Emphysema- and airway-dominant COPD phenotypes defined by standardised quantitative computed tomography.

EvA (Emphysema versus Airway disease) is a multicentre project to study mechanisms and identify biomarkers of emphysema and airway disease in chronic obstructive pulmonary disease (COPD). The objective of this study was to delineate objectively imaging-based emphysema-dominant and airway disease-dominant phenotypes using quantitative computed tomography (QCT) indices, standardised with a novel phantom-based approach.441 subjects with COPD (Global Initiative for Chronic Obstructive Lung Disease (GOLD) stages 1-3) were assessed in terms of clinical and physiological measurements, laboratory testing and standardised QCT indices of emphysema and airway wall geometry.QCT indices were influenced by scanner non-conformity, but standardisation significantly reduced variability (p<0.001) and led to more robust phenotypes. Four imaging-derived phenotypes were identified, reflecting "emphysema-dominant", "airway disease-dominant", "mixed" disease and "mild" disease. The emphysema-dominant group had significantly higher lung volumes, lower gas transfer coefficient, lower oxygen (PO2 ) and carbon dioxide (PCO2 ) tensions, higher haemoglobin and higher blood leukocyte numbers than the airway disease-dominant group.The utility of QCT for phenotyping in the setting of an international multicentre study is improved by standardisation. QCT indices of emphysema and airway disease can delineate within a population of patients with COPD, phenotypic groups that have typical clinical features known to be associated with emphysema-dominant and airway-dominant disease.

[Liposarcoma--a rare primary neoplasm of the thorax]. / Tluszczakomiesak--rzadki pierwotny nowotwór w klatce piersiowej.

Liposarcoma is a rare tumour localised within the thorax. It can originate from a different thoracic structures (for example: lung parenchyma, mediastinum, pleura) or thoracic wall. We present a case of a 35-year-old woman with a giant tumour of the left hemithorax, who had two weeks history of non-productive cough and progressive dyspnoea from eight months. Chest radiography showed a large, round opacity in the left hemithorax, which displaced the cardiac silhouette to the right. Contrast-enhanced computed tomography showed a huge, heterogeneous, well-circumscribed mass in the left pleural cavity. The tumour expanded locally towards the thoracic wall and left lung, and displaced mediastinal structures to the right. There was no lymphadenopathy on the physical examination and CT scan. Abdominal ultrasonography was normal. There was no proof of tumour in the extremities and trunk. The patient underwent surgical excision of the tumour via thoracotomy, but because of tumour infiltration, part of the third rib and surrounding wall were resected. There was no evidence of lung and mediastinum involvement. The mass measured 17.5 × 18 × 10 cm and weighed 1690 g. A final diagnosis of a well-differentiated liposarcoma - sclerosing subtype - was established after histological and immunohistochemical staining. In our opinion, the liposarcoma in this case originated from the chest wall. Surgical resection was the only treatment. During six months after surgery the patient had no evidence of disease progression.

[Pneumocystis pneumonia in HIV-infected patients with cytomegalovirus co-infection. Two case reports and a literature review]. / Pneumocystozowe zapalenie pluc u zakazonych HIV przy wspólistniejacym zakazeniu cytomegalowirusem. Opis dwóch przypadków i przeglad literatury.

Pneumocystis pneumonia (PCP) is one of the infectious lung diseases diagnosed in HIV-infected patients. The pathogen responsible for the development of this opportunistic infection is an atypical fungus called Pneumocystis jiroveci. PCP remains the most common disorder diagnosed at the onset of acquired immunodeficiency syndrome (AIDS), especially in individuals not aware of their HIV infection. The most important risk factor of PCP development in HIV-infected person is the decrease of T CD4+ cell number below 200/mcL. Clinical symptoms consist of: chronic cough, dyspnoea and weakness. Arterial blood gas analysis often reveals the presence of hypoxaemia, and high-resolution computed tomography imaging shows diffuse ground glass opacities. Treatment is based on intravenous administration of trimetoprim-sulfamethoxasole. In patients with moderate and severe symptoms of PCP it is recommended that corticosteroids are used. Co-infection with cytomegalovirus (CMV) is a poor prognostic sign in PCP. CMV-related organ disease should be suspected in patients with T CD4+ cell number lower than 50/mcL. It is arguable whether CMV infection in symptomatic PCP patients should be treated. There are suggestions that corticosteroids used in PCP patients with CMV co-infection could promote the development of CMV pneumonia. In the present paper we present two patients with PCP, unaware of their HIV infection. In both cases a CMV co-infection was found.

