Factors Predictive for Immunomodulatory Therapy Response and Survival in Patients with Hypersensitivity Pneumonitis-Retrospective Cohort Analysis.

Hypersensitivity pneumonitis (HP) is one of the interstitial lung diseases with clearly established diagnostic criteria. Nevertheless, pharmacologic treatment recommendations are still lacking. Most specialists use steroids as first-line drugs, sometimes combined with an immunosuppressive agent. Aim: The aim of the present retrospective study was to establish predictive factors for treatment success and survival advantage in HP patients. Methods: We analyzed the short-term treatment outcome and overall survival in consecutive HP patients treated with prednisone alone or combined with azathioprine. Results: The study group consisted of 93 HP patients, 54 (58%) with fibrotic HP and 39 (42%) with non-fibrotic HP. Mean (± SD) VCmax % pred. and TL,co % pred. before treatment initiation were 81.5 (±20.8)% and 48.3 (±15.7)%, respectively. Mean relative VCmax and TL,co change after 3−6 months of therapy were 9.5 (±18.8)% and 21.4 (±35.2)%, respectively. The short-term treatment outcomes were improvement in 49 (53%) patients, stabilization in 16 (17%) patients, and progression in 28 (30%) patients. Among those with fibrotic HP, improvement was noted in 19 (35%) cases. Significant positive treatment outcome predictors were fever after antigen exposure, lymphocyte count in broncho-alveolar lavage fluid (BALF) exceeding 54%, RV/TLC > 120% pred., and ill-defined centrilobular nodules in high-resolution computed tomography (HRCT). An increased eosinophil count in BALF and fibrosis in HRCT were significant negative treatment outcome predictors. The presence of fibrosis in HRCT remained significant in a multivariate analysis. A positive response to treatment, as well as preserved baseline VCmax (% pred.) and TLC (% pred.), predicted longer survival, while fibrosis in HRCT was related to a worse prognosis. Conclusion: Immunomodulatory treatment may be effective in a significant proportion of patients with HP, including those with fibrotic changes in HRCT. Therefore, future trials are urgently needed to establish the role of immunosuppressive treatment in fibrotic HP.

Tuberculosis diagnosed by thoracic surgeons.

INTRODUCTION: Tuberculosis is frequently omitted in the diagnostic workup and may be identified accidentally following thoracic surgeries, mostly those targeting lung cancer. OBJECTIVES: This study aimed to assess the clinical characteristics of patients who underwent thoracic surgery that resulted in the diagnosis of tuberculosis and to review lesions initially found on chest imaging in the context of the potential presence of tuberculosis. PATIENTS AND METHODS: We analyzed medical records of all patients hospitalized at the Department of Thoracic Surgery of the National Tuberculosis and Lung Diseases Research Institute, Warsaw, Poland, between the years 2014 and 2018 (n = 57) in whom tuberculosis was diagnosed. Two radiologists who knew the diagnosis retrospectively analyzed preprocedural chest computed tomography scans of the study patients. RESULTS: Tuberculosis was diagnosed by culture of specimens obtained during video­ assisted thoracoscopy (21 patients), thoracotomy (24 patients), mediastinoscopy (6 patients), transthoracic fine­ needle biopsy (3 patients), and transbronchial biopsy (1 patient). In the remaining 2 individuals, the diagnosis was established based on the microbiological examination of drained pleural fluid. In 42 patients (73.7%), the diagnosis of tuberculosis was unexpected to thoracic surgeons. Radiological findings suggestive of tuberculosis were present in 38 patients (66.7%). The radiologists who retrospectively analyzed the imaging records suggested tuberculosis in 31 persons (54.3%), whereas those who carried out the initial preprocedural evaluation, in 11 (19.3%). CONCLUSIONS: The majority of the study patients presented with radiological findings encountered in tuberculosis, which should have led to a less invasive diagnostic workup. This highlights the role of radiologists in the identification of the disease.

