Acute Exacerbation of Interstitial Fibrosis After Pulmonary Resection

doi:10.1016/j.athoracsur.2005.09.036

The Annals of Thoracic Surgery

Volume 82, Issue 1, July 2006, Pages 336-338

https://doi.org/10.1016/j.athoracsur.2005.09.036 Get rights and content

Idiopathic pulmonary fibrosis is a chronic diffuse lung disease of unknown cause, and a risk factor for increasing morbidity and mortality after lung resection. Acute exacerbation of idiopathic pulmonary fibrosis after lung surgery for lung cancer is rare. The outcome is unsatisfactory despite therapy. We report two cases of acute exacerbation of idiopathic pulmonary fibrosis after resection for lung cancer.

Section snippets

Patient 1

An 80-year-old man presented with a left lower lobe mass. A high-resolution computed tomographic scan revealed a mass in the left lower lobe superior segment, bilateral subpleural interstitial reticular opacities with traction bronchiectasis, and honeycombing (Fig 1). The patient had diabetes mellitus and pulmonary fibrosis. He had been on a bronchodilator and antidiabetics. He had a 54-pack year history of cigarette smoking. Preoperative respiratory function tests were normal, but his blood

Comment

More than 100 disorders have been classified as diffuse lung diseases [6]. These entities are grouped together because of certain common clinical, histologic, and radiographic features [6]. Idiopathic pulmonary fibrosis is a diffuse lung disease that more commonly affects male patients older than 50 years of age [1, 6]. It is controversial whether or not all patients with presumed IPF require biopsy [3, 6]. Idiopathic pulmonary fibrosis is also associated with an increased risk of lung cancer [2

References (7)

P. Kumar et al.
Pulmonary fibrosis and lung cancerrisk and benefit analysis of pulmonary resection
J Thorac Cardiovasc Surg
(2003)
V. Ambrosini et al.
Acute exacerbation of idiopathic pulmonary fibrosisreport of a series
Eur Respir J
(2003)
American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias
Am J Respir Crit Care Med
(2002)

There are more references available in the full text version of this article.

Cited by (39)