Non-tuberculous mycobacterial lung disease (NT MLD ) in patients with chronic thromboembolic pulmonary hypertension and idiopathic pulmonary arterial hypertension.

INTRODUCTION: Non-tuberculous mycobacterial lung diseases (NTMLD) occur rarely and are diagnosed mainly in patients belonging to risk groups. Pulmonary hypertension (PH) has not been recognised as a risk factor for NTMLD yet. The aim of the study was to analyse the clinical course and predisposing factors of NTMLD recognised in our centre between 2002 and 2012 in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and idiopathic pulmonary arterial hypertension (IPAH). MATERIAL AND METHODS: Thirteen patients (10 - CTEPH, 3 - IPAH) entered the study. PH was recognised during right heart catheterisation. Median value of mean pulmonary artery pressure (mPAP) was 49 mm Hg (39-65 mm Hg). NTMLD was diagnosed according to ATS guidelines (2007). RESULTS: M. kansasii was the most frequent pathogen. Most patients complained of the exaggeration of dyspnoea and productive cough. Computed tomography of the chest with angiography revealed infiltrations with cavitation in seven patients and cavities surrounded by micronodules in six patients. In all CTEPH patients, NTMLD developed in the hypoperfused lung areas. No parenchymal abnormalities preceded the development of NTMLD. After diagnosis all of the patients received antituberculous treatment; in 12/13 improvement was achieved. By the end of March 2014 seven patients died due to right heart insufficiency, no deaths due to NTMLD were noted. CONCLUSIONS: NTMLD should be suspected in patients with CTEPH or IPAH, presenting with productive cough and a new pulmonary infiltrate with cavitation. In patients with CTEPH, special attention should be paid to a new cavitary lesions without accompanying thrombus in the artery supplying the area. High mPAP (CTEPH/IPAH) and hypoperfusion (CTEPH) are predisposing to NTMLD.

Severe congestive heart failure as the main symptom of eosinophilic granulomatosis and polyangiitis (Churg-Strauss syndrome).

Patients with cardiovascular symptoms are mainly diagnosed in cardiological wards. However, sometimes the other reasons for acute coronary syndrome and heart failure are found. One of such reasons is hypereosinophilia which can be recognized if number of blood eosinophils exceeds 1500/mm3. High eosinophilia is connected with production of cytotoxic eosinophilic proteins which can cause eosinophilic vasculitis or eosinophilic myocarditis. One of the better known hypereosinophilic syndromes is EGPA described by the pathomorphologists Churg and Strauss. The further research works allowed for the clinical characteristics of patients with EGPA. In the course of this disease the following three phases were recognized : prodromal-allergic, eosinophilic, vasculitic. The definitive diagnosis can be established only in the third phase, when vasculitis causes organ involvement. Besides symptoms of the respiratory tract (asthma, nasal polyps, eosinophilic lung infiltrations) also cardiovascular symptoms, gastrointestinal tract symptoms, as well as skin lesions and kidneys involvement can appear. The most dangerous for patients is involvement of the nervous and cardiovascular systems. We present a patient with asthma and eosinophilia in whom EGPA was diagnosed in the course of acute recurrent substernal chest pain, with subsequent signs of cardiac insufficiency.

Accuracy of FDG PET/CT in the evaluation of solitary pulmonary lesions - own experience.

