Venous Thromboembolic Disease in COVID-19, Pathophysiology, Therapy and Prophylaxis.

For over two years, the world has been facing the epidemiological and health challenge of the coronavirus disease 2019 (COVID-19) pandemic, caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Growing problems are also complications after the development of COVID-19 in the form of post and long- COVID syndromes, posing a challenge for the medical community, both for clinicians and the scientific world. SARS-CoV-2 infection is associated with an increased risk of cardiovascular complications, especially thromboembolic complications, which are associated with both thrombosis of small and very small vessels due to immunothrombosis, and the development of venous thromboembolism. Low molecular wight heparin (LMHW) are the basic agents used in the prevention and treatment of thromboembolic complications in COVID-19. There is still a great deal of controversy regarding both the prevention and treatment of thromboembolic complications, including the prophylaxis dose or the optimal duration of anticoagulant treatment in patients with an episode of venous thromboembolism.

Intrapericardial recombinant tissue plasminogen activator in purulent pericarditis- case series.

BACKGROUND: Pericardial constriction is one of the complications of purulent pericarditis (PP). Most difficult to treat, which may develop both in early and in the late period of the disease, resulting in a very poor prognosis. CASE PRESENTATION: We present case series of 4 patients with purulent pericarditis, in whom direct intrapericardial administration of recombinant tissue plasminogen activator (r-tPA) was used. Management of PP requires a combined surgical and medical approach. The most important is complete drainage of the effusion by subxiphoid pericardiotomy connected with complementary use of broad-spectrum antibiotics. Despite the use of broad- spectrum antibiotics, in some patients a large volume of daily drainage is still present. Constrictive pericarditis as a complication of PP is observed in majority of patients. Intrapericardial administration of fibrinolytic agents, although not strongly recommended, can improve efficacy of antibiotic treatment especially in patients with loculation fluid and can prevent the development of constrictive pericarditis. r-tPA was applied at a dose of 20 mg dissolved in 100 ml of normal saline in a 100 ml syringe, administered by a large pericardial drain (Pezzer drain) installed into the pericardial cavity during pericardioscopy. The tube was closed and re-opened after 24 h. No serious complications, such as bleeding, allergy or hypotension, were noted. CONCLUSION: We present case series of 4 patients with purulent pericarditis, in whom direct intrapericardial administration of recombinant tissue plasminogen activator (r-tPA), prevented the development of constrictive pericarditis, and increased efficacy of antibiotic treatment without any significant complications.

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.

Organizing pneumonia appearing in B-cell chronic leukemia malignancy progression - a case report.

Patients with chronic lymphocytic leukemia or non-Hodgkin's lymphoma are at risk of infectious diseases of respiratory system because of immunodeficiency. Occurrence of organizing pneumonia in leukemic patients is most commonly correlated with bone marrow transplant or treatment with antimitotic agents. There have been only four reported cases of organizing pneumonia related solitarily to leukemia or lymphoma. We present a case of 65-year old gentlemen, diagnosed 8 months earlier with B-cell chronic lymphocytic leukemia with no previous hematologic treatment, who presented symptoms of persistent pneumonia with no significant reaction to antibiotics. Chest computed tomography scans showed well-localized consolidation with ground glass opacities and some air bronchogram, suggesting infectious disease. All results of microbiological examinations were negative. Due to radiological progression of parenchymal consolidation despite two intravenous courses of antibiotics open lung biopsy was performed. The histologic examination of lung specimen revealed structures typical for organizing pneumonia pattern. There was no evidence for leukemic involvement in lung tissue, as no sign for infectious factors from histological staining was observed. In the inferior mediastinal lymph node sample progression of chronic lymphatic leukemia to mixed cell lymphoma was diagnosed. Patient was commenced on prednisone 60 mg/daily with fast improvement. We believe that this is the first case of organizing pneumonia as a reaction to the conversion of B-cell chronic lymphocytic leukemia progression to more malignant stage.

Atypical image of pulmonary alveolar proteinosis - a case report.