Guidelines of the Polish Respiratory Society for diagnosis and treatment of idiopathic pulmonary fibrosis.

INTRODUCTION: This document presents the guidelines of the Polish Respiratory Society (PTChP, Polskie Towarzystwo Chorób Pluc) for diagnosis and treatment of idiopathic pulmonary fibrosis (IPF), developed by agroup of Polish experts. MATERIAL AND METHODS: The recommendations were developed in the form of answers to previously formulated questions concer-ning everyday diagnostic and therapeutic challenges. They were developed based on acurrent literature review using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. RESULTS: We formulated 28 recommendations for diagnosis (8), pharmacological treatment (12) as well as non-pharma-cological and palliative therapy (8). The experts suggest that surgical lung biopsy (SLB) not be performed in patients with the probable usual interstitial pneumonia (UIP) pattern, with an appropriate clinical context and unanimous opinion of a multidisciplinary team. The experts recommend using antifibrotic agents in IPF patients and suggest their use irrespective of the degree of functional impairment. As regards non-pharmacological and palliative treatment, strong re-commendations were formulated regarding pulmonary rehabilitation, oxygen therapy (in patients with chronic respiratory failure), preventive vaccinations as well as referring IPF patients to transplant centres. Table 1 presents an aggregate list of recommendations. CONCLUSIONS: The Polish Respiratory Society Working Group developed guidelines for IPF diagnosis and treatment.

Hypersensitivity pneumonitis recognised in a single pulmonary unit, between 2005 and 2015 - comparison with recently proposed diagnostic criteria.

INTRODUCTION: Hypersensitivity pneumonitis (HP) is the third most common interstitial lung disease after idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Pathogenesis of HP is related to repeated exposure to inhaled environmental antigens that sensitise the susceptible, genetically predisposed persons. The aim of the present retrospective study was to summarise the diagnostic methods used in consecutive patients with HP, recognised in a single pulmonary unit, between 2005 and 2015, and to compare them with current diagnostic criteria. MATERIAL AND METHODS: 135 patients, 68 males, 67 females, median age 53 years (18-75 years), entered the study. Chest CT features characteristic of HP were defined as: mosaic attenuation of lung parenchyma, air trapping and/or ill-defined centrilobular nodules. Lymphocytosis in BAL was defined as ≥ 30%. RESULTS: Median time from first symptoms to diagnosis was 12 months. The exposure to one or more allergens was found in 94% of patients, chest CT features characteristic of HP have been reported in 87%, BAL lymphocytosis - in 86%. According to recent diagnostic criteria - in 54% of patients, clinical diagnosis of HP was confident, in 16% - probable, in 26% - possible and in 4% - unlikely. The confirmation of HP with lung biopsy has been obtained in 36% of non-confident cases (16% of the study group). CONCLUSION: HP diagnosis was confirmed according to current diagnostic criteria in 70% of patients diagnosed between 2005 and 2015. Contradictions to lung biopsy have been the main reason for inability to confirm HP in non-confident cases.

CT imaging features of thin-walled cavitary squamous cell lung cancer.

Primary lung cancer manifesting as a thin-walled solitary cavity, occurs relatively infrequently. The most common histologic type presenting such a pattern is squamous cell cancer, followed by adenocarcinoma, and finally - large cell cancer. Cavitation is typically not seen in small cell lung cancer. Entities indicating malignancy of such lesions include irregular cystic wall, wall nodule formation, nodular septa or increased standard uptake on positron emission tomography (PET). We are presenting a case of a squamous cell lung cancer manifesting on chest CT as a thin-walled septated cavity with irregular margins mimicking a cyst. The lesion was reported unchanged in a follow-up computed tomography after 3 months. A follow-up scan obtained 2 years after initial examination showed thickening of a cyst wall, solid structures within its lumen and thoracic lymph nodes enlargement.