INTRODUCTION: In recent years, positron emission tomography (PET) has been increasingly applied in the diagnosis of neoplastic lung diseases. In contrast to conventional imaging studies, PET-CT enables the visualisation of not only the morphology of the suspicious lesion, but also its metabolism. The aim of the present study was to investigate the role of PET-CT in the initial assessment of patients with indeterminate solitary pulmonary lesions. MATERIAL AND METHODS: The study was conducted on a group of 82 patients with indeterminate lung nodule diagnosed at the National Institute of Tuberculosis and Lung Diseases in the period from January 2008 to May 2011. CT and PET-CT were performed in all of the patients. Histological or cytological examination of the biopsy specimens obtained from bronchoscopy, mediastinoscopy and intraoperatively were the reference tests. RESULTS: Malignancy was documented in 40 patients (48.8%). Histopathological analysis of all tumours revealed 12 cases of squamous cell carcinoma, 18 cases of adenocarcinoma and 1 case of carcinoid, whereas in 9 patients the diagnosis of "non-small cell cancer not otherwise specified" was made. All lesions except one were of solid character on chest CT. SUV(max) values exceeding 2.5 were found in 38 cancer patients (true positives, TP). The mean value of SUV(max) was 9.1 (1-26.8). Forty-two lesions were documented as benign (51.2%). SUV(max) values equal to or less than 2.5 were found in 37 patients (true negatives, TN). The mean value of SUV(max) in this group was 1.9 (0.5-8.6). The diagnostic value of PET-CT SUV(max) exceeding 2.5 in the prediction of neoplastic origin of solitary pulmonary lesions was: sensitivity - 95% (95% CI 84-99%), specificity - 88% (95% CI 75-95%) and accuracy - 91.5% (95% CI 83-96%). Positive predictive value (PPV) was 88.4% (95% CI 76-95%), and negative predictive value (NPV) was 94.8% (95% CI 83-99%). False negative results concerned two patients, with final diagnosis of carcinoid and adenocarcinoma; false positive results were obtained in 5 patients with various inflammatory lesions. CONCLUSIONS: In the present study, PET-CT appeared to have high sensitivity (95%), but lower specificity (88%) for predicting the malignant character of solitary pulmonary lesions. Overall diagnostic value of PET-CT SUV(max) > 2.5 was high - PPV was 88.4%, NPV was 94.8%. In the authors' opinion, the PET-CT value may increase when clinical data as well as other radiological documentation (with retrospective assessment) are taken into consideration.

[Pneumocystosis in a patient with lymphangioleiomyomatosis]. / Pneumocystozowe zapalenie pluc u chorej na limfangioleiomiomatoze

A 47-year-old, non-smoking woman was admitted to the National Tuberculosis and Lung Diseases Research Institute for diagnosis of progressive exertional dyspnoea and numerous small thin-walled, air-filled cysts equally distributed throughout both lungs revealed in HRCT (high resolution computed tomography) examination. Histological assessment of specimens obtained by open lung biopsy revealed proliferation of immature smooth muscle, showing the expression of the antigen HMB45. On this basis, diagnosis of lymphangioleiomyomatosis was established. The disease caused essential ventilation damage of the lungs (FEV1 1.34 L; 39.71% pred, VC 4.02 L; 94.96% pred, FEV1/ /VC 0.33-4 1.81% pred, DLCO 3.65 mmol/min/Kpa 38.35% pred).During the observation, despite the lack of immunological disorders, the patient developed Pneumocystis jiroveci pneumonia (PCP) that was treated with trimethoprimsulfamethoxazole. Lymphangioleiomyomatosis is a rare disease which results from a defect of TSC genes. The disease is not related to immunological defects or disorders. However, the considerable cystic destruction of the lungs can predispose the patient to opportunistic infections such as the one in the presented case.

[Fibrosing mediastinitis with pulmonary hypertension as a rare complication of sarcoidosis]. / Wlóknienie sródpiersia z nadcisnieniem plucnym jako rzadkie powiklanie sarkoidozy.