Pulmonary alveolar proteinosis is a very rare interstitial lung disease caused by abnormal intra-alveolar surfactant accumulation. Usually, it appears as a "crazy-paving" pattern on high-resolution computed tomography. The image is so typical, that together with the characteristic bronchoalveolar lavage examination with presence of Periodic Acid Schiff positive substance is sufficient for establishing diagnosis, without histological confirmation. We present the case of the young woman with severe dyspnoea suspected of acute hypersensitivity pneumonia. The computed tomography showed numerous intralobular nodules uniformly distributed troughout the lungs. Treatment by corticosteroids had no clinical effect and next computed tomography showed progression. Despite the high risk of complications (patient had a respiratory failure), a surgical lung biopsy was performed and the histopathological diagnosis of pulmonary alveolar proteinosis was made. The whole lung lavage procedure performed twice caused regression of radiological lesions and respiratory failure.

[Synchronous multiple primary lung cancers in a 65-year old heavy smoker. Case report]. / Synchroniczne mnogie pierwotne raki pluca u 65-letniego mezczyzny z wieloletnim wywiadem nikotynowym. Opis przypadku.

Here we present a 65-year old ex-smoker with history of recent surgery for vocal cord tumor (histology: moderate grade intraepithelial neoplasia), who reported to the pulmonary outpatient clinic for the nodular lesions in the left lung seen on chest X-ray. Subsequent chest CT scan revealed focal lesion of 18 mm in diameter with spicular margins located in the right upper lobe, another irregular cyst with septa, 62 × 58 mm in the right lower lobe, and calcified nodule in the left lung, no enlarged lymph nodes or pleural effusion was seen. He underwent upper right lobe resection and wedge resection of the lower right lobe. Histological examination revealed adenocarcinoma in the right upper lobe with lymph node metastasis (pT2aN2M0). Examination of the right lower lobe showed squamous cell carcinoma (pT2bN0M0). He was subsequently treated with adjuvant chemotherapy and radiotherapy. During 20 months of the follow-up, he remained in good health with no signs of the disease progression. Patients with synchronous multiple primary lung cancers have significantly less favorable outcome than those with single primary lung malignancies, although it can be considerably improved with radical surgical treatment. Basing on the above case report, we discussed diagnostic and therapeutical scheme in patients with the primary multiple lung cancers, and have analyzed epidemiological data and some aspects of MPM etiology.

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.

[Acute lupus pneumonitis--case report and literature review]. / Ostre toczniowe zapalenie pluc--opis przypadku i przeglad pismiennictwa.

Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease that is characterized by its chronic course and the involvement of many organs and systems. The most common abnormality in the respiratory system of SLE patients is lupus pleuritis. Less common is parenchymal involvement, which may present as acute lupus pneumonitis (ALP) or chronic interstitial lung disease. Other possible pulmonary manifestations of SLE include pulmonary embolism, diffuse alveolar haemorrhage, acute reversible hypoxaemia, and shrinking lung syndrome. We present the case report of a young woman with previously diagnosed membranous glomerulonephritis with nephrotic syndrome and antiphospholipid syndrome, who was admitted with marked of shortness of breath. The diagnostic process, including imaging studies and laboratory tests, enabled us to confirm a diagnosis of ALP. After initiation of treatment with high doses of methyloprednisolone, nearly complete remission of pulmonary changes was observed. We also perform a literature review regarding acute lupus pneumonitis.

[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).

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.

Imaging Plays a Key Role in the Diagnosis and Control of the Treatment of Bone Sarcoidosis.

Sarcoidosis is a multisystem granulomatous disease of unknown origin. The most frequent localizations are thoracic lymph nodes and/or parenchymal lung disease, nevertheless any other organ may be involved. Musculoskeletal sarcoidosis, previously considered a rare manifestation of the disease, is presently recognized with increasing frequency, due to the development of modern imaging modalities. The classical X-ray sign of bone sarcoidosis is the image of lace in the phalanges of the hands. Most other locations present with atypical radiological images. Therefore, they may mimic metastatic neoplastic disease, especially when they are the first sign of sarcoidosis not previously recognized. On such occasions, none of the imaging methods will give the correct diagnosis, histopathological verification, monitoring of lesions or clinical data in a patient with confirmed sarcoidosis are indicated. The article summarizes the current status of knowledge concerning the recognition and therapy of bone sarcoidosis. In addition, an illustrative case of patient with bone and bone marrow sarcoidosis is presented.

