Current Issue : April - June Volume : 2020 Issue Number : 2 Articles : 6 Articles
Background: Eosinophilic lung diseases represent a heterogeneous group of disorders with prominent infiltrate of\neosinophils in lung interstitium and alveolar spaces. Peripheral blood eosinophilia is often present. Infections, drugs,\nallergens, toxic agents have to be evaluated as possible causes of eosinophilic lung infiltrates. The category of\nmyeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB, FGFR1 and PCM1-JAK2\nrepresents an uncommon cause of eosinophilic lung infiltrate.\nCase presentation: We report the case of a 70-year old man complaining of dry cough and dyspnea. Ground\nglass-opacities were seen on imaging studies and peripheral blood eosinophilia was present. A thorough step-wise\npatientâ??s evaluation led to identify the clonal nature of eosinophilia and the diagnosis of myeloid/lymphoid\nneoplasm with eosinophilia and rearrangement of PDGFRA was made.\nConclusions: Correlation with clinical history, laboratory tests and imaging studies is essential to achieve the\ncorrect diagnosis when facing with eosinophilic lung infiltrates. A prolonged eosinophilia can cause life-threatening\norgan damage. Identification of PDGFRA rearrangement, as in the present case, is particularly critical given the\nsensitivity and excellent response to imatinib, which has completely changed the natural history of this neoplasm....
Background: Acute exacerbation (AE) is the major cause of morbidity and mortality in patients with idiopathic\npulmonary fibrosis (IPF). AEs also occur in other forms of fibrosing interstitial lung disease (fILD). The clinical features\nand prognosis of AE patients with connective tissue diseases (CTDs) associated-ILD has not been fully described.\nMethods: We retrospectively reviewed 177 patients with either IPF or a characterized CTD-ILD admitted to Nanjing\nDrum Tower Hospital with an AE from January 2010 to December 2016.\nResults: The study cohort included 107 subjects with AE-IPF and 70 cases with AE-CTD-ILD. Female gender, prior\nuse of corticosteroid and immunosupressants, lower serum albumin, higher D-dimer level, TLC% pred, survival, and\ntreatment with immunosupressants and caspofungin were more common in the CTD-ILD group (all p<0.05). The\nincidences of AE-CTD-ILD and AE-IPF were similar in our single center (p = 0.526). TLC% pred was the risk factor for\nAE after ILD diagnosis for 1 year in CTD patients (p = 0.018). Log-rank tests showed patients with CTD-ILD had a\nsignificantly lower mortality rate compared with IPF patients after AEs (p = 0.029). No significant difference in\nsurvival was noted among CTD subgroups (p = 0.353). The survival was negatively correlated with WBC count, LDH\nand CT score, (p = 0.006, p = 0.013 and p = 0.035, respectively), and positively correlated with PaO2/FiO2 ratio (p<\n0.001) in the CTD-ILD group. WBC count and PO2/FiO2 ratio were the independent predictors for survival in AECTD-\nILD after adjusting for other clinical variates in Cox regression Models (p = 0.038 and p < 0.001, respectively).\nConclusions: The clinical characteristics of patients with AE-CTD-ILD differed from those with AE-IPF, while AE\nincidences were similar between the two groups. Subjects with AE-CTD-fILD tended to have a better prognosis,\nand WBC count and PO2/FiO2 ratio were the independent survival predictors for these patients....
Background: Malignant central airway obstruction (CAO) occurs in approximately 20â??30% of patients with lung\ncancer and is associated with debilitating symptoms and poor prognosis. Multimodality therapeutic bronchoscopy\ncan relieve malignant CAO, though carries risk. Evidence to guide clinicians regarding which patients may benefit\nfrom such interventions is sparse. We aimed to assess the clinical and radiographic predictors associated with\ntherapeutic bronchoscopy success in relieving malignant CAO.\nMethods: We reviewed all cases of therapeutic bronchoscopy performed for malignant CAO at our institution from\nJanuary 2010â??February 2017. Therapeutic bronchoscopy success was defined as establishing airway patency......................