Astragaloside IV protects against lung injury and pulmonary fibrosis in COPD by targeting GTP-GDP domain of RAS and downregulating the RAS/RAF/FoxO signaling pathway
2023, Phytomedicine
Pulmonary fibrosis is a chronic progressive interstitial lung disease characterized by the replacement of lung parenchyma with fibrous scar tissue, usually as the final stage of lung injury like COPD. Astragaloside IV (AST), a bioactive compound found in the Astragalus membranaceus (Fisch.) used in traditional Chinese medicine, has been shown to improve pulmonary function and exhibit anti-pulmonary fibrosis effects. However, the exact molecular mechanisms through which it combats pulmonary fibrosis, especially in COPD, remain unclear.
This study aimed to identify the potential therapeutic target and molecular mechanisms for AST in improving lung injury especially treating COPD type pulmonary fibrosis both in vivo and in vitro.
Multi lung injury models were established in mice using lipopolysaccharide (LPS), cigarette smoke (CS), or LPS plus CS to simulate the processes of pulmonary fibrosis in COPD. The effect of AST on lung function protection was evaluated, and proteomic and metabolomic analysis were applied to identify the signaling pathway affected by AST and to find potential targets of AST. The interaction between AST and wild-type and mutant RAS proteins was studied. The RAS/RAF/FoxO signaling pathway was stimulated in BEAS-2B cells and in mice lung tissues by LPS plus CS to investigate the anti-pulmonary fibrosis mechanism of AST analyzed by western blotting. The regulatory effects of AST on the RAS/RAF/FoxO pathway dependent on RAS were further confirmed using RAS siRNA.
RAS was predicted and identified as the target protein of AST in anti-pulmonary fibrosis in COPD and improving lung function. The administration of AST was observed to impede the conversion of fibroblasts into myofibroblasts, reduce the manifestation of inflammatory factors and extracellular matrix, and hinder the activation of epithelial mesenchymal transition (EMT). Furthermore, AST significantly suppressed the RAS/RAF/FoxO signaling pathway in both in vitro and in vivo settings.
AST exhibited lung function protection and anti-pulmonary fibrosis effect by inhibiting the GTP-GDP domain of RAS, which downregulated the RAS/RAF/FoxO signaling pathway. This study revealed AST as a natural candidate molecule for the protection of pulmonary fibrosis in COPD.
Treatment-Related Toxicity in Patients With Early-Stage Non-Small Cell Lung Cancer and Coexisting Interstitial Lung Disease: A Systematic Review
2017, International Journal of Radiation Oncology Biology Physics
Citation Excerpt :
Full text evaluation yielded 13 SABR studies (13-25), 3 proton beam therapy (PBT) studies (26-28), 1 carbon ion beam therapy (CIBT) study (29), and 3 RFA studies (30-32) that fulfilled all inclusion and exclusion criteria and underwent full abstraction. Data was also abstracted from 30 surgical studies (33-62) for reference. No articles were identified for treatment-related mortality or ILD-specific toxicity after conventionally fractionated radiation therapy in patients with ES-NSCLC and coexisting ILD.
Definitive treatment for patients with early-stage non-small cell lung cancer (ES-NSCLC) is usually well tolerated. Patients with ES-NSCLC and coexisting interstitial lung disease (ILD) are at increased risk of severe treatment-related toxicity after definitive therapy. The main objective of this systematic review is to provide a pooled estimation of treatment-related mortality and ILD-specific toxicity in this population of patients and to identify trends for further study.
The MEDLINE and Embase databases were queried from respective dates of inception to January 2016. Studies that included patients who underwent definitive treatment for ES-NSCLC not combined with other treatments were included. Patients with oligometastases who were treated with aggressive palliation were included if it did not constitute the majority of patients in a specific study. The results were summarized with weighted proportions according to the sample size of individual studies.
Overall, 3056 records were reviewed and 50 journal articles were included in the abstraction. The weighted proportion of treatment-related mortality (and ILD-specific toxicity) in primarily medically inoperable patients was as follows: stereotactic ablative radiation therapy (SABR) 15.6% (25%), particle beam therapy 4.3% (18.2%) and radiofrequency ablation (RFA) 8.7% (25%). The data for medically operable patients who underwent surgery were extracted for reference. Treatment-related mortality and ILD-specific toxicity were 2.2% and 12%, respectively, in the surgical population. On analysis of reported SABR dose parameters, V20 ≤ 6.5% and mean lung dose ≤4.5 Gy were found to be metrics associated with reduced mortality.
A consistently high level of treatment-related mortality and ILD-specific toxicity was observed in primarily medically inoperable patients treated with SABR, particle beam therapy, and RFA. For these patients, curative treatment should be considered in the context of the high toxicity rates and overall poor prognosis. Stringent radiation dosimetric parameters may result in reduced toxicity.
Effect of Perioperative Pirfenidone Treatment in Lung Cancer Patients With Idiopathic Pulmonary Fibrosis
2016, Annals of Thoracic Surgery
Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a life-threatening complication of lung cancer operation for patients with IPF, and no effective prophylaxis has ever been reported. In this study, we investigate the effect of perioperative treatment with an anti-IPF agent on reduction of the risk of developing AE-IPF.
A consecutive series of 50 lung cancer patients with IPF who underwent operations at our institution from October 2006 to October 2014 was retrospectively investigated. Since September 2009, pirfenidone was orally administered to patients from 4 weeks before operation to 4 weeks after operation. Thirty-one patients received the perioperative pirfenidone treatment (PPT), and their clinical outcome was retrospectively compared with that of 19 patients who did not receive PPT.