Fibrosing mediastinitis is a rare disease, mostly associated with previous granulomatous diseases, such as histoplasmosis, tuberculosis, or sarcoidosis. We present a case of 42-year-old woman with sarcoidosis, who had developed fibrosing mediastinitis and pulmonary hypertension. Contrast-enhanced computer tomography showed abnormal, bilateral, solid tissues surrounding the hila and mediastinum. Magnetic resonance imaging showed: abnormal, intermediate signal tissue in the mediastinum, surrounding hila, narrowing both lower lobe arteries and both lobe bronchi, left upper lobe atelectasis, and contrast enhancement of mediastinal infiltration. The patient was treated with steroids for 14 months (initial dose of prednisone was 1 mg/kg/day, then tapered) with clinical and radiological improvement. We reviewed the literature concerning fibrosing mediastinitis. The various causes of that disorder, the radiological manifestation, and possible treatment modalities are discussed. The causes of pulmonary hypertension in our patient are another aspect of the article. It could be associated with both the fibrosing mediastinitis and the sarcoidosis.

[Staging of non-small cell lung cancer using CT and integrated PET-CT]. / Ocena zaawansowania niedrobnokomórkowego raka pluca metodatomografii komputerowej i pozytonowej tomografii emisyjnejskojarzonej z tomografia komputerowa

INTRODUCTION: Lung cancer is the leading cause of death from cancer in developed countries. Radiological imaging methods are the basic methods in early diagnosis of this disease. TNM classification is a very important tool for optimal treatment in non-small lung cancer (NSCLC). Conventional radiological techniques allow the evaluation of the stage on the basis of anatomical changes only, while PET-CT provides information about the biochemical processes that may precede anatomical changes. The aim of this study was to compare the accuracy and sensitivity of CT and PET-CT in the staging of NSCLC. MATERIAL AND METHODS: The study was conducted on a group of 99 patients with NSCLC diagnosed at the Institute of Tuberculosis and Lung Diseases in the period from January 2008 to May 2010. CT and PET-CT were performed in all patients. Histological or cytological examination of the material obtained from biopsy, bronchoscopy, mediastinoscopy, and intraoperatively was the reference test. TNM classification was performed independently after CT and PET-CT. RESULTS AND CONCLUSIONS: It has been shown that PET-CT is a more accurate and sensitive method than CT in the staging process in NSCLC. PET-CT allowed the correct classification of the T, N, M, and total TNM in, respectively, 97%, 95%, 99%, and 89% of cases, while for CT it was, respectively, 95%, 84%, 84%, and 68% (p = 0.0002).

[Interstitial lung disease in patients with polymyositis and dermatomyositis--report of three cases]. / Zmiany sródmiazszowe w plucach u chorych na zapalenie wielomiesniowe i skórno-miesniowe--prezentacja trzech przypadków.

Polymyositis (PM) and dermatomyositis (DM) are connective tissue diseases (CTD) characterized by proximal muscle weakness along with changes in various internal organs, with the lungs most frequently involved. Presentation of the disease in the lungs comprises diffuse alveolar haemorrhage due to vasculitis and interstitial lung disease (ILD), which is the most frequent manifestation of CTD in the lungs and worsens the outcome and prognosis. The mechanisms involved in the ILD are not fully known, but the role of autoimmune response is unquestioned. No relationship between the severity of CTD and the changes in the lungs was observed. ILD may present at any time in the course of CTD, sometimes before the signs and symptoms of myositis occur. The more accurate imaging methods are, the more frequently changes in the lungs are detected. High resolution computed tomography (HRCT) is a gold standard in ILD imaging. Treatment of PM/DM-related ILD relays on systemic glucocorticosteroids as the first choice drugs. We present three cases of PM/DM-related ILD in middle-aged men, with a different clinical and radiological presentation. In all cases, apart from imaging (plain X-ray and HRCT of the chest) and pulmonary function tests, histological evaluation of lung changes was performed. In two cases non-specific interstitial pneumonitis (NSIP) was diagnosed, and in the third--organizing pneumonia along with sarcoid changes in the lymph nodes. Because of decreased pulmonary function all patients were treated with systemic corticosteroids and two of them additionally with azathioprine or cyclophosphamide, and the outcome was good in all of them.

