Mortality risk prediction with ILD-GAP index in a fibrotic hypersensitivity pneumonitis cohort.

BACKGROUND: Fibrotic hypersensitivity pneumonitis (fHP) is associated with significant morbidity and mortality. Interstitial lung disease-gender-age-physiology (ILD-GAP) performance in fHP outside the initial cohort was never performed. AIM: To assess the ILD-GAP index's ability to predict mortality in a Portuguese cohort of patients with fHP and analyse whether other clinical variables add value. METHODS: Retrospective analysis of fHP cohort in two Portuguese ILD centres. The baseline ILD-GAP index was calculated. Survival was analysed in months; mortality was the primary outcome. Univariate and multivariate analyses to identify mortality risk factors were performed. RESULTS: A total of 141 patients were included. Fifty-three patients (37.6%) died during the follow-up. The usual interstitial pneumonia (UIP) pattern was found in 49.6%, and their survival was inferior to non-UIP [32 months (interquartile range, IQR = 19, 60) versus 52 months (IQR = 28, 98), p = 0.048]. Patients with an ILD-GAP index higher than three double their risk of mortality [hazard ratio (HR) = 6.48, 95% confidence interval (CI) = (3.03-13.96)] when compared with the patients with an index between 2 and 3 [HR = 3.04, 95% CI = (1.62-5.71)] adjusting for acute exacerbation history. Even though UIP patients had worse survival, it did not reach statistical significance when UIP pattern was added to this model. Acute exacerbation history was an independent risk factor for mortality; however, ILD-GAP still predicted mortality after adjusting for this factor. PaO2 and 6-minute walk test desaturation were not significant risk factors. CONCLUSION: ILD-GAP index is a good predictor for mortality in fHP, even after adjusting for other mortality risk factors.

Pleuroparenchymal fibroelastosis as a complication of chemotherapy: A case report.

Pleuroparenchymal fibroelastosis (PPFE) is a rare and recently described distinct pattern of lung apical fibrosis involving the upper lobe parenchyma and pleural dome. PPFE has definable and reproducible clinical, radiological and histopathological criteria, which allowed its classification as an independent interstitial lung disease. Several factors have been associated with PPFE, such as chemotherapy, especially with alkylating agents. The authors present a case of a 34-year-old female with previous history of Hodgkin lymphoma treated with first line chemotherapy (doxorubicin, bleomycin, vinblastine and dacarbazine). The patient had no other known comorbidities or relevant exposure to lung irritants. A total of 2 years after completing cancer treatment, the patient developed clinical and radiological features of PPFE. Given their previous history of malignancy, a biopsy of the lesion was obtained, which confirmed the diagnosis of PPFE. The authors present this case to raise awareness of this disease and to demonstrate that PPFE can develop months to years following chemotherapy treatment. Moreover, to date, none of these chemotherapy agents have been associated with the development of PPFE.

Pleuroparenchymal Fibroelastosis as Another Potential Lung Toxicity Pattern Induced by Amiodarone / Fibroelastosis pleuroparenquimal como posible patrón de toxicidad pulmonar inducido por amiodarona

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Transbronchial lung cryobiopsy in smoking-related interstitial lung diseases.

