Idiopathic pulmonary fibrosis (IPF) is a rare lung disease with poor prognosis. The diagnosis and... more Idiopathic pulmonary fibrosis (IPF) is a rare lung disease with poor prognosis. The diagnosis and treatment possibilities are dependent on the health systems of countries. Hence, comparison among countries is difficult due to data heterogeneity. Our aim was to analyse patients with IPF in Central and Eastern Europe using the uniform data from the European Multipartner IPF registry (EMPIRE), which at the time of analysis involved 10 countries. Newly diagnosed IPF patients (N = 2,492, between March 6, 2012 and May 12, 2020) from Czech Republic (N = 971, 39.0%), Turkey (N = 505, 20.3%), Poland (N = 285, 11.4%), Hungary (N = 216, 8.7%), Slovakia (N = 149, 6.0%), Israel (N = 120, 4.8%), Serbia (N = 95, 3.8%), Croatia (N = 87, 3.5%), Austria (N = 55, 2.2%), and Bulgaria (N = 9, 0.4%) were included, and Macedonia, while a member of the registry, was excluded from this analysis due to low number of cases (N = 5) at this timepoint. Baseline characteristics, smoking habit, comorbidities, lung...
The Mount Sinai journal of medicine, New York, 2005
This is a case report of a patient diagnosed with three distinct primary intrathoracic tumors (me... more This is a case report of a patient diagnosed with three distinct primary intrathoracic tumors (mesothelioma, carcinoid and B-cell lymphoma). The patient had previously had mycosis fungoides. The occurrence of multiple neoplasms in a single patient, synchronous or metasynchronous, is not a rare phenomenon; the incidence varies from 1-11% of all neoplasms. They can be hereditary, or connected with some environmental agents or previous therapies. The incidence of multiple neoplasms increases with age. We report an extremely rare case of multiple intrathoracic neoplasms in a 71-year-old man. A left upper lobectomy was performed, followed by 6 courses of chemotherapy and irradiation of the sternum. The patient was stable two years later.
Various genetic and environmental factors have been implicated in the development of sarcoidosis.... more Various genetic and environmental factors have been implicated in the development of sarcoidosis. HLA-DRB1*15 has been proposed as a marker of disease chronicity in Irish ad HLA-DQB1*0602-DRB1*150101 of poor outcome in Dutch sarcoidosis patients, but validation in other ethnic groups is necessary. We have tested 134 Croatian sarcoidosis patients and 285 ethnically-matched controls for HLA-DRB1 specificities by PCR-SSP method. Diagnosis of sarcoidosis was based on consistent clinical features, together with biopsy-proven noncaseating epitheloid cell granulomas. All patients were followed up for 2-18, average 4.5 years. The follow up armamentarium included the clinical examination, chest X ray, pulmonary function measurements, and other tests to evaluate the course of extrapulmonary sarcoidosis, if present. At the end of follow up period 40 patients (29.85%) had chronic sarcoidosis while the remaining 94 subjects showed no sign of sarcoidosis. DRB1*03 specificity was significantly les...
Sarcoidosis is a heterogeneous multisystem granulomatous disease that primarily affects the lungs... more Sarcoidosis is a heterogeneous multisystem granulomatous disease that primarily affects the lungs. Numerous studies have reported an association of HLA alleles with sarcoidosis, with variation of alleles in different ethnic groups. Therefore, to identify the significance of HLA alleles in the development of sarcoidosis in the Croatian population, we investigated the HLA-DRB1 alleles in patients and healthy control subjects. Ninety patients with sarcoidosis were diagnosed at the Clinical Hospital for Lung diseases Jordanovac, Zagreb. Diagnosis was based on the presence of typical clinical features, chest X-ray findings and biopsy evidence of granuloma. As a control group we used 141 untreated healthy individuals. All subjects were typed for HLA-DRB1 polymorphism by PCR-SSP method. 1) Analysis of DRB1 alleles between all patients and controls showed significant differences and higher frequencies only for DRB1*0301 allele (p=0.0023), while lower frequencies of DRB1*07 and DRB1*13 allel...
