintigrafija je dopunila radiološko određivanje stadija bolesti pruživši dodatne informacije o proširenosti bolesti. Tako je kod 35,7% bolesnika u I. rtg stadiju bolesti patološko nakupljanje Ga-67 bilo očito i u plućnom parenhimu, dok je u čak 70% bolesnika s III. rtg stadijem patološko nakupljanje Ga-67 upućivalo na aktivnost procesa i u limfnim čvorovima hilusa. Samo troje (6,7%) bolesnika nije imalo patološko nakupljanje Ga-67 u bilo kojem obliku. Bolesnici s patološkim nakupljanjem Ga-67 u plućnom parenhimu (n=31) imali su teži oblik bolesti s nižim vrijednostima testova plućnih funkcija, tj. forsiranog vitalnog kapaciteta, forsiranog ekspiracijskog volumena u prvoj sekundi, difuzijskog kapaciteta za ugljični monoksid i parcijalnog tlaka kisika u arterijskoj krvi. Forsirani vitalni kapacitet kao pokazatelj restriktivnih smetnja ventilacije bio je statistički značajno niži u skupini bolesnika s patološkim nakupljanjem Ga-67 u plućnom parenhimu u usporedbi sa skupinom bez patološkog nakupljanja (p<0,01). Pad difuzijskog kapaciteta je statistički značajno korelirao s porastom patološkog nakupljanja Ga-67 u plućnom parenhimu (p<0,01). Dakle, scintigrafija pluća s Ga-67 vrlo je osjetljiv biljeg proširenosti i aktivnosti kod sarkoidoze, međutim, ima nisku specifičnost, pa ovu metodu u dobroj kliničkoj praksi treba rabiti u dijagnostički i terapijski dvojbenim slučajevima, vodeći pritom računa o dozi ozračivanja i cijeni

Korelacija scintigrafije pluća Ga-67 s RTG pluća i testovima plućne funkcije u oboljelih od sarkoidoze

In order to assess the usefulness of Ga-67 scintigraphy in the evaluation of sarcoidosis activity, 44 patients including 14, 20 and 10 patients with radiologic stage I, II and III, respectively, were examined. Control group consisted of 22 subjects with healthy lungs who underwent Ga-67 scintigraphy for extrathoracic disease. An objective method of quantitative computer analysis was used on scintigram interpretation. Results were expressed as mean impulse count per pixel of the given area in the left and right hilar region, left and right lung, and in the area of focal accumulation if present. In sarcoidosis patients, the mean impulse count was statistically significantly higher in all regions than in the control group (p<0.0001 for left hilum, right hilum and right lung; and p<0.001 for left lung). The mean impulse count showed correlation with pathologic finding. Pathologic accumulation of Ga-67 in the hilar region was evident in 37 (84.1%) patients, and in the region of pulmonary parenchyma in 31 (70.5%) patients, and showed statistically significant correlation with radiologic staging of sarcoidosis (p<0.01). However, scintigraphy supplemented radiologic staging by providing additional information on the disease extent. In 35.7% of patients with X-ray stage I sarcoidosis, scintigraphy revealed pathologic accumulation of Ga-67 also in pulmonary parenchyma, whereas in as many as 70% of patients with X-ray stage III sarcoidosis the pathologic accumulation of Ga-67pointed to the disease activity in the lymph nodes of the hilum. Only three (6.7%) patients were free from any form of pathologic Ga-67 accumulation. The patients with pathologic Ga-67 accumulation in the lung parenchyma (n=31) had a more severe form of the disease with lower values of pulmonary function tests, i.e. forced vital capacity (FVC), forced expiratory volume in first second (FEV 1), diffusion capacity for carbon monoxide (DLCO) and arterial blood partial oxygen pressure (pO 2). Forced vital capacity as an indicator of ventilation restrictive impairments was statistically significantly lower in the group of patients with pathologic accumulation of Ga-67 in pulmonary parenchyma as compared with the group of patients without it (p<0.01). The decrease in diffusion capacity correlated statistically significantly with the increase in the pathologic accumulation of Ga-67 in pulmonary parenchyma (p<0.01). Thus, lung Ga-67scintigraphy was found to be a highly sensitive marker of the extent and activity of sarcoidosis, however, due to the low specificity of the method its use in good clinical practice should be reserved for diagnostically and therapeutically vague cases, with due consideration of the irradiation dose and cost.Radi procjene značenja scintigrafije pluća s Ga-67 u oboljelih od sarkoidoze ispitana su 44 bolesnika, i to 14 u I., 20 u II. i 10 u III. radiološkom stadiju bolesti. Kontrolnu skupinu činile su 22 osobe sa zdravim plućima u kojih je scintigrafija s Ga-67 rađena zbog ekstratorakalne bolesti. Pri tumačenju scintigrama rabljena je objektivna metoda kvantitativne kompjutorske analize gdje se rezultat izražava kao prosječan broj impulsa po kvadrantu zadane regije u području lijevog i desnog hilusa, lijevog i desnog pluća, te u području žarišnog nakupljanja ako je ono izraženo. U oboljelih od sarkoidoze prosječan broj impulsa za svaku regiju bio je statistički značajno viši od onoga u kontrolnoj skupini (p<0,0001 za lijevi hilus, desni hilus i desno pluće, te p<0,001 za lijevo pluće). Prosječan broj impulsa pratio je radiološki nalaz. Patološko nakupljanje Ga-67 u području hilusa bilo je očito u 37(84,1%) bolesnika, a u području plućnog parenhima u 31 (70,5%) bolesnika, uza statistički značajnu korelaciju s radiološkim stadijem sarkoidoze (p<0,01). Međutim, scintigrafija je dopunila radiološko određivanje stadija bolesti pruživši dodatne informacije o proširenosti bolesti. Tako je kod 35,7% bolesnika u I. rtg stadiju bolesti patološko nakupljanje Ga-67 bilo očito i u plućnom parenhimu, dok je u čak 70% bolesnika s III. rtg stadijem patološko nakupljanje Ga-67 upućivalo na aktivnost procesa i u limfnim čvorovima hilusa. Samo troje (6,7%) bolesnika nije imalo patološko nakupljanje Ga-67 u bilo kojem obliku. Bolesnici s patološkim nakupljanjem Ga-67 u plućnom parenhimu (n=31) imali su teži oblik bolesti s nižim vrijednostima testova plućnih funkcija, tj. forsiranog vitalnog kapaciteta, forsiranog ekspiracijskog volumena u prvoj sekundi, difuzijskog kapaciteta za ugljični monoksid i parcijalnog tlaka kisika u arterijskoj krvi. Forsirani vitalni kapacitet kao pokazatelj restriktivnih smetnja ventilacije bio je statistički značajno niži u skupini bolesnika s patološkim nakupljanjem Ga-67 u plućnom parenhimu u usporedbi sa skupinom bez patološkog nakupljanja (p<0,01). Pad difuzijskog kapaciteta je statistički značajno korelirao s porastom patološkog nakupljanja Ga-67 u plućnom parenhimu (p<0,01). Dakle, scintigrafija pluća s Ga-67 vrlo je osjetljiv biljeg proširenosti i aktivnosti kod sarkoidoze, međutim, ima nisku specifičnost, pa ovu metodu u dobroj kliničkoj praksi treba rabiti u dijagnostički i terapijski dvojbenim slučajevima, vodeći pritom računa o dozi ozračivanja i cijeni