BACKGROUND: Transbronchial lung cryobiopsy (TBLC) is an emerging technique in the diagnostic approach to diffuse parenchymal lung diseases. However, the role of TBLC in smoking-related Interstitial Lung Diseases (ILDs) is still under discussion. OBJECTIVES: The aim of the present study was to describe our experience with TBLC in diagnostic work-up of patients with smoking-related ILDs. METHOD: We retrospectively reviewed data of patients evaluated in a tertiary hospital ILDs outpatient clinic, who underwent TBLC, from September 2014 to December 2019. TBLC was performed in accordance with the 2018 expert statement from the Cryobiopsy Working Group. RESULTS: Forty-five patients (25 men [55.6%]) with a mean age of 53.9 years [SD, 9.1] were included. The most frequent radiological pattern was ground glass opacity (42 patients). TBLC was performed in different segments of the same lobe in 38 patients and in two lobes in 7 patients. The mean maximal diameter of the samples was 5.2 mm (range, 3-16 mm [SD 2.0]). Pneumothorax occurred in seven patients (15%) and moderate bleeding occurred in one patient. A specific pathological diagnosis was achieved in 43 of 45 patients. The most frequent histopathologic pattern found was desquamative interstitial pneumonia (33 patients), followed by smoking-related interstitial fibrosis (7 patients), respiratory bronchiolitis - ILD (1 patient) and pulmonary Langerhans cell histiocytosis (1 patient). Two patients had alternative diagnosis (Pneumoconiosis and Interstitial Pneumonia with unspecific features) and one patient had normal lung parenchyma. A definitive multidisciplinary team (MDT) diagnosis was reached in 95.5% (43 of 45 cases). Two patients were submitted to additional diagnostic techniques. CONCLUSIONS: The results from this series support TBLC as a safe procedure with a meaningful diagnostic value in the context of a MDT approach of smoking-related ILDs. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (4): e2020013).

Pulmonary Sarcoidosis: Prognostic Factors at Diagnosis in Patients from North of Portugal.

BACKGROUND: Sarcoidosis is a multisystemic granulomatous disease that affects the lungs in more than 90% of the patients. It is associated with a variable clinical course and considering all the different forms of disease presentation, there are an absence of reliable clinical prognostic markers that can predict the outcome at diagnosis. OBJECTIVE: The aim of our study was to investigate prognostic factors at diagnosis in a population of sarcoidosis patients from Northern Portugal. METHODS: A group of 110 patients with chronic evolution was compared with 129 patients with disease resolution regarding their clinical, radiologic and laboratorial features. RESULTS: We found a positive association between the chronic forms and lung function impairment, radiologic stage II, lower lymphocyte CD4/CD8 and extrapulmonary disease. Löfgren syndrome and asthenia instead had a protective significant association to chronicity. Our final logistic regression model found a significant independent association between age (adjusted OR=1.06), extrapulmonary involvement (adjusted OR=2.68), Löfgren's syndrome (adjusted OR=0.15) with outcome toward chronicity. CONCLUSIONS: In this first study searching for prognostic factors at diagnosis in a Northern Portuguese population, we found clinical prognosis factors that have been described in other populations that should be considered whenever sarcoidosis is identified.

Pleuroparenchymal Fibroelastosis in association with Connective Tissue Disease: a new interstitial pneumonia to be aware of.

Pleuroparenchymal fibroelastosis (PPFE) is a rare and recently described interstitial pneumonia. It consists of progressive fibrosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobes, with defined and reproducible clinical, radiological and histopathological criteria. No effective treatment has yet been shown to modify the natural course of the disease, which vary greatly in the literature. Several conditions have been associated with PPFE, including connective tissue diseases (CTD). The authors present two cases of female patients with a CTD (rheumatoid arthritis and limited cutaneous systemic sclerosis, respectively) who presented with typical bilateral upper lobe thickening in chest-HRCT. In the first case, diagnosis was based on "definite" radiological and histopathological criteria for PPFE, while in the second case diagnosis was established on clinical grounds after discussion in a multidisciplinary team meeting. The authors present these cases of CTD-associated PPFE in order to raise awareness of this entity among clinicians.

Role of Bronchoscopic Techniques in the Diagnosis of Thoracic Sarcoidosis.