CPFE-combined pulmonary fibrosis and emphysema is a new term for a syndrome whose main characteri... more 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 pol...
91 Ivieeviae A. et al. Correlation of lung gallium-67 scintigraphy with lung x-ray and pulmonary ... more 91 Ivieeviae A. et al. Correlation of lung gallium-67 scintigraphy with lung x-ray and pulmonary function tests in patients with sarcoidosis CORRELATION OF LUNG GALLIUM-67 SCINTIGRAPHY WITH LUNG X-RAY AND PULMONARY FUNCTION TESTS IN PATIENTS WITH SARCOIDOSIS Antonija Ivieeviae 1, Tatjana Pero-Golubieiae 1, Draen Huiae 2, Stanko Teak 2, Marjan Goreean 1, Jasna Tekavec-Trkanjec 1 and Marija Aliloviae 1 1Jordanovac University Hospital for Lung Diseases; 2University Department of Nuclear Medicine and Radiation Protection, Zagreb University Hospital Center, Zagreb, Croatia
Introduction Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by widespread ve... more Introduction Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by widespread venous/arterial thrombosis and pulmonary artery aneurysms (PAAs), which is associated with serious morbidity and mortality. All fatalities reported in HSS resulted from unpredictable fatal suffocating hemoptysis. Therefore, it is necessary to recognize pulmonary complications at an early stage of the disease. Objectives The aims of this study are to develop a reference atlas of images depicting the characteristic features of HSS by computed tomography pulmonary angiography (CTPA). To make a guide for physicians by developing a classification of PAAs according to the severity and risk of complications associated with each distinct lesion type. Methods The Members of the HSS International Study Group (HSSISG) collected 42 cases, with high-quality CTPA images in one radiology station and made reconstructions from the source images. These detailed CTPA studies were reviewed for final image sel...
Background Several registries of idiopathic pulmonary fibrosis (IPF) have been established to bet... more Background Several registries of idiopathic pulmonary fibrosis (IPF) have been established to better understand its natural history, though their size and duration of follow-up are limited. Here, we describe the large European MultiPartner IPF Registry (EMPIRE) and validate predictors of long-term survival in IPF. Methods The multinational prospective EMPIRE registry enrolled IPF patients from 48 sites in 10 Central and Eastern European countries since 2014. Survival from IPF diagnosis until death was estimated, accounting for left-truncation. The Cox proportional hazards regression model was used to estimate adjusted hazard ratios (HR) of death for prognostic factors, using restricted cubic splines to fit continuous factors. Results The cohort included 1620 patients (mean age at diagnosis 67.6 years, 71% male, 63% smoking history), including 75% enrolled within 6 months of diagnosis. Median survival was 4.5 years, with 45% surviving 5 years post-diagnosis. Compared with GAP stage I...
Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of small-vessel... more Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of small-vessel vasculitis characterized by asthma, hyper-eosinophilia, and progressive multiorgan involvement. EGPA is traditionally treated using corticosteroids, either alone or in combination with conventional immunosuppressants. Mepolizumab, a novel anti-interleukin (IL)-5 agent, has been approved as an add-on therapy for adult patients with EGPA. The recommended dose of mepolizumab is 300 mg (subcutaneous [SC]) every 4 weeks.Case Study: The present report discusses three cases of refractory and/or relapsing EGPA in patients regularly taking a stable dose of prednisolone, all of whom were successfully treated with a lower-than-recommended dose of mepolizumab (100 mg SC, every 4 weeks).Results: Treatment with a low dose of mepolizumab enabled us to gradually reduce steroid doses. In addition, patients treated with low doses of mepolizumab exhibited better asthma control and experienced sustained relapse-free periods. Responses to 100 mg of mepolizumab were comparable to those previously observed in patients taking the recommended dose of 300 mg.Conclusion: Our findings suggest that mepolizumab at a third of the recommended dose can achieve reasonable clinical efficacy in the long-term treatment of EGPA in some patients. Initiation of mepolizumab therapy in the early, eosinophilic phase of EGPA-which is characterized by asthma, marked blood eosinophilia, pulmonary infiltrates, and sinonasal abnormalities-may help to prevent the deleterious side-effects of long-term steroid use while reducing the cost of EGPA treatment.