Morphometric and DNA Image Analysis of Bronchoalveolar Lavage Fluid Macrophages Nuclei in Interstitial Lung Diseases with Lymphocytic Alveolitis

Lymphocytic alveolitis is a characteristic of diverse interstitial lung diseases (ILD-s), but macrophages are often more numerous cell population in bronchoalveolar lavage fluid (BALF). Aim of this study is to analyze morphometric characteristics of macrophages nuclei in BALF in patients with ILD-s and to detect possible differences allowing distinguishing sarcoidosis from other lymphocytic alveolitis ILD-s. Thirty-one patient with interstitial lung disease who had lymphocytic alveolitis in BALF cell count (17 sarcoidosis and 14 other ILD-s) and nine controls were included in the study. The following patients data were numbered: age, lymphocyte percentage and CD4/CD8 ratio in BALF. Investigated morphometric parameters of macrophages nuclei were: area, outline, maximal radius, minimal radius, length, breadth, form factor (FF), elongation factor (EF) and DNA image cytometry ploidy status determined with Van Velthoven method. Predicted classifications in classification matrix (forward step-wise method in multivariate discriminant function analysis) based on macrophages nuclei length mean, minimum and maximum, breadth SD, FF mean and lymphocyte % were 100% (9/9) correct for control group, 88.235% (15/17) correct for sarcoidosis, and 92.857% (13/14) correct for other lymphocytic alveolitis ILD group. In total, 92.5% (37/40) of the examinees were correctly classified in particular group upon the observed variables

Epidemiological Characteristics of Sarcoidosis Patients Hospitalized in the University Hospital for Lung Diseases »Jordanovac« (Zagreb, Croatia) in the 1997–2002 Period

The aim of our study was to explore the characteristics of hospitalized patients with sarcoidosis concerning age, gender, clinical forms and staging, seasonality, geographical distribution, smoking habit and profession, familial clustering and mortality. We included 476 biopsy-proven sarcoidosis patients who were diagnosed at the University Hospital for Lung Diseases »Jordanovac« in the period from 1997–2002. Most of the patients (44%) were in the group of age between 20 and 40 years. The ratio of women to men was 1.4:1. The onset of the disease usually appeared in spring and summer, especially in the patients presenting with erythema nodosum, with majority of patients hospitalized in the period from May to August (51%). More patients came from urban, than from rural areas (1.5:1), and they were mostly nonsmokers (3.3:1). In 2% of sarcoidosis patients we found familial clustering. Although these data are biased regarding the selection of patients they give new insights into characteristics of sarcoidosis patients in Croatia