Familial pneumothoraces - Birt-Hogg-Dubé syndrome. Differentiation with other cystic lung diseases.

Birt-Hogg-Dubé syndrome (BHDS) is a rare, genetic, autosomal dominant disease caused by mutation in a folliculin gene. This syndrome is characterised by three main symptoms: benign lesions originating from hair follicles, variously shaped cysts in the lungs, and various types of benign and malignant kidney neoplasms. In our article we are going to present cases of two sisters with BHDS. In the case of the first sister skin lesions were accompanied by lung abnormalities. The second sister, however, presented with recurrent pneumothoraces associated with variously shaped lung cysts located mainly below the tracheal carina. In both instance diagnosis was confirmed by genetic test.

Lung Function in Pregnancy in Langerhans Cell Histiocytosis.

Pulmonary Langerhans cell histiocytosis (LCH) is a rare disease, affecting usually young people. The course of the disease is variable. In some pulmonary LCH patients a severe lung destruction and progression in spite of chemotherapy is observed, but in others just a cessation of smoking induces a regression of the disease. In the present study we seek to determine the influence of pregnancy on pulmonary function in LCH patients, an unchartered area of research. We addressed the issue by investigating eight pregnant women out of the 45 women hospitalized with the diagnosis of pulmonary LCH in the period from 2000 to 2015. For five of the eight pregnant women it was the second gestation. The median follow-up period was 120 months (range 72-175 months). Ten healthy children were born by a C-section. Two spontaneous miscarriages in the seventh week of gestation, and one tubal ectopic pregnancy were recorded. We found that pregnancy did not significantly influence pulmonary function assessed by the following indices: forced expiratory volume in 1 s (FEV1), lung vital capacity (VC), total lung capacity (TLC), residual volume (RV), diffusing capacity of the lungs for carbon monoxide (DLCO), and the distance and arterial oxygen saturation in 6-min walk test. Only one patient in the third trimester of pregnancy experienced bilateral pneumothorax, with persistent air leak. In all patients, delivery and postpartum period were uneventful. We conclude that pregnancy in pulmonary LCH patients is safe and not associated with deterioration of pulmonary function or blood oxygenation.

Solitary pulmonary nodule - the role of imaging in the diagnostic process.

A solitary pulmonary nodule is a round opacity less than 30 mm in diameter surrounded by normally aerated lung tissue. Determination of the character of the lesion following its detection (particularly when the identification was incidental) may require a complex diagnostic process. In most cases, nodules are benign in character; however, the probability of malignancy increases significantly for part-solid lesions. The main features that describe the solitary pulmonary nodule in computed tomography scans include their size, shape, density, presence of calcification and rate of growth. PET-CT examination provides additional information on the metabolic activity of the lesions, and MRI is helpful in assessment of local invasion of surrounding structures. Due to limited availability and highly specialized character, these examinations are not routinely used. Therefore, despite development of other imaging modalities, computed tomography remains the most important and crucial diagnostic tool. Clinical risk factors such as age or smoking status are very important for evaluation of the likelihood of malignancy of a nodular lesion. Due to the multidisciplinary nature of data required for complex assessment of a solitary nodular lesion, management routines are needed in the diagnostic process such as those proposed by the Fleischner Society.

Cryptogenic organizing pneumonia-Results of treatment with clarithromycin versus corticosteroids-Observational study.