Nontuberculous Mycobacterial Lung Disease in the Patients with Cystic Fibrosis-A Challenging Diagnostic Problem.

BACKGROUND: Cystic fibrosis (CF) is an autosomal, recessive genetic disorder, caused by a mutation in the cystic fibrosis transmembrane conductance receptor regulator (CFTR) gene. Dysregulated mucous production, and decreased bronchial mucociliary clearance, results in increased susceptibility to bacterial and fungal infections. Recently, nontuberculous mycobacteria (NTM) infections were identified as an emerging clinical problem in CF patients. AIM: The aim of the present study was to assess the frequency of NTM isolations in CF patients hospitalized in the pulmonary department, serving as a hospital CF center, and to describe challenges concerning the recognition of NTMLD (nontuberculous mycobacterial lung disease) in those patients. METHODS: Consecutive CF patients, who were hospitalized due to pulmonary exacerbations (PEX), in a single CF center, between 2010 and 2020, were retrospectively assessed for the presence of NTM in respiratory specimens. Clinical and radiological data were retrospectively reviewed. RESULTS: Positive respiratory specimen cultures for NTM were obtained in 11 out of 151 patients (7%), mean age-35.7 years, mean BMI-20.2 kg/m2, mean FEV1-58.6% pred. Cultures and phenotyping revealed the presence of Mycobacterium avium (M. avium)-in six patients, Mycobacterium chimaera (M. chimaera) in two, Mycobacterium kansasii (M. kansasii)-in one, Mycobacterium abscessus (M. abscessus)-in one, Mycobacterium lentifavum (M. lentiflavum)-in one. Simultaneously, respiratory cultures were positive for fungi in 91% of patients: Candida albicans (C. albicans)-in 82%, Aspergillus fumigatus (A. fumigatus)-in 45%. Clinical signs of NTMLD were non-specific, chest CT indicated NTMLD in five patients only. CONCLUSION: Due to non-specific clinical presentation, frequent sputum cultures for NTM and analysis of serial chest CT examinations are crucial for NTMLD recognition in CF patients. Further studies concerning the predictive role of fungal pathogens for NTMLD development in CF patients are needed.

Acute Eosinophilic Pneumonia Complicated with Venous Thromboembolic Disease-Diagnostic and Therapeutic Considerations.

Acute Eosinophilic Pneumonia (AEP) is a rare idiopathic disease caused by an accumulation of eosinophils in the pulmonary alveoli and interstitial tissue of the lungs. The onset of symptoms is acute; some patients develop respiratory failure. The diagnosis is based on clinical symptoms, diffuse interstitial infiltrates in the lungs on imaging studies, and eosinophilia exceeding 25% on bronchoalveolar lavage or pleural fluid smear. Smokers are primarily at increased risk for the disease. We present a case of venous thromboembolic disease (VTE) that developed in the course of AEP in a previously healthy male smoker. Complete remission of the disease was achieved with anticoagulation therapy combined with a low dose of steroids. Surprisingly, further diagnostics revealed the presence of thrombophilia: antithrombin (AT) deficiency and increased homocysteine level. According to our knowledge, this is the first case of VTE diagnosed in the course of AEP combined with thrombophilia.

Novel folliculin gene mutations in Polish patients with Birt-Hogg-Dubé syndrome.

BACKGROUND: Birt-Hogg-Dubé syndrome (BHDS) is a rare, autosomal dominant, inherited disease caused by mutations in the folliculin gene (FLCN). The disease is characterised by skin lesions (fibrofolliculomas, trichodiscomas, acrochordons), pulmonary cysts with pneumothoraces and renal tumours. We present the features of Polish patients with BHDS. MATERIALS AND METHODS: The first case of BHDS in Poland was diagnosed in 2016. Since then, 15 cases from 10 families have been identified. Thirteen patients were confirmed via direct FLCN sequencing, and two according to their characteristic clinical and radiological presentations. RESULTS: BHDS was diagnosed in 15 cases (13 women and 2 men) from 10 families. The mean ages at the time of first pneumothorax and diagnosis were 38.4 ± 13.9 and 47.7 ± 13 years, respectively. Five patients (33%) were ex-smokers (2.1 ± 1.37 packyears), and 10 (67%) had never smoked cigarettes. Twelve patients (83%) had a history of recurrent symptomatic pneumothorax. Three patients had small, asymptomatic pneumothoraces, which were only detected upon computed tomography examination. All patients had multiple bilateral pulmonary cysts, distributed predominantly in the lower and middle, peripheral, and subpleural regions of the lungs. Generally, patients exhibited preserved lung function. Skin lesions were seen in four patients (27%), one patient had renal angiomyolipoma, and one had bilateral renal cancer. Different mutations of the FLCN gene were identified (mainly in exon 6), with two novel heterozygous variants: c.490delA p.(Arg164GlyTer13) and c.40delC p.(His14ThrsfTer41). CONCLUSIONS: All analysed patients with BHDS presented with lung lesions and with less frequent skin and renal lesions than previously reported in other populations. In addition, more frequent mutations located in exon 6 were detected, and two novel FLCN gene mutations were identified.