Aim: Treatment either with nintedanib (N) or pirfenidone (P) can slow progression of idiopathic p... more Aim: Treatment either with nintedanib (N) or pirfenidone (P) can slow progression of idiopathic pulmonary fibrosis (IPF). Long term effect of this treatment in the real world has not been adequately documented to date. We aimed to compare the overall survival (OS) and progression free survival (PFS) in the patients from the European MultiPartner IPF registry (EMPIRE) treated either by P or N or switch from either N to P or P to N (S) with other or no treatment (O). Patients and Methods: 2745 IPF patients from the EMPIRE registry (809 F and 1936 M, mean age at diagnosis 66.5 years) were enrolled to the retrospective study (821 on P, 637 on N, 148 S and 1139 O). Progression was defined as death, or transplantation, or decline of FVC ˃ 10% or TLCO ˃ 15% compared to previous value. OS and PFS in the subgroups were counted and compared. Results: Median OS in months was from the entry to the registry: 56.7 P, 84.7 N, 76.9 S, 37.5 O; 5-years survival rate was: 0.461 P, 0.622 N, 0.65 S, 0.333 O and 10-years survival rate was: 0.216 P, 0.364 N, 0.491 S and 0.139 O (p ˂0.001). Median PFS in months was from the entry to the registry: 20.5 P, 22.9 N, 30.4 S and 17.3 O, 5-years PFS rate was: 0.173 P, 0.232 N, 0.333 S, 0.094 O and 10-years PFS rate was: 0.04 P, 0.055 N, 0.084 S and 0.008 O (p ˂0.001). Conclusion: The results show a consistent effect of antifibrotic treatment on OS and PFS in a large cohort of the real-world IPF patients. Moreover, the effect of a switch of antifibrotics on OS and PFS is even higher than either P or N alone. The EMPIRE registry is supported by Boehringer Ingelheim
Idiopathic pulmonary fibrosis (IPF) is a rare lung disease with poor prognosis. The diagnosis and... more Idiopathic pulmonary fibrosis (IPF) is a rare lung disease with poor prognosis. The diagnosis and treatment possibilities are dependent on the health systems of countries. Hence, comparison among countries is difficult due to data heterogeneity. Our aim was to analyse patients with IPF in Central and Eastern Europe using the uniform data from the European Multipartner IPF registry (EMPIRE), which at the time of analysis involved 10 countries. Newly diagnosed IPF patients (N = 2,492, between March 6, 2012 and May 12, 2020) from Czech Republic (N = 971, 39.0%), Turkey (N = 505, 20.3%), Poland (N = 285, 11.4%), Hungary (N = 216, 8.7%), Slovakia (N = 149, 6.0%), Israel (N = 120, 4.8%), Serbia (N = 95, 3.8%), Croatia (N = 87, 3.5%), Austria (N = 55, 2.2%), and Bulgaria (N = 9, 0.4%) were included, and Macedonia, while a member of the registry, was excluded from this analysis due to low number of cases (N = 5) at this timepoint. Baseline characteristics, smoking habit, comorbidities, lung...
The Mount Sinai journal of medicine, New York, 2005
This is a case report of a patient diagnosed with three distinct primary intrathoracic tumors (me... more This is a case report of a patient diagnosed with three distinct primary intrathoracic tumors (mesothelioma, carcinoid and B-cell lymphoma). The patient had previously had mycosis fungoides. The occurrence of multiple neoplasms in a single patient, synchronous or metasynchronous, is not a rare phenomenon; the incidence varies from 1-11% of all neoplasms. They can be hereditary, or connected with some environmental agents or previous therapies. The incidence of multiple neoplasms increases with age. We report an extremely rare case of multiple intrathoracic neoplasms in a 71-year-old man. A left upper lobectomy was performed, followed by 6 courses of chemotherapy and irradiation of the sternum. The patient was stable two years later.