BACKGROUND: Cryptogenic organizing pneumonia (COP) is a clinicopathological syndrome of unknown origin. Corticosteroids are the standard treatment, but clarithromycin (CAM) is also effective. The aim of this observational retrospective study was to compare the results of CAM versus prednisone (PRE) treatment in patients with biopsy-proven OP without respiratory insufficiency. MATERIAL AND METHODS: In a 15-year period, 40 patients were treated with CAM (500 mg twice daily orally for 3 months) and 22 with PRE (mean initial dose of 0.67 ± 0.24 mg/kg/d for a mean of 8.59 ± 3.05 months). RESULTS: The clinical presentation, laboratory, and radiological findings did not differ markedly between patients treated with CAM and PRE, with the exception of a higher frequency of sweats (55% vs. 23%; p < 0.015), ground glass opacities (95% vs. 50%; p <0.0001) and nodular lesions (45% vs. 18%; p = 0.036) in the CAM group. A complete response was achieved in 35(88%) patients treated with CAM and in all treated with PRE. Patients treated with PRE relapsed more frequently than those treated with CAM (54.5% vs. 10%; p < 0.0001). Corticosteroid-related adverse events were noticed in 8(6.5%) patients (with one death), but CAM caused only one (2.5%) allergic reaction. A FVC >80% identified patients who might be successfully treated with CAM with a sensitivity of 60% and a specificity of 88.57% (AUC 0.869; 95% CI 0.684-1; p = 0.008); the figures for the FEV1 were >70%, a sensitivity of 60%, and a specificity of 91.43% (AUC 0.809; 95%CI 0.609-1; p = 0.027). CONCLUSIONS: CAM can be used to treat COP patients in whom the pulmonary function parameters are within normal limits. Such therapy is shorter, better tolerated, and associated with fewer adverse events and relapses than is PRE. However, the therapy is ineffective in some patients.

Centrilobular nodules in high resolution computed tomography of the lung in IPAH patients - preliminary data concerning clinico-radiological correlates.

INTRODUCTION: Inhomogeneity of lung attenuation pattern is observed in high resolution chest computed tomography (HRCT) in some IPAH patients despite lack of interstitial lung disease. Such radiological changes are described either as ill-defined centrilobular nodules (CN) or as focal ground glass opacities (FGGO). There is no consensus in the literature, whether they indicate the distinct type of IPAH, or pulmonary venoocclusive disease (PVOD) with subtle radiological changes. Thus the aim of the present pilot study was to assess the frequency and clinical significance of inhomogenic lung attenuation pattern in IPAH. MATERIAL AND METHODS: 52 IPAH patients (38 females, 14 males, mean age 41 years ± 15 years), entered the study. All available chest CT scans were reviewed retrospectively by the experienced radiologist, not aware about the clinical data of the patients. RESULTS: CN were found in 10 patients (19%), FGGO - in 12 patients (23%). No lymphadenopathy or interlobular septal thickening suggestive of PVOD were found. The significant differences between CN and the remaining patients included: lower mean age - 31 and 43.5 years, (p = 0.02), lack of persistent foramen ovale (PFO) - 0% and 43% (p = 0.03), and higher mean right atrial pressure (mRAP) - 12.5 mm Hg and 7.94 mm Hg (p = 0.01). No significant survival differences were observed between the groups of CN, FGGO and the remaining patients. CONCLUSION: Centrilobular nodules in IPAH were combined with lack of PFO, higher mRAP and younger age of patients.

[Hypersensitivity pneumonitis: Diagnostic criteria, treatment, prognosis and prevention]. / Alergiczne zapalenie pecherzyków plucnych - kryteria rozpoznania, leczenie, rokowanie i zapobieganie.

Hypersensitivity pneumonitis (HP) is caused by inhalation of environmental antigens. Farmers and bird keepers are most frequently affected by this desease. The HP diagnosis is based on clinical symptoms (cough, dyspnea) in a person exposed to environmental antigens, and the presence of characteristic changes in high resolution chest computed tomography (HRCT) (bilateral, mosaic, ground glass opacities in the middle and lower lung zones, ill-defined centrilobular nodules and the sign of air-trapping on expiration). This type of HRCT pattern is most frequently found in the patients with subacute HP. Bronchioloalveolar lavage fluid (BALF) examination is helpful in establishing the HP diagnosis, when the increased total number of cells, with the predominance of T lymphocytes (> 50%), and the increased number of neutrophils (> 3%) and mastocytes (> 1%) are found. The presence of specific serum precipitins increases the likelihood of HP. In case of atypical clinical presentation, lung biopsy is recommended. The diagnostic criterion of HP is the presence of ill-defined non-necrotising granulomas, after excluding other granulomatous lung diseases. The prevention and treatment of HP is based on the elimination of the antigen from the environment. Corticosteroids may contribute to the improvement in the acute and sub-acute form of the disease but their long term effectiveness is uncertain. The prognosis of HP patients is generally perceived as good, especially in those patients in whom antigen avoidance is possible. Nevertheless, in some patients progressive pulmonary fibrosis and development of severe respiratory insufficiency is observed. Med Pr 2016;67(4):517-527.