No differences were found in age, smoking history, sex, vital capacity, KL-6, procedure, or risk score (10.5 ± 2.2 versus 11.2 ± 1.5) between the PPT and non-PPT groups. The incidence of AE-IPF for the PPT/non-PPT groups was 0.0%/10.5% within 30 postoperative days (p = 0.07) and 3.2%/21.1% within 90 postoperative days (p = 0.04), respectively. Logistic regression analysis showed a significant association between PPT and the incidence of AE-IPF within 30 (p = 0.045) and 90 (p = 0.04) postoperative days.
A prophylactic effect of PPT for postoperative AE-IPF in patients with lung cancer was suggested. Further confirmatory prospective studies should be considered for PPT.
Acute exacerbation of idiopathic pulmonary fibrosis
2011, Medicina Clinica
La fibrosis pulmonar idiopática (FPI) es una enfermedad crónica y progresiva. En su evolución puede presentar episodios de exacerbación aguda, cuya definición más aceptada sería aquel episodio de deterioro agudo, con significado clínico, de causa no identificada, en un paciente con una FPI subyacente. La incidencia de la FPI con exacerbación aguda oscila entre un 5–19% de pacientes por año, y se estima que un 47% de los pacientes con FPI presentará un deterioro clínico agudo antes del fallecimiento.
El signo que invariablemente está presente es la disnea, acompañada de tos, fiebre y síntomas seudogripales. La presencia de leucocitosis en el hemograma, neutrofilia en el lavado broncoalveolar y opacidades difusas en vidrio deslustrado superpuestas a las habituales de la FPI en la radiogafía de tórax orientan el diagnóstico. El hallazgo histológico típico es el daño alveolar difuso.
La respuesta al tratamiento es pobre, aunque la metilprednisolona parece ser la mejor opción terapéutica.
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive disease. Its natural history may have episodes of acute exacerbation (AE-IPF), whose best accepted definition would be a clinically significant acute worsening, without identified cause, in patients with an underlying IPF.
The incidence of AE-IPF ranges from 5%–19% of patients per year. It is estimated that acute deterioration of the respiratory status occurred in 47% of these patients before death.
A rapidly progressive dyspnoea is the most prominent symptom, with associated cough, fever and flu-like symptoms. The diagnosis is strengthened by the presence of leukocytosis on the blood count, neutrophilia on the bronchoalveolar lavage, and development of new diffuse bilateral ground-glass opacities superimposed on the pre-existing radiographic findings.
The major histological finding is diffuse alveolar damage.
Methylprednisolone seems to be the best therapeutic option, although the treatment response is usually poor.
Management of acute exacerbations of pulmonary fibrosis
2011, Revue des Maladies Respiratoires
L’exacerbation aiguë (EA) de fibrose pulmonaire idiopathique (FPI) est une aggravation aiguë de la FPI sans étiologie retrouvée.
La FPI est la plus fréquente des pneumopathies interstitielles chroniques. Son profil évolutif est le plus souvent une aggravation progressive avec une médiane de survie d’environ trois ans. L’EA de FPI est actuellement reconnue comme une complication grave de la maladie. Elle se développe le plus souvent en moins d’un mois et se manifeste par une aggravation de la dyspnée avec apparition de nouvelles images radiologiques. Son diagnostic nécessite surtout d’éliminer toute cause de décompensation aiguë dont les principales sont l’embolie pulmonaire, l’infection bronchopulmonaire, l’insuffisance cardiaque gauche et le pneumothorax. Son traitement n’est pas codifié et, même si des succès thérapeutiques ont été rapportés, son pronostic est sombre. Pour les formes les plus graves, le recours à la ventilation mécanique est classiquement considéré comme inefficace, ce qui conduit souvent à une décision de non-transfert en réanimation. Cependant, cette position doit être remise en question car un accès rapide à la transplantation pulmonaire est maintenant possible dans plusieurs pays dont la France.
L’EA de FPI est une complication grave de la FPI dont la prise en charge optimale n’est pas encore codifiée.
Acute exacerbation of idiopathic pulmonary fibrosis (IPF) is defined by an acute worsening of the respiratory status without any identified cause.
IPF is the most frequent type of chronic interstitial pneumonia. In general, its course is a progressive worsening with a median survival at 3 years after diagnosis. Acute exacerbation is now recognized as a severe complication of IPF. It develops most often within less than 1 month and is characterized by a worsening of dyspnoea associated with the occurrence of new images on chest radiograph. Its diagnosis requires the exclusion of an identified cause for acute deterioration such as pulmonary embolism, bronchopulmonary infection, left heart failure or pneumothorax. The treatment of acute exacerbations of IPF is not well standardized and even though isolated cases of therapeutic success have been reported, its prognosis remains poor. In the most severe cases, mechanical ventilation is generally considered to be ineffective, thus leading most often to a conservative management strategy with no transfer to ICU. However, this attitude is now being questioned since it is now potentially possible to perform an urgent lung transplantation in some patients at least in several countries, including France.
Acute exacerbation of IPF is a severe complication of IPF, but its optimal management is not yet clearly defined.
Idiopathic pulmonary fibrosis: the risk of cancer and the impact on cancer care
2022, ERS Monograph

View all citing articles on Scopus

View full text

Case reportAcute Exacerbation of Interstitial Fibrosis After Pulmonary Resection

Section snippets

Patient 1

Comment

J Thorac Cardiovasc Surg

Acute exacerbation of idiopathic pulmonary fibrosisreport of a series

Eur Respir J

American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias

Am J Respir Crit Care Med

Case report
Acute Exacerbation of Interstitial Fibrosis After Pulmonary Resection