Combination of computed tomography imaging pattern and severity of respiratory failure as factors associated with prognosis for acute exacerbation of idiopathic chronic fibrosing interstitial pneumonia.

BACKGROUND AND OBJECTIVES: The prognosis of idiopathic chronic fibrotic interstitial pneumonitis (CFIP) in patients with acute exacerbation (AE) is variable. We examined whether the imaging pattern on thoracic computed tomography (CT) or the severity of respiratory failure with AE-CFIP is associated with short-term prognosis. METHODS: Patients admitted to two university hospitals were retrospectively analyzed and divided into derivation and validation cohorts. The distribution of newly appearing parenchymal abnormalities on thoracic CT was classified into peripheral, multifocal, and diffuse patterns. Respiratory failure was defined as severe if a fraction of inspired oxygen ≥ 0.5 was required to maintain percutaneous oxygen saturation ≥ 90% on admission. Factors associated with 90 day-mortality were analyzed using univariate and Cox proportional hazard regression. RESULTS: In 59 patients with AE-CFIP of the derivation cohort, diffuse pattern on CT was associated with higher mortality within 90 days (43%) than peripheral/multifocal pattern (17%, p = 0.03). Additionally, compared with non-severe failure, severe respiratory failure was associated with higher mortality (47% vs. 21%, p = 0.06). Cox proportional hazard regression analysis demonstrated that a combination of diffuse pattern on CT and severe respiratory failure was associated with the poorest prognosis (hazard ratio [HR] 3.51 [interquartile range 1.26-9.80], p = 0.016) in the derivation cohort, which was confirmed in the validation cohort (n = 31, HR 4.30 [interquartile range 1.51-12.2], p = 0.006). CONCLUSION: The combination of imaging pattern on thoracic CT and severity of respiratory failure was associated with the prognosis of idiopathic AE-CFIP.

Prognosis of acute exacerbation in idiopathic pulmonary fibrosis with pulmonary emphysema: a retrospective cohort study in Japan.

OBJECTIVES: To analyse the clinical characteristics and prognosis of acute exacerbation (AE) in patients with idiopathic pulmonary fibrosis (IPF) and pulmonary emphysema. DESIGN: A multicentre retrospective cohort study SETTING: Two university hospitals in Japan PARTICIPANTS: Patients admitted to hospitals due to AE of IPF diagnosed based on a multidisciplinary discussion. INTERVENTIONS: None PRIMARY AND SECONDARY OUTCOME MEASURES: 90-day mortality rate METHODS: We retrospectively analysed consecutive patients with AE of IPF, with or without pulmonary emphysema, admitted to two university hospitals between 2007 and 2018. RESULTS: Among 62 patients (median age, 75 years; 48 men) admitted for AE of IPF, 29 patients (46%) presented with concomitant pulmonary emphysema. There was no significant difference in the arterial partial oxygen pressure/fraction of inhaled oxygen (P/F) ratio or other laboratory and radiographic data between patients with and without emphysema. The 90-day mortality rate was significantly lower in patients with emphysema than in those with IPF alone (23% vs 52%, p=0.03). The median survival time was significantly longer in patients with emphysema than in those with IPF alone (405 vs 242 days, p=0.02). CONCLUSION: Patients with IPF and emphysema had better short-term survival after AE than those with non-emphysematous IPF.

Regression of Lung Squamous Cell Carcinoma after the Withdrawal of Cyclosporin A Combined with Pirfenidone Treatment in a Patient with Idiopathic Pulmonary Fibrosis.

A 72-year-old man was treated with prednisolone and cyclosporine A for idiopathic pulmonary fibrosis. A nodule with a diameter of 19 mm was found in the right lung and diagnosed as lung squamous cell carcinoma. Anti-cancer treatments were not performed because of the presence of advanced interstitial pneumonia and chronic respiratory failure. Cyclosporine A was tapered to avoid suppression of anti-tumor immunity, and pirfenidone was initiated. Within 2 months, the tumor had shrunk to 10 mm in diameter and remained regressed for 9 months. This is the first report of a non-hematologic solid organ tumor responding to the discontinuation of immunosuppressants.

Cytokine/Chemokine/Growth Factor Levels in Malignant Pleural Effusion of Non-small Cell Lung Cancer.

OBJECTIVE: Malignant pleural effusions (MPEs) deteriorate the quality of life in patients with advanced stages of cancer. Although vascular endothelial growth factor (VEGF) is known to be a key factor for MPE formation, it is not fully clarified whether there are other components related to its appearance. METHODS: Pleural effusion and serum samples were collected from patients with MPEs of non-small cell lung cancer. Cellular analysis of pleural effusion was performed using fluorescence flow cytometry. The concentrations of 12 cytokines, chemokines, and growth factors in MPEs and serum samples were analyzed using the cytometric bead array method. RESULTS: Fifteen patients (median age: 70 years, 11 males) with non-small cell lung cancer (13 adenocarcinoma, 2 squamous cell carcinoma) were enrolled in this study. Concentrations of VEGF, interleukin (IL)-5, IL-6, IL-8, IL-12/IL-23p40, and C-C motif chemokine ligand (CCL) 2 were significantly higher in MPE than in serum. Pleural IL-5 levels correlated with malignant cell numbers in MPE. There was no factor related to the total amount of drained effusion or period of chest tube insertion. CONCLUSIONS: Production of six molecules were increased in the pleural cavity with MPE of non-small cell lung cancer. Complex interactions among these molecules may regulate MPE formation.