Benign pulmonary metastasizing leiomyoma uteri. Case report and review of literature.

Benign metastasizing leiomyoma (BML) is a rare condition in middle-aged women with a history of uterine leiomyomata. It is characterized by the proliferation of, usually multiple, smooth muscle nodules. Approximately 100 cases have been reported in the literature, and the lungs were the most common site of metastases. We report a case of 52-year-old obese woman (BMI 31), hospital worker, smoker, admitted to the hospital with exertional dyspnoea, night sweats, loss of weight, and productive cough. Hysterectomy for a uterine leiomyoma was performed 9 years earlier. In addition, a history of two episodes of superficial vein thrombosis 3 and 2 years before admission was noted. Chest X-ray and subsequently CT chest examinations revealed multiple, non-calcified nodules within the middle and lower parts of both lungs. Specimens obtained by transbronchial biopsy (TBLB) and from open lung biopsy displayed benign muscle cell proliferation compatible with BML. The levels of sex hormones were characteristic for the menopause; therefore, observation was advised. Additionally, Streptococcus pneumoniae was cultured from bronchial washing, and bronchitis was diagnosed. Antibiotics, bronchodilators, and mucolytics were administered, and dyspnoea and cough with expectoration were diminished. Two years later pulmonary lesions have been stable; however, she has put on weight. Subsequently the patient has developed deep vein thrombosis with pulmonary embolism. Anticoagulant treatment was introduced, with some improvement.

[The basis of Churg-Strauss syndrome diagnosis in own material]. / Podstawy rozpoznania zespolu Churga-Strauss w materiale wlasnym.

INTRODUCTION: The diagnosis of Churg-Strauss syndrome (CSS) is difficult because pathological criteria are present in minority of patients and in advanced stages. Several centers elaborated criteria which allowed to suspect CSS in patients with asthma, hypereosinophilia and clinical manifestations consistent with systemic vasculitis with or without histologic evidence. The aim of the study is the presentation of the basis of CSS diagnosis in our material. MATERIAL AND METHODS: The analysis included 38 patients. Blood and biochemical analysis, radiological examinations of the chest and sinuses, echocardiography were performed in all patients. In 22/23 patients with cardiac symptoms magnetic resonance of the heart was performed. In two patients mediastinoscopy was performed, in 4--laparotomy. RESULTS: Only in 13/38 patients vasculitis was histologically proven, in the rest the diagnosis was established mainly on clinical ground. In 23 patients the diagnosis was established on the clinical cardiac symptoms--in all of them the cardiac involvement was confirmed by magnetic resonance. In 9 cases skin leasions were cause of further diagnostic procedures, 6 patients presented gastrointestinal symptoms, in 15--paralysis of peroneal nerve was observed, and 1 patient had polyneuropathy and symptoms of central nervous system. CONCLUSIONS: The diagnosis of CSS in our material was established mainly on clinical ground, because histologic material was difficult to obtain (it needs invasive procedures). Only in 13 from 38 patients vasculitis was histologically proven. The presence of asthma, sinusitis associated with peripheral eosinophilia and organ symptoms suggesting a vasculitis could support the diagnosis, without histologic evidence.

[Interstitial lung disease in patients with primary biliary cirrhosis]. / Zmiany sródmiazszowe w plucach u chorych na pierwotna zólciowa marskosc watroby.

Primary biliary cirrhosis (PBC) is a chronic autoimmune disorder of unknown etiology. The disease affects middle-aged women and is characterized by the destruction of the intralobular bile ducts that causes consequent cholestasis. AMA is a hallmark of PBC, composed mostly of IgG and IgM class. The M2 antibody is the most specific one, with sensitivity range of 54-98% depending on type of test used. PBC is often accompanied by other autoimmune diseases, such as Sjögrens syndrome, thyroiditis, rheumatoid arthritis, dermatomyositis, polymyositis. Interstitial lung disease (ILD) has been reported in patients with primary biliary cirrhosis but its frequency and nature are poorly understood. We report pulmonary involvement in the course of PBC in 4 middle-aged women. Histopatological examination of lung specimens was available in three patients: two presented with sarcoid - like granulomas, one with lymphocytic interstitial pneumonia (LIP). In one patient the diagnosis of pulmonary fibrosis was based on clinical and radiological features. Because of abnormal pulmonary function tests (PFT) results all the patients were treated with prednisone, one, additionally with azathioprine. The treatment was successful in all of the patients.