The diagnosis of sarcoidosis relies on clinical and radiological presentation, evidence of non-caseating granulomas in histopathology and exclusion of alternative causes of granulomatous inflammation. Currently, a proper diagnosis, with a high level of confidence, is considered as key to the appropriate diagnosis and management of the disease. In this sense, this review aims to provide a brief overview on the role of bronchoscopy in the diagnosis of thoracic sarcoidosis, incorporating newer techniques to establish, including endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA), endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA), transesophageal ultrasound-guided needle aspiration with the use of an echo bronchoscope (EUS-B-FNA) and transbronchial lung cryobiopsy (TBLC). Most of the literature reports the diagnostic superiority of endosonographic techniques, such as EBUS-TBNA alone or in combination with EUS-FNA, over conventional bronchoscopic modalities in diagnosing Scadding stages I and II of the disease. Moreover, TBLC may be considered a useful and safe diagnostic tool for thoracic sarcoidosis, overcoming some limitations of transbronchial lung biopsy (TBLB), avoiding more invasive modalities and being complementary to endosonographic procedures such as EBUS-TBNA.

Diagnostic Yield of Computed Tomography-Guided Transthoracic Lung Biopsy in Diffuse Lung Diseases.

BACKGROUND: Accurate diagnosis is essential for successful management of diffuse lung disease (DLD). Histopathology may sometimes be necessary. Surgical lung biopsy, the gold standard, carries a risk of morbidity and mortality. Computed tomography (CT) guided transthoracic lung biopsy (CT-TLB) is a minimally invasive method for obtaining lung tissue. However, its diagnostic yield is unknown in DLD. OBJECTIVE: To assess the diagnostic yield of CT-TLB in DLD according to the predominant high-resolution CT (HRCT) patterns. METHODS: Between January 2009 and December 2016, we enrolled all consecutive adult patients with suspicion of DLD who underwent CT-guided transthoracic lung biopsy during the diagnostic work-up. All biopsies were performed by a senior interventional radiologist using CT fluoroscopy. RESULTS: The study included 169 patients (50.3% men) with a mean (±SD) age of 58.3 ± 14 years. Consolidation was the predominant HRCT pattern. A definitive or probable diagnosis was made in 66.3%. The most frequent diagnosis was organizing pneumonia (36.2%). Diagnostic yield was higher when the predominant HRCT pattern was consolidation or nodular. The most common complication was pneumothorax (17.8%); other complications included mild hemoptysis (7.7%), hemothorax (1.2%), and death (0.59%). No acute exacerbation of the underlying condition was observed. CONCLUSIONS: CT-TLB proved to be accurate and safe for the diagnosis of DLD. The overall diagnostic yield of the procedure was 66.3%. Given its low complication rates, CT-TLB can be an option in patients whose respiratory function is seriously impaired and in those with substantial comorbidities, where more invasive procedures cannot be performed for reasons of safety.

Lung and renal transplantation.

UNLABELLED: Renal transplantation is the most common type of solid organ transplantation and kidney transplant recipients are susceptible to pulmonary complications of immunosuppressive therapy, which are a diagnostic and therapeutic challenge. AIM: To evaluate patients admitted to the Renal Transplant Unit (RTU) of Hospital de S. João with respiratory disease. SUBJECT AND METHODS: We performed a retrospective study of all patients admitted to RTU with respiratory disease during a period of 12 months. RESULTS: Thirty-six patients were included. Mean age 55.2 (+/-13.4) years; 61.1% male. Immunosuppressive agents most frequently used were prednisolone and mycophenolate mofetil associated with ciclosporin (38.9%) or tacrolimus (22.2%) or rapamycin (13.9%). Thirty-one patients (86.1%) presented infectious respiratory disease. In this group the main diagnoses were 23 (74.2%) pneumonias, 5 (16.1%) opportunistic infections, 2 (6.5%) tracheobronchitis, and 1 case (3.2%) of lung abscesses. Microbiological agent was identified in 7 cases (22.6%). Five patients (13.9%) presented rapamycin-induced lung disease. Fibreoptic bronchoscopy was performed in 15 patients (41.7%), diagnostic in 10 cases (66.7%). Mean hospital stay was 17.1 (+/-18.5) days and no related death was observed. CONCLUSION: Respiratory infections were the main complications in these patients. Drug-induced lung disease implies recognition of its features and a rigorous monitoring of drug serum levels. A more invasive diagnostic approach was determinant in the choice of an early and more specific therapy.