Background: Decades of clinical research into pulsed oxygen delivery has shown variable efficacy between users,\nand across a userâ??s behaviours (sleep, rest, activity). Modern portable oxygen concentrators (POCs) have been\nshown as effective as other oxygen delivery devices in many circumstances. However, there are concerns that they\nare not effective during sleep when the breathing is shallow, and at very high respiratory rates as during physical\nexertion. It can be challenging to examine the determinants of POC efficacy clinically due to the heterogeneity of\nlung function within oxygen users, the diversity of user behaviour, and measurement issues. Representative bench\ntesting may help identify key determinants of pulsed-oxygen device efficacy.\nMethods: Three contemporary devices were bench-evaluated across three simulated breathing behaviours: activity,\nrest, & oronasal breathing during sleep. Emphasis was placed on breathing patterns representative of oxygen users.\nResults: All three POCs performed well during simulated breathing during exertion and at rest. Differences in\ntriggering ability were noted for the scenario of oronasal breathing during sleep.\nConclusions: The results are supportive of contemporary POC triggering abilities. The differences shown in ultimate\ntrigger sensitivity may have relevance to oronasal breathing during sleep or other challenging scenarios for pulsed\noxygen delivery, such as dominant mouth breathing during exertion or unfavourable nasal geometry....
Introduction: The high resolution computed tomography (HRCT) is an important\npart in the diagnostic approach of interstitial lung disease (ILD) associated\nwith connective tissue diseases (CTD) by providing detailed information\non the elementary lesion and the radiological pattern of ILD. Aim: to\npoint out the role of HRCT in the diagnosis of ILD associated with CTD\n(ILD-CTD). Methods: A Retrospective descriptive study was conducted between\n2008 and 2017. Data of 24 patients presenting ILD-CTD were collected.\nA review of HRCT was performed by a radiologist without knowledge of the\nCTD. Results: Predominant elementary lesion of ILD associated with dermatomyositis\n(9 cases) was ground glass opacity (n = 9) followed by consolidation\n(n = 6). Non Specific Interstitial Pneumonia (NSIP) was the most reported\npattern (5 cases). Ground glass opacity was also the predominant elementary\nlesion for the 2 cases of scleroderma and in Sjogrenâ??s syndrome (4\ncases/5). NSIP was the predominant radiological presentation in these two\nCTD. Lymphoid interstitial pneumonia revealed Sjogrenâ??s syndrome in one\ncase. In rheumatoid arthritis (6 cases), the elementary HRCT lesions were irregular\ninterlobular septal thickening (n = 4) and honeycombing (n = 4) consistent\nwith Usual Interstitial Pneumonia (UIP) in 2 cases. Similarly UIP has\nbeen described for the 2 patients with lupus and mixed connective tissue disease.\nConclusion: HRCT plays an important role in the management of\nILD-CTD. Description of the HRCT elementary lesions and the radiological\npattern of ILD can be helpful for CTDâ??s diagnosis....
Background: Fibrosing, non-idiopathic pulmonary fibrosis (non-IPF) interstitial lung diseases (fILDs) are a\nheterogeneous group of diseases characterized by a different amount of inflammation and fibrosis. Therapy is\ncurrently based on corticosteroids and/or immunomodulators. However, response to these therapies is highly\nvariable, sometimes without meaningful improvement, especially in more fibrosing forms. Pirfenidone and\nnintedanib have recently demonstrated to reduce functional decline in patients with IPF. However, their antifibrotic\nmechanism makes these two drugs an interesting approach for treatment of fibrosing ILDs other than IPF.\nObjectives: We here report our experience with antifibrotic drugs in fibrosing non-IPF ILDs patients having a\nprogressive phenotype during immunosuppressive therapy.\nMethods: Patients with a multidisciplinary team diagnosis of fibrosing non-IPF ILDs experiencing a progressive\nphenotype during treatment with corticosteroids and/or immunomodulators between October-2014 and January-\n2018 at our tertiary referral Center for ILDs were retrospectively analyzed. Antifibrotic therapy was administered after\napplication with the respective health insurance company and after consent by the patient. Pulmonary-functiontests\nand follow-up visits were performed every .............................
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