Atypical Pulmonary Tuberculosis as the First Manifestation of Advanced HIV Disease-Diagnostic Difficulties.

Tuberculosis (TB) is the leading cause of morbidity, hospitalisations, and mortality in people living with HIV (PLWH). The lower CD4+ T-lymphocyte count in the course of HIV infection, the higher risk of active TB, and the higher odds for atypical clinical and radiologic TB presentation. These HIV-related alterations in TB presentation may cause diagnostic problems in patients not knowing they are infected with HIV. We report on a patient without any background medical conditions, who was referred to a hospital with a 4-month history of chest and feet pains, mild dry cough, fatigue, reduced appetite, and decreasing body weight. Chest X-ray revealed mediastinal lymphadenopathy, bilateral reticulonodular parenchymal opacities, and pleural effusion. A preliminary diagnosis of lymphoma, possibly with a superimposed infection was established. Further differential diagnostic process revealed pulmonary TB in the course of advanced HIV-1 disease, with a CD4+ T-lymphocyte count of 107 cells/mm3. The patient completed anti-tuberculous therapy and successfully continues on antiretroviral treatment. This case underlines the importance of screening for HIV in patients with newly diagnosed TB.

[Eosinophilic pneumonia in response to cutaneous larva migrans syndrome--a case report]. / Eozynofilowe zapalenie pluc w przebiegu zespolu larwy skórnej wedrujacej--opis przypadku.

Cutaneous larva migrans is a parasitic dermatosis imported by travelers returning from tropical and subtropical regions. In cutaneous larva migrans syndrome humans are incidental hosts and the larvae are unable to complete their natural cycle. Adult hookworms live in the intestines of dogs and cats, shedding eggs in feces that hatch and mature into larvae that can remain infective for months in the soil. Larvae penetrate the skin after contact with infected soil and cause an itchy creeping eruption. Cutaneous larva migrans is not usually associated with systemic symptoms and is rarely accompanied by peripheral blood eosinophilia. We report a patient who had both cutaneous larva migrans syndrome caused by Ancylostoma brasiliense and eosinophilic pneumonia after returning from Sri Lanka. The patient has been applied intravenous corticosteroids and local treatment with albendazole ointment with a very good clinical response.

[Reexpansion pulmonary edema and pleural bleeding after suction drainage of pneumothorax]. / Rozprezeniowy obrzek pluca i krwawienie oplucnowe po leczeniu drenazem ssacym odmy oplucnowej.

We present a case of 26 years old man with large spontaneous pneumothorax of about 5 days duration. Application of suction drainage was complicated by unilateral reexpansion pulmonary edema (REPE) and hemothorax developed during first 24 hours of treatment. On thoracotomy multifocal superficial pleural bleeding was seen which was probably attributed to the pathomechanisms similar to suggested in reexpansion pulmonary edema. The patient received supplemental oxygen therapy and completely recovered during next few days. The patient presented typical risk factors of REPE including: young age, large and prolonged pneumothorax. In such cases chest tube should be initially left off suction to prevent REPE.

[Bacteriologically confirmed pulmonary tuberculosis in a patient with lymphangioleiomyomatosis accompanying tuberous sclerosis syndrome]. / Gruzlica pluc potwierdzona bakteriologicznie u chorejna limfangioleiomiomatoze w przebiegu stwardnienia guzowatego.