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.

[Recurrence of endobronchial lipoma]. / Nawrót tluszczaka wewnatrzoskrzelowego.

Benign tumors of the lung and endobronchial tree are uncommon. Endobronchial lipomas are extremely rare, with incidence ranging from 0.1 to 0.5% of all lung tumors. Endobronchial lipomas originate from fat cells located in the peribronchial and occasionally the submucosal tissue of the main bronchi. The paper presents a case, in whom four years after transbronchial resection of the endobronchial lipoma, recurrence of the lesion in the same lung was confirmed. The diagnosis was made on the basis of CT scan, which detected lesions with previously identified morphology.

Primary Sjögren's Syndrome with two extraglandular sites involvement - case report.

Primary Sjögren's Syndrome (pSS) is a chronic, slowly progressive inflammatory autoimmune disorder, characterised by lymphocytic infiltration of the exocrine glands, leading to decrease of glandular secretion. In 40-60% of pSS patients, extraglandular disease develops. We present the case of a patient with two extraglandular sites involvement in the course of pSS manifesting with progressive respiratory and gastrointestinal symptoms.

[Simultaneous detection of tumor cells and the positive result of genetic test for Mycobacterium tuberculosis in pleural effusion]. / Jednoczesne wykrycie komórek nowotworowych i materialu genetycznego pratków gruzlicy w plynie oplucnowym.

Pleural effusion is a frequently observed lesion in the course of respiratory diseases such as inflammatory process and cancer metastasis. Its cause may be either tuberculosis (the most common extrapulmonary location is the pleura) and malignant disease of the pleura. Confirmation of tuberculosis is often troublesome. The primary site of cancer may be also difficult to find despite the application of difficult diagnostic methods. Below we present history of 79-year-old female in whom carcinomatous cells and positive result of PCR for Mycobacterium tuberculosis in pleural fluid were discovered simultaneously suggesting the tuberculosis and cancer of unknown primary origin.

Mycobacterium szulgai Lung Disease or Breast Cancer Relapse-Case Report.

Cancers are one of the risk factors of non-tuberculous mycobacterial (NTM) lung disease. The majority of data in this group of patients concern infections caused by Mycobacterium avium-the most prevalent NTM species worldwide. In contrast, limited information can be found regarding the uncommon NTM such as Mycobacterium szulgai. We present the case of M. szulgai lung disease in a patient with a history of breast cancer. Coexistence of NTM lung disease and breast cancer lung metastasis as well as primary lung cancer was suspected. Finally, neoplastic disease was ruled out based on negative results of endobronchial biopsy and negative tumor markers for lung and breast cancer. M. szulgai lung disease was successfully treated with rifampicin, ethambutol and clarithromycin.

SARS-CoV-2 lung disease in a patient with pulmonary sarcoidosis - case report.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is the recently identified cause of the current pandemic. In patients with chronic respiratory lung diseases, SARS-CoV-2 may result in significant morbidity and increased mortality. We present a case of a 69-year-old male with stage II pulmonary sarcoidosis who had been under observation for 30 months without immunosuppressive treatment. He then developed severe SARS-CoV-2 disease with typical radiological and laboratory findings. Therapy with oxygen, antibiotics, low-molecular-weight heparin in a prophylactic dose, and dexamethasone resulted in marked clinical improvement. We will discuss the rationale for corticosteroid use in both SARS-CoV-2 disease and in SARS-CoV-2 disease that is complicating comorbid sarcoidosis.