Successful Treatment of a Patient with Lung Adenocarcinoma Harboring Compound EGFR Gene Mutations, G719X and S768I, with Afatinib.

Mutations in the gene encoding epidermal growth factor receptor (EGFR) are the most frequent driver mutations in lung adenocarcinoma in Japan. Exon 19 deletion and L858R mutation in exon 21 are the most common EGFR mutations. Uncommon mutations, such as G719X, S768I, and L861Q, and compound mutations, combinations of 2 common or uncommon mutations, have also been reported. EGFR tyrosine kinase inhibitors (TKIs) are effective against cancers harboring common mutations; however, their efficacy against cancers with uncommon or compound mutations remains unclear. We report the case of a 67-year-old man with lung adenocarcinoma (clinical stage IIIA [cT1N2M0]), harboring an uncommon compound mutation, G719X and S768I. The cancer progressed within 2 months of initial chemoradiotherapy. Treatment with afatinib (40 mg/day) produced a partial response, which was maintained for 17 months with continued treatment. A literature review revealed that lung cancer with G719X/S768I compound mutation exhibited good response to EGFR-TKIs, even better than that of lung cancers with single uncommon mutations.

Etiology and prognosis of spontaneous pneumothorax in the elderly.

AIM: Spontaneous pneumothorax shows a bimodal age distribution, with the secondary peak including patients aged ≥50 years. The purpose of this study was to clarify the etiology and prognosis of spontaneous pneumothorax in the elderly. METHODS: Patients aged ≥50 years who were admitted to a tertiary university hospital between 2006 and 2016 due to spontaneous pneumothorax were retrospectively investigated. RESULTS: Among 136 consecutive patients aged ≥50 years with spontaneous pneumothorax (mean age, 70 years; 114 men), 124 (91%) had underlying lung diseases, including pulmonary emphysema (42%) and interstitial pneumonia (27%). The median period of thoracic drainage was longer (14 days) in the cases with interstitial pneumonia than in the cases of primary pneumothorax (4 days; P < 0.001) and emphysema (9 days; P < 0.005). Eighteen patients (13%) died within 180 days after the onset of pneumothorax. The mortality rate was highest in the cases with interstitial pneumonia (27%) and was mostly associated with infectious complications. Death or worsened respiratory failure within 180 days from admission was associated with older age, systemic corticosteroid use and interstitial pneumonia in multivariate logistic regression analysis. CONCLUSIONS: Pulmonary emphysema is the most common underlying disease associated with spontaneous pneumothorax in the elderly population. Pneumothorax associated with interstitial pneumonia is less frequent, but it requires prolonged tube thoracostomy and demonstrates higher mortality and morbidity, particularly in those receiving systemic corticosteroids. Different treatment strategies are warranted for patients with interstitial pneumonia-related pneumothorax. Geriatr Gerontol Int 2020; 20: 878-884.

An Autopsy Case of Paradoxical Pulmonary Thromboembolism by Arteriovenous Fistula from Right Common Iliac Artery to Common Iliac Vein: A Rare Cause of Pulmonary Embolism.

BACKGROUND Pulmonary thromboembolism (PTE) sometimes leads to a shock state and sudden death due to acute massive pulmonary arterial thrombosis. The origins of pulmonary arterial thrombi are varied, but most arise from deep vein thrombosis. We herein presented a very rare cause of PTE due to paradoxical embolism caused by arteriovenous fistula from common iliac artery to common iliac vein. CASE REPORT A 74-year-old man was admitted because of increasing dyspnea on exertion. The diagnosis of idiopathic pulmonary fibrosis was made and corticosteroid therapy was started. On the 5th hospital day, the patient suddenly developed cardiopulmonary arrest and died despite cardiopulmonary resuscitation. An autopsy revealed that the left main pulmonary artery was occluded by a massive but organized thrombus that was similar to an arterial thrombus in the right common iliac artery. On histopathological examination, the emboli in the pulmonary artery demonstrated characteristics similar to thrombus in the common iliac artery. This suggested that the emboli had passed through the arteriovenous fistula from the right common iliac artery to the common iliac vein. CONCLUSIONS This is a very rare cause of fatal paradoxical pulmonary thromboembolism. Paradoxical emboli passing through arteriovenous fistula from the right common iliac artery to the common iliac vein are a rare cause of PTE.

Progressive Multifocal Micronodular Pneumocyte Hyperplasia in the Lungs of a Patient with Tuberous Sclerosis Complex: A Case Report.

We report a case of multifocal micronodular pneumocyte hyperplasia (MMPH) in a patient with tuberous sclerosis complex, in whom the lung nodules increased in the number and size over the course of 8 years. We diagnosed MMPH following a lung biopsy performed during video-assisted thoracic surgery. In most of the previously reported cases, the number and size of lung nodules is unchanged during the clinical course. Our case is the first report of progressive disease in pathologically proven MMPH.