Methotrexate as a single agent for treating pulmonary sarcoidosis: a single centre real-life prospective study.

INTRODUCTION: The first-line therapy in chronic sarcoidosis, according to WASOG/ATS/ERS recommendations, is GCS. This therapy is associated with significant adverse effects and finally does not alter the natural history of the disease. The objective of our study was to evaluate the efficacy and safety of monotherapy with MTX, as an alternative to GCS, in progressive pulmonary sarcoidosis. MATERIAL AND METHODS: An open prospective real-life, single-centre trial was performed on 50 patients with biopsy proven sarcoidosis, 28M and 22F, mean age 45.55 ± 8.9 years. The average duration of disease before MTX therapy was 12.34 ± 20.49 years, GCS therapy in the past was applied in 41 patients. All patients received MTX (10 mg or 15 mg weekly) between 2004 and 2013 because of chronic progressive pulmonary sarcoidosis. Therapy was planned for 24 months. Patients underwent regular clinical evaluation, pulmonary function assessment, exercise ability testing (6MWT), and chest radiography for therapy effectiveness every six months and side effects monitoring every 4-6 weeks. Forty-nine patients were included for statistical analysis of treatment efficacy. They were retrospectively allocated to "MTX responder" group if an improvement of 10% of FEV1, FVC, TLC, or 15% of DLCO from the initial value was documented for at least one parameter or "non-responders" if the patient did not meet the above-mentioned criteria. RESULTS: Duration of treatment ranged from 6 to 24 months, mean time 60.75 ± 34.1 weeks. For the whole cohort significant improvement after MTX therapy was observed for minimal SaO2 (%) (p = 0.043) and for decrease of DSaO2 (%) (p = 0.048) in six-minute walk test. The results were significantly better for patients treated with 15 mg than for those treated with 10 mg weekly and for those who obtained a greater total amount of MTX during therapy. Significant difference of DLCO%pred was observed after six months of MTX therapy between groups treated 15 mg vs 10 mg weekly (73.27 ± 12.7% vs. 63.15 ± 16.4%, p = 0.03). Twenty-five patients (55%) met the criteria of "MTX responders" group. Patients who responded well to treatment had significantly lower TLC and FVC initial values comparing to "MTX non-responders". After treatment the only significant difference in PFT between groups was noted for DLCO%pred. Eleven patients (22%) stopped the treatment due to adverse events of MTX, mild hepatic abnormalities were observed in ten patients (20%), and concomitant infection was found in four patients. There were no patients with a fatal outcome. CONCLUSIONS: MTX as a single agent in the treatment of sarcoidosis has proved to be a safe and effective steroid alternative. Selected patients with chronic pulmonary sarcoidosis experience definite PFT improvements after MTX treatment. There is need to search for predictors of MTX treatment effectiveness.

Prognostic value of serum C-reactive protein (CRP) and cytokeratin 19 fragments (Cyfra 21-1) but not carcinoembryonic antigen (CEA) in surgically treated patients with non-small cell lung cancer.

