Clinical Utility of Surgical Lung Biopsy for Patients with Acute Respiratory Distress Syndrome: A Systematic Review and Meta-Analysis

<b><i>Background:</i></b> Surgical lung biopsy (SLB) is performed in patients with acute respiratory distress syndrome (ARDS); however, its clinical utility remains unclear. <b><i>Objectives:</i></b> We categorized the pathological diagnoses and investigated the predictive value for short-term mortality. <b><i>Method:</i></b> Three electronic databases (MEDLINE, the Cochrane Central Register of Controlled Trials, and ClinicalTrials.gov) were searched for the included studies. The QUADAS-2 was used to evaluate the risk of bias and its applicability. The types and populations of pathological diagnoses were investigated. The pooled sensitivity, positive likelihood ratio (LR+), negative likelihood ratio (LR−), and diagnostic odds ratio (DOR) were estimated at a fixed specificity. Hierarchical summary receiver operating characteristic curves were drawn. <b><i>Results:</i></b> A total of 16 studies that enrolled 758 patients were included. The pathological diagnoses were as follows: diffuse alveolar damage (DAD) 29.9%; infection 24.7%; interstitial lung disease 17.2%; malignancy 3.6%; cardiovascular disease 3.6%; drug toxicity 2.3%; connective tissue disease 2.2%; allergic disease 1.1%; and nonspecific diagnosis 15.4%. To predict short-term mortality, 13 studies that enrolled 613 patients used DAD as an index test and recorded a mortality rate of 56.9% (349 of 613 patients). A total of 3 studies that used index tests other than DAD were excluded. The pooled sensitivity, fixed specificity, LR+, LR−, and DOR were 0.46 (95% confidence interval [CI]: 0.29–0.56), 0.69, 1.48 (95% CI: 0.92–1.81), 0.78 (95% CI: 0.63–1.03), and 1.90 (95% CI: 0.89–2.86), respectively. <b><i>Conclusions:</i></b> SLB is unlikely to provide a specific diagnosis and should not be recommended for confirming DAD or predicting ARDS prognosis.

Pulmonary fibrosis in dyskeratosis congenita: a case report with a PRISMA-compliant systematic review

Background Dyskeratosis congenita (DC) is a rare genetic disorder of poor telomere maintenance. Pulmonary fibrosis (PF) related to DC is rarely reported. Case presentation A 23-year-old student presented with a four-year history of progressive cough and exertional dyspnea. Physical examination was remarkable for typical mucocutaneous abnormalities. Chest computerized tomography scan revealed interstitial fibrosis. Testing of peripheral blood leukocytes confirmed that his telomeres were 30th percentile of age-matched controls. A heterozygous missense mutation located in exon 22 of PARN gene was identified in the patient by whole exome sequencing. The patient refused danazol therapy and lung transplantation, and died of respiratory failure 2 years later. In addition, this case and 26 reported cases of DC-related PF identified through the comprehensive search of PubMed, Web of Science, WANFANG and CNKI were reviewed. Later-onset PF was observed in 11 patients (40.7%). Radiological usual interstitial pneumonia (UIP) pattern or possible UIP pattern was noted only in half of patients. However, histopathological UIP or probable UIP patterns were found in 63.6% of patients. Age at bone marrow failure (BMF) and the frequency of normal to mild thrombocytopenia in later-onset patients was significantly higher than in early-onset patients (p = 0.017 and p = 0.021, respectively). Age at PF and age at BMF in DC patients with TERC/TERT variants was significantly higher than in those with TINF2 variants or DKC1/NHP2 variants (p = 0.004 and p = 0.003, respectively). The patients with TERT/TERC/RTEL1/PARN variants had a significantly better transplant-free survival than those with TINF2 variants or DKC1/NHP2 variants (p < 0.05). Patients who underwent surgical lung biopsy had significantly worse transplant-free survival than those without lung biopsy (p = 0.042). Worse survival was found in patients with immunosuppression therapy than in those without (p = 0.012). Conclusions It is common for DC-associated PF to occur later in life without significant hematological manifestations. Mutations in the genes encoding different components of the telomere maintenance pathway were associated with clinical phenotypes and prognosis. PF caused by DC should be kept in mind by clinicians in the differential diagnosis of patients with unexplained PF and should be excluded before diagnostic surgical lung biopsy is undertaken or empirical immunosuppression therapy is prescribed.

Awake or intubated surgery in diagnosis for interstitial lung diseases? A prospective study

BackgroundRisks associated with Video-Assisted Surgical Lung Biopsy (VASLB) for interstitial lung disease (ILD) with endotracheal intubation and mechanical ventilation are not nil. Awake Video-Assisted Surgical Lung Biopsy (Awake-VASLB) has been proposed as a method to obtain a precise diagnosis in several different thoracic diseases.ObjectivesTo compare clinical outcomes of Awake-VASLB and Intubated-VASLB in patients with suspected ILDs.MethodsFrom June 2016 to February 2020, all patients submitted to elective VASLB for suspected ILD were included. Differences in outcomes between Awake-VASLB and Intubated-VASLB were assessed through univariable, multivariable-adjusted, and a propensity score-matched (PS) analysis.Measurements and main resultsAwake-VASLB was performed in 66 out of 100 patients, while 34 underwent Intubated-VASLB. The Awake- VASLB resulted in a lower postoperative morbidity (OR 0.025; CI95% 0.001, 0.35; p=0.006), a less unexpected Intensive Care Unit (ICU) admission, a less need for rescue therapy for pain, a reduced surgical and anaesthesiologic time, a reduced chest drain duration, a lower postoperative length of stay.ConclusionAwake-VASLB in patients affected by ILD is feasible and seems safer than Intubated-VASLB.

Diagnosis of idiopathic pulmonary fibrosis

Interstitial lung disease (ILD) is a group of diseases, involving the inflammation and fibrosis of the interstitium of the lung. ILD is classified according to whether or not the cause is known. Known causes of ILDs include inhalation of environmental substances, drugs, infection, and related connective tissue disease. ILD of unknown cause is called idiopathic ILD. The most common form of idiopathic ILD is idiopathic pulmonary fibrosis (IPF). IPF is a chronic progressive fibrosing ILD that results in the decline of lung function with exertional dyspnea, cough, bibasilar inspiratory crackles, and digital clubbing. The incidence of IPF increases with age, and is predominant in men. The most characteristic feature of IPF is a usual interstitial pneumonia (UIP) pattern detected on high-resolution computed tomography (HRCT). The typical HRCT pattern in case of UIP is honeycombing, with or without traction bronchiectasis or bronchiolectasis; this may be superimposed with fine reticulation. The typical distribution of UIP is subpleural, and there is basal predominance with heterogeneity. A definitive diagnosis of IPF in patients with clinically suspected IPF is made when there is presence of a UIP pattern on HRCT. Bronchoalveolar lavage or surgical lung biopsy is not recommended if a UIP pattern is detected on HRCT. However, bronchoalveolar lavage and surgical lung biopsy are required if probable UIP pattern, indeterminate UIP pattern, or an alternative diagnosis pattern are found on HRCT in order to diagnose IPF. A specific combination of HRCT patterns and histopathological patterns requiring multidisciplinary discussion is necessary to rule in IPF or rule it out.