Lymphangioleiomyomatosis (LAM) is a rare disease of unknown origin, that may be sporadic or develop in the course of tuberous sclerosis (TS). Patients do not present immune deficiency, but structural changes in the lung parenchyma (cysts) may encourage various infections, for example tuberculosis. Radiologic findings are often difficult to interpret, because of changes related to LAM itself. We present a young women with a history of TS and LAM, in whom protracting respiratory tract infection was finally diagnosed as tuberculosis. Initial diagnosis was based primarily on clinical signs and symptoms and treatment was started despite the negative result of the sputum microscopy for acid fast bacilli. In the course of treatment the diagnosis was supported by positive tuberculin skin test, interferon-gamma release assay and genetic test for M. tuberculosis in bronchoalveolar lavage fluid, and finally, positive sputum culture in liquid medium.

[Recurrent effusive pericarditis in the course of adult-onset Still's disease--case reports of two patients]. / Nawracajace wysiekowe zapalenie osierdzia w przebiegu choroby Stilla u doroslych--opis dwóch przypadków.

Pericardial effusion is caused by various pathological agents. In differential diagnosis infectious as well as non-infectious factors have to be considered. Adult-onset Still disease (AOSD)--relatively uncommon systemic inflammatory disorder of unknown etiology--is among possible diagnosis. The disease typically affects patients in the age between 16-35 years and is characterized by spiking fever, arthralgia, evanescent salmon rash with other abnormalities including pharingitis, serositis (especially pleuritis and pericarditis) and leucocytosis as well as increased serum levels of inflammatory indicators. We present two patients with recurrent pericardial effusion in the course of AOSD.

Dominating Cause of Pulmonary Hypertension May Change Over Time-Diagnostic and Therapeutic Considerations in a Patient with Pulmonary Hypertension Due to Rheumatoid Arthritis with Lung Involvement.

Chronic lung diseases are one of the most frequent causes of pulmonary hypertension (PH). The diagnostic challenge is to differentiate PH due to chronic lung disease from pulmonary arterial hypertension (PAH) with coexisting chronic lung disease. Moreover, the dominating cause of PH may change over time, requiring the implementation of new diagnostic procedures and new treatment modalities. We present a 68-year-old female, initially diagnosed with PH in the course of interstitial lung disease, with restrictive impairment of lung function. Therapy with immunosuppressive drugs resulted in significant clinical, radiological and functional improvement. However, five years later, arthritis symptoms developed, with PH worsening, despite stable lung disease. The patient was diagnosed with PAH in the course of rheumatoid arthritis. The introduction of sildenafil resulted in marked clinical and hemodynamic responses. Long-term survival (eleven years from PH onset and five years from PAH confirmation) has been achieved, and the patient remains in good functional condition. As the differential diagnosis of PH in patients with lung diseases is complex, the cooperation of pulmonologists and cardiologists is mandatory to obtain therapeutic success.

Non-Tuberculous Mycobacteria in Respiratory Specimens of Patients with Obstructive Lung Diseases-Colonization or Disease?

Non-tuberculous mycobacteria (NTM) are increasingly a cause of human respiratory tract colonization and mycobacterial lung disease (NTM-LD), especially in patients with chronic lung diseases. The aim of the present study was to find the factors predictive of NTM-LD in patients with obstructive lung diseases and NTM respiratory isolates. A total of 839 isolates of NTM, obtained from 161 patients between 2010 and 2020 in a single pulmonary unit, have been retrospectively reviewed. Of these isolates, 73 concerned 36 patients with obstructive lung diseases (COPD-26, asthma-3, COPD/asthma overlap syndrome-7). NTM-LD was recognized according to the American Thoracic Society (ATS) and the Infectious Diseases Society of America (IDSA) criteria in 17 patients, colonization in 19. Lower BMI, elevated body temperature on admission, infiltrative/cavitary lesions on chest CT, and NTM species other than Mycobacterium gordonae were the significant predictors of NTM-LD recognition. Based on the above-mentioned predictive factors, an original scoring system was implemented. The diagnostic utility of the scoring system was higher than that of single parameters. We conclude that NTM-LD prediction in patients with obstructive lung diseases and positive respiratory isolates is difficult. A scoring system based on clinical, radiological and microbiological characteristics was capable of facilitating the differential diagnosis, but it needs further validation in a larger study group.