INTRODUCTION: The aim of the study was to assess the prognostic value of cytokeratin 19 fragments (Cyfra 21-1), carcinoembryonic antigen (CEA) and C-reactive protein (CRP) in surgically treated NSCLC patients. MATERIAL AND METHODS: 50 NSCLC patients (25 adenocarcinoma, 21 squamous cell and 4 adenosquamous), clinical stages I and II, age 42-89 years, entered the study. CEA, Cyfra 21-1 and CRP concentrations were measured in serum taken before surgery, CEA and Cyfra 21-1 in 50 patients, CRP - in 46 patients. The survival was calculated from the date of surgical treatment until death or until the end of the observation time. The results were expressed as medians (95%CI). RESULTS: Cyfra 21-1 concentration was 2.1 (0.7-14.5) ng/mL. Survival time in the patients with Cyfra 21-1 ≤ 2 ng/mL, and > 2 ng/ /mL was 79 (14.85-88.2) and 29 (5.7-87.6) months, (p < 0.026). CEA concentration was 2.68 (0.87-72.7) ng/mL, significantly higher in adenocarcinoma than in squamous cell lung cancer - 4.38 ng/mL (1.67-41.35) vs. 2.2 ng/mL (1.0-6.1), p = 0.002. CRP concentration was 5.45 (0-122.6) mg/L. Significant dependence was found between CRP and pathological tumour size (pT). Median CRP values in pT1, pT2 and pT3+4 tumours were: 2.8 mg/L, 6.9 mg/L and 23.5 mg/L, respectively. Survival time of the patients with CRP ≤ 10 mg/L and CRP > 10 mg/L was 79 (14.85-88.2) and 29.5 (5.7-87.6) months, respectively (p = 0.045). CRP > 10 mg/L and Cyfra 21-1 > 2 ng/mL were the only significant preoperative prognostic indicators (HR 2.08 and 2.04, respectively). Among the postoperative parameters, pathological stage of disease (p-stage) and pT were the significant prognostic indicators (HR 2.1 and 2.42, respectively). CONCLUSIONS: In the present study, concerning surgically treated NSCLC patients, preoperative CRP > 10 mg/L and Cyfra 21-1 > 2 ng/mL were the only negative prognostic indicators, while pT and p-stage were significant postoperative prognostic indicators.

Pulmonary pathology in patients with ulcerative colitis treated with mesalazine--a challenging and complex diagnostic problem. Case series and literature review.

Pulmonary involvement in the course of inflammatory bowel disease has been a subject of interest to clinicians for long time, but despite this, its epidemiology and potential pathomechanisms remain obscured. Equally unclear is the role of medications used for bowel disease treatment in lung disease development. We present three patients with ulcerative colitis, all treated with mesalazine, in whom unexplained lung disease developed. Due to different clinical and radiological presentation, different conditions were initially placed on the top of the differential list in each of them. The outcome was favourable in all patients despite differences in management. We compared our patients with similar cases from literature. We show the level of difficulty and complexity in the issue of lung disease in patients with inflammatory bowel disease.

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.

Pulmonary alveolar proteinosis during a 30-year observation. Diagnosis and treatment.