Various genetic and environmental factors have been implicated in the development of sarcoidosis.... more Various genetic and environmental factors have been implicated in the development of sarcoidosis. HLA-DRB1*15 has been proposed as a marker of disease chronicity in Irish ad HLA-DQB1*0602-DRB1*150101 of poor outcome in Dutch sarcoidosis patients, but validation in other ethnic groups is necessary. We have tested 134 Croatian sarcoidosis patients and 285 ethnically-matched controls for HLA-DRB1 specificities by PCR-SSP method. Diagnosis of sarcoidosis was based on consistent clinical features, together with biopsy-proven noncaseating epitheloid cell granulomas. All patients were followed up for 2-18, average 4.5 years. The follow up armamentarium included the clinical examination, chest X ray, pulmonary function measurements, and other tests to evaluate the course of extrapulmonary sarcoidosis, if present. At the end of follow up period 40 patients (29.85%) had chronic sarcoidosis while the remaining 94 subjects showed no sign of sarcoidosis. DRB1*03 specificity was significantly les...
Sarcoidosis is a heterogeneous multisystem granulomatous disease that primarily affects the lungs... more Sarcoidosis is a heterogeneous multisystem granulomatous disease that primarily affects the lungs. Numerous studies have reported an association of HLA alleles with sarcoidosis, with variation of alleles in different ethnic groups. Therefore, to identify the significance of HLA alleles in the development of sarcoidosis in the Croatian population, we investigated the HLA-DRB1 alleles in patients and healthy control subjects. Ninety patients with sarcoidosis were diagnosed at the Clinical Hospital for Lung diseases Jordanovac, Zagreb. Diagnosis was based on the presence of typical clinical features, chest X-ray findings and biopsy evidence of granuloma. As a control group we used 141 untreated healthy individuals. All subjects were typed for HLA-DRB1 polymorphism by PCR-SSP method. 1) Analysis of DRB1 alleles between all patients and controls showed significant differences and higher frequencies only for DRB1*0301 allele (p=0.0023), while lower frequencies of DRB1*07 and DRB1*13 allel...
CPFE-combined pulmonary fibrosis and emphysema is a new term for a syndrome whose main characteri... more 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 pol...
91 Ivieeviae A. et al. Correlation of lung gallium-67 scintigraphy with lung x-ray and pulmonary ... more 91 Ivieeviae A. et al. Correlation of lung gallium-67 scintigraphy with lung x-ray and pulmonary function tests in patients with sarcoidosis CORRELATION OF LUNG GALLIUM-67 SCINTIGRAPHY WITH LUNG X-RAY AND PULMONARY FUNCTION TESTS IN PATIENTS WITH SARCOIDOSIS Antonija Ivieeviae 1, Tatjana Pero-Golubieiae 1, Draen Huiae 2, Stanko Teak 2, Marjan Goreean 1, Jasna Tekavec-Trkanjec 1 and Marija Aliloviae 1 1Jordanovac University Hospital for Lung Diseases; 2University Department of Nuclear Medicine and Radiation Protection, Zagreb University Hospital Center, Zagreb, Croatia
Introduction Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by widespread ve... more Introduction Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by widespread venous/arterial thrombosis and pulmonary artery aneurysms (PAAs), which is associated with serious morbidity and mortality. All fatalities reported in HSS resulted from unpredictable fatal suffocating hemoptysis. Therefore, it is necessary to recognize pulmonary complications at an early stage of the disease. Objectives The aims of this study are to develop a reference atlas of images depicting the characteristic features of HSS by computed tomography pulmonary angiography (CTPA). To make a guide for physicians by developing a classification of PAAs according to the severity and risk of complications associated with each distinct lesion type. Methods The Members of the HSS International Study Group (HSSISG) collected 42 cases, with high-quality CTPA images in one radiology station and made reconstructions from the source images. These detailed CTPA studies were reviewed for final image sel...