INTRODUCTION: Pulmonary alveolar proteinosis (PAP) is a rare disease characterised by the abnormal accumulation of surfactant-like material in macrophages within the alveolar spaces and distal bronchioles. The course of the disease is variable and the prognosis is often good. However, progressive disease in some patients can cause respiratory dysfunction and can be life threatening. In this situation, the only effective treatment is whole lung lavage. The objective of the study was to present the characteristics and the course of pulmonary alveolar proteinosis in our own material, the diagnostic methods used, the indications for treatment and the treatment efficacy. MATERIAL AND METHODS: Retrospective analysis included 17 patients: 6 women and 11 men, aged from 32 to 56 years, who were observed in the Third Lung Department of Pneumonology at the National Institute of Tuberculosis and Lung Diseases between 1984 and 2013. In all patients chest X-ray, pulmonary function test and blood gases were performed. In 15 patients, high-resolution computed tomography (HRCT) was obtained. Bronchoscopy was performed in all of the patients, and in 7/17, bronchoalveolar lavage (BAL) was carried out. Fourteen patients underwent open lung biopsy. The indications for whole lung lavage (WLL) were progression of dyspnoea with restriction of daily activity and/or hypoxaemia. RESULTS: In most of the patients (13/17) the diagnosis was established outside our institute. Patients were referred to our department to establish further procedures. The criteria of diagnosis of PAP in most patients (16/17) was the histological examination of lung tissue, obtained by open lung biopsy (14 cases) and transbronchial lung biopsy (TBLB) (2 cases). Only in one patient the diagnosis was established on the basis of BAL. HRCT imaging was characteristic of proteinosis in 11/15 patients, and BAL examination in 6/7 patients, in whom BAL was performed. In four patients, who had been exposed to injurious factors for many years, secondary proteinosis was recognised; in other patients, no exposure or no other disease was found, and primary alveolar proteinosis was diagnosed. In one patient granulocyte macrophage colony stimulating factor autoantibody was detected. The majority of patients (10/17) had clinical symptoms at the diagnosis. The most commonly reported was dyspnoea, followed by respiratory tract infections. The most common abnormality (12/17) in pulmonary lung test was a decrease of diffusing capacity of the lung for carbon monoxide (DLCO). Respiratory distress at rest was found in two patients. Patients were observed for the period of 6 months to 19 years. Spontaneous partial remission was observed in 10 out of 13 untreated patients, including one complete remission; in 3 cases stabilisation was found in radiological examinations; and in other 4 patients, whole lung lavagewas used, resulting in clinical improvement with partial resolution of lesions in radiological examinations in 3 patients. In one patient, despite WLL being repeated three times, improvement was not achieved. CONCLUSIONS: Pulmonary alveolar proteinosis is a rare interstitial disease with a mild course in most cases. In 13/17 patients diagnosis was based on histological examination of samples from open lung biopsy. The presented patients were observed in the years 1984-2004, and at that time histologic examination was the main diagnostic method. The most common abnormality in pulmonary function tests was decrease of DLCO. In most cases, spontaneous remission of the disease was observed. In four patients with severe course of PAP, WLL was performed with subjective, functional and radiological improvement in 3 of them.

The application of MDCT in the diagnosis of chest trauma.

INTRODUCTION: Traumas are the third most common cause of death worldwide, after cardiovascular diseases and neoplasms, and the main cause of death of patients under 40 years of age. Contemporary image diagnosis of chest trauma uses chest X-ray (CXR), multidetector computed tomography (MDCT), transthoracic and transoesophageal ultrasound (USG), X-ray angiography and magnetic resonance. The aim of the present study was to evaluate MDCT results in the examination of posttraumatic chest injuries and to compare the results of CXR and MDCT in chosen chest traumatic injuries. MATERIAL AND METHODS: The sixty patients with chest trauma included in the study were diagnosed at the Department of Radiology of the Institute of Tuberculosis and Lung Diseases between May 2004 and October 2007. MDCT was performed in all patients. Two groups with different types of injury (blunt or penetrating chest trauma) were distinguished. The analysis of injuries in both groups was conducted depending on the mechanism of trauma. The detection of 20 selected injuries at CXR and MDCT was compared. Moreover, the compatibility of MDCT with the results of intraoperative assessment and bronchoscopy was analysed. The influence of MDCT on the treatment modality was also assessed. RESULTS: History of blunt chest trauma was found in 51 patients (group 1) and of penetrating trauma in 9 patients (group 2). The most frequent injuries among group 1 were lung contusion and rib fractures, and among group 2 it was pericardial hematoma. Compared to MDCT, the sensitivity and specificity of CXR were 66.7 and 58%, respectively. Change of treatment modality was observed after MDCT in 83% of patients. The sensitivity and specificity of MDCT in diagnosing tracheobronchial injury, compared to bronchoscopy, were 72.7% and 100%, respectively. Compatibility of MDCT results and intraoperative assessment was observed in 43% of patients, and the main reason for discrepancy was underdiagnosis of diaphragm injury in MDCT. CONCLUSIONS: MDCT was a valuable diagnostic method in recognition of chest trauma, characterized by high sensitivity and specificity in the assessment of life-threatening injures and for depicting tracheal and bronchial injuries. The diagnostic value of CXR was low. The compatibility of MCTD and intraoperative assessment was confirmed, with the exception of diaphragm injures and lung laceration. Change of treatment modality was certified after MDCT in 83% of patients.

Fatal clinical outcome in a patient with sarcoidosis-lymphoma syndrome.

A 62-year-old female suspected of malignant disease underwent a splenectomy that revealed noncaseating granulomas in the histological specimen. Chest X-ray (CXR) and lung CT scans suggested sarcoidosis stage II. TBLB showed noncaseating granulomas. A diagnosis of sarcoidosis was made. Initially no treatment was needed as partial remission on CXR and normal lung function were observed. During the follow up she underwent open lung biopsy and axillary lymph node biopsy because of radiological progression with presence of CXR opacities imitating metastases and recurrent lymphadenopathy. No malignant cells were found. Spontaneous partial resolution of disseminated changes on the CXR was observed. Because of progressive deterioration in lung function and the clinical course of the disease strongly suggesting the progression of systemic sarcoidosis, the patient was given steroid treatment, which initially resulted in partial remission of pulmonary disseminated changes, peripheral lymphadenopathy and improvement in lung function test. Eight months later severe deterioration in general condition, anaemia, leukocytosis, hypoxemia, massive hepatomegaly and recurrence of general lymphadenopathy along with progression of disseminated changes were found. She died before the final diagnosis was established. Post-mortem examination showed a nodal marginal zone B-cell lymphoma with monocytoid B-cells, according to WHO classification. The malignant cells were found in the jugular, mediastinal, paratracheal, paragastric, paraintestinal and retroperitoneal lymph nodes and they infiltrated the lungs, pleura, liver, thyroid gland and pancreas. No sarcoid granulomas were found in the autopsy.

Assessment of recurrence of non-small cell lung cancer after therapy using CT and Integrated PET/CT.

INTRODUCTION: Non-small cell lung cancer (NSCLC) has become the leading cause of cancer-related deaths in Poland. Follow-up of patients with NSCLC is aimed at early detection of local recurrence, metastatic process, treatment-related complications or second primary lung cancer. We investigated the diagnostic accuracy of FDG-PET-CT in the detection of recurrence of NSCLC after treatment. MATERIAL AND METHODS: Seventy-two NSCLC patients (19 females, 56 males), stage I to IV, who had undergone surgery and/ /or radiation therapy, occasionally associated with chemotherapy, were retrospectively included in our study. Chest radiographs and thoracic computed tomography (CT) were performed to localize the abnormality prior to PET-CT. All the patients underwent CT and PET-CT in the period from January 2008 until January 2012. All PET images were interpreted in conjunction with thoracic CT. PET-CT and CT diagnoses were correlated with pathological diagnoses. RESULTS: Forty-five patients had recurrent tumour. Tumour recurrence was observed more often in men than in women and also in case of neoplastic cell emboli in lymphatic or blood vessels. In three patients second primary lung cancer was diagnosed. False positive diagnosis of relapse based on PET-CT was obtained in 4 patients, mainly due to inflammatory lesions. The accuracy of PET-CT for diagnosis of recurrence was 94.4% (95% CI 91; 100). CONCLUSIONS: FDG PET-CT was the best method to differentiate recurrent bronchogenic carcinoma from inflammatory lesions, especially at post-therapeutic sites. It has been shown that PET-CT is more accurate method than CT in recurrent NSCLC. PET-CT results had a further impact on the clinical management and treatment planning.