Background Several registries of idiopathic pulmonary fibrosis (IPF) have been established to bet... more Background Several registries of idiopathic pulmonary fibrosis (IPF) have been established to better understand its natural history, though their size and duration of follow-up are limited. Here, we describe the large European MultiPartner IPF Registry (EMPIRE) and validate predictors of long-term survival in IPF. Methods The multinational prospective EMPIRE registry enrolled IPF patients from 48 sites in 10 Central and Eastern European countries since 2014. Survival from IPF diagnosis until death was estimated, accounting for left-truncation. The Cox proportional hazards regression model was used to estimate adjusted hazard ratios (HR) of death for prognostic factors, using restricted cubic splines to fit continuous factors. Results The cohort included 1620 patients (mean age at diagnosis 67.6 years, 71% male, 63% smoking history), including 75% enrolled within 6 months of diagnosis. Median survival was 4.5 years, with 45% surviving 5 years post-diagnosis. Compared with GAP stage I...
Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of small-vessel... more Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of small-vessel vasculitis characterized by asthma, hyper-eosinophilia, and progressive multiorgan involvement. EGPA is traditionally treated using corticosteroids, either alone or in combination with conventional immunosuppressants. Mepolizumab, a novel anti-interleukin (IL)-5 agent, has been approved as an add-on therapy for adult patients with EGPA. The recommended dose of mepolizumab is 300 mg (subcutaneous [SC]) every 4 weeks.Case Study: The present report discusses three cases of refractory and/or relapsing EGPA in patients regularly taking a stable dose of prednisolone, all of whom were successfully treated with a lower-than-recommended dose of mepolizumab (100 mg SC, every 4 weeks).Results: Treatment with a low dose of mepolizumab enabled us to gradually reduce steroid doses. In addition, patients treated with low doses of mepolizumab exhibited better asthma control and experienced sustained relapse-free periods. Responses to 100 mg of mepolizumab were comparable to those previously observed in patients taking the recommended dose of 300 mg.Conclusion: Our findings suggest that mepolizumab at a third of the recommended dose can achieve reasonable clinical efficacy in the long-term treatment of EGPA in some patients. Initiation of mepolizumab therapy in the early, eosinophilic phase of EGPA-which is characterized by asthma, marked blood eosinophilia, pulmonary infiltrates, and sinonasal abnormalities-may help to prevent the deleterious side-effects of long-term steroid use while reducing the cost of EGPA treatment.
Aim: Treatment either with nintedanib (N) or pirfenidone (P) can slow progression of idiopathic p... more Aim: Treatment either with nintedanib (N) or pirfenidone (P) can slow progression of idiopathic pulmonary fibrosis (IPF). Long term effect of this treatment in the real world has not been adequately documented to date. We aimed to compare the overall survival (OS) and progression free survival (PFS) in the patients from the European MultiPartner IPF registry (EMPIRE) treated either by P or N or switch from either N to P or P to N (S) with other or no treatment (O). Patients and Methods: 2745 IPF patients from the EMPIRE registry (809 F and 1936 M, mean age at diagnosis 66.5 years) were enrolled to the retrospective study (821 on P, 637 on N, 148 S and 1139 O). Progression was defined as death, or transplantation, or decline of FVC ˃ 10% or TLCO ˃ 15% compared to previous value. OS and PFS in the subgroups were counted and compared. Results: Median OS in months was from the entry to the registry: 56.7 P, 84.7 N, 76.9 S, 37.5 O; 5-years survival rate was: 0.461 P, 0.622 N, 0.65 S, 0.333 O and 10-years survival rate was: 0.216 P, 0.364 N, 0.491 S and 0.139 O (p ˂0.001). Median PFS in months was from the entry to the registry: 20.5 P, 22.9 N, 30.4 S and 17.3 O, 5-years PFS rate was: 0.173 P, 0.232 N, 0.333 S, 0.094 O and 10-years PFS rate was: 0.04 P, 0.055 N, 0.084 S and 0.008 O (p ˂0.001). Conclusion: The results show a consistent effect of antifibrotic treatment on OS and PFS in a large cohort of the real-world IPF patients. Moreover, the effect of a switch of antifibrotics on OS and PFS is even higher than either P or N alone. The EMPIRE registry is supported by Boehringer Ingelheim
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