Outcomes of patients with interstitial lung disease referred for lung transplant assessment

Background: Patients with interstitial lung disease (ILD) very frequently die before the opportunity to receive lung transplantation (LTx). This retrospective study describes the clinical course of 86 patients with ILD referred for LTx assessment between January 1999 and December 2002. Aims: (i) To describe the outcomes, (ii) to identify reasons of delay to transplantation, (iii) to describe the causes of death/complications and (iv) to assess the pathological diagnosis and concordance with explanted lung pathology. Methods: Data were collected from the case notes of all patients with ILD referred to the Alfred Hospital over a 4‐year period. Results: Twenty women and 66 men, mean age of 55 ± 8 years, were referred for LTx assessment. Forty‐five patients were deemed not suitable for LTx and 41 were listed. Twenty‐two patients underwent transplantation, 16 died on the waiting list and 7 are still on the waiting list. Complications were frequent (e.g. pulmonary embolism, malignancy and infection) and carried high mortality. Patients dying on the waiting list appeared generally to be in accelerated decline, dying shortly after listing, with no evidence in their lung function test assessment predicting them as a poor prognosis group. Conclusions: Serious complications and death on the waiting list of patients with idiopathic pulmonary fibrosis are high, not apparently because of delayed referral but usually in patients undergoing very rapid decline.     

Contemporary Concise Review 2022: Interstitial lung disease

• Novel genetic associations for idiopathic pulmonary fibrosis (IPF) risk have been identified. Common genetic variants associated with IPF are also associated with chronic hypersensitivity pneumonitis.
• The characterization of underlying mechanisms, such as pathways involved in myofibroblast differentiation, may reveal targets for future treatments.
• Newly identified circulating biomarkers are associated with disease progression and mortality.
• Deep learning and machine learning may increase accuracy in the interpretation of CT scans.
• Novel treatments have shown benefit in phase 2 clinical trials.
• Hospitalization with COVID-19 is associated with residual lung abnormalities in a substantial number of patients.
• Inequalities exist in delivering and accessing interstitial lung disease specialist care.

     

Cytomegalovirus pneumonitis in a patient with Hodgkin disease and ataxia-telangiectasia

We describe a child with with Hodgkin disease and ataxia-telangiectasia who also developed an unusual pneumonitis caused by a cytomegalovirus which was fatal.     

Diagnostic utility of surgical lung biopsies in elderly patients with indeterminate interstitial lung disease

Background and objective

Idiopathic pulmonary fibrosis (IPF) is increasingly diagnosed by clinical and computed tomography (CT) criteria; however, surgical lung biopsy (SLB) may still be required in patients who lack definite CT features of usual interstitial pneumonia (UIP). We reviewed a cohort of elderly patients who underwent SLB, to evaluate the benefit of SLB in diagnosing idiopathic interstitial pneumonia (IIP).

Methods

We searched the pathology records of Mayo Clinic for ambulatory patients at least 75 years old, who underwent SLB between 2000 and 2012 for indeterminate IIP. Histologic slides were reviewed and clinical data were extracted from the record.

Results

A total of 55 patients (35 male) were enrolled. Median (interquartile range) age was 77 (76–80) years. Forced vital capacity was 70 (61–76)% and diffusing capacity of the lungs for carbon monoxide was 48 (42–54)% of predicted. In total, 37 (67%) patients had IPF, including 61% of those with HRCT findings inconsistent with UIP. Thirty‐day mortality was 10% and 90‐day mortality was 15%.

Conclusion

The high mortality rate of SLB complicates the risk–benefit analysis in elderly patients with IIP. The expected value of the SLB is probably highest when the HRCT features are inconsistent with UIP, due to the frequent (39%) retrieval of patterns other than UIP.

     

间质性肺疾病与肺癌

间质性肺疾病(ILD)患者中肺癌的发病率增高,特别是特发性肺纤维化合并肺癌的报道最多.ILD合并肺癌的病理类型以鳞状细胞癌最多,好发于肺下叶和外周部位.ILD的一些肿瘤标记物增高,与疾病的活动程度、严重程度、肺纤维化程度和病情预后等有关,并且具有一定的诊断价值.ILD的肿瘤标记物增高可能是其合并肺癌的危险因素.本文对I...     

The prevalence of pulmonary fibrosis combined with emphysema in patients with lung cancer

Background and objective: Combined pulmonary fibrosis and emphysema (CPFE) is a unique disorder of the upper lobe, whereas emphysema is usually associated with lower lobe fibrosis. Although CPFE might increase the risk of lung cancer, the prevalence of CPFE in patients with lung cancer and the incidence of lung cancer in patients with CPFE are unknown. The objective of this study was to determine the prevalence of CPFE in lung cancer patients and to assess the clinical features of these patients. Methods: A total of 1143 patients with lung cancer were reviewed. Based on HRCT performed at diagnosis of lung cancer, patients were categorized into four groups: normal, emphysema, fibrosis and CPFE. The clinical characteristics of patients with CPFE were compared with those of the other groups. Results: CPFE, emphysema and fibrosis were identified in 101 (8.9%), 404 (35.3%) and 15 (1.3%) patients with lung cancer, respectively. The median overall survival of CPFE patients (n = 101, 10.8 months) was significantly less than that of normal patients (n = 623, 53.0 months) or that of patients with emphysema alone (n = 404, 21.9 months). Acute lung injury occurred in 20 (19.8%) patients with CPFE. Conclusions: CPFE is more prevalent than fibrosis in patients with lung cancer, and patients with CPFE had a poorer prognosis in the present study. Further investigation is therefore necessary to elucidate whether CPFE is an independent risk factor for lung cancer.     

Investigation burden for patients with fibrotic interstitial lung disease at the end of life

Fibrotic interstitial lung disease (f‐ILD) has a guarded prognosis, and the goal of therapy in advanced‐stage disease should be symptom‐based. Despite this, patients may still undergo burdensome investigation at the end of life. A retrospective audit was performed on 67 patients who died from f‐ILD at the Royal Melbourne and Austin Hospitals between 2012 and 2016. Increased investigation burden was associated with lack of outpatient palliative care referral and documented advance care plan, and admission to a high‐dependency unit. Eighteen per cent of patients underwent ongoing investigations after the institution of comfort care. These findings highlight the unmet end‐of‐life care needs of people with f‐ILD.     

Unclassifiable interstitial lung disease: A review

Accurate classification of interstitial lung disease (ILD) requires a multidisciplinary approach that incorporates input from an experienced respirologist, chest radiologist and lung pathologist. Despite a thorough multidisciplinary evaluation, up to 15% of ILD patients have unclassifiable ILD and cannot be given a specific diagnosis. The objectives of this review are to discuss the definition and features of unclassifiable ILD, identify the barriers to ILD classification and outline an approach to management of unclassifiable ILD. Several recent studies have described the characteristics of these patients; however, there are inconsistencies in the definition and terminology of unclassifiable ILD due to limited research in this population. Additional studies are required to determine the appropriate evaluation and management of patients with unclassifiable ILD.     

结缔组织病肺间质病变的临床特点及治疗效果分析

目的探讨不同结缔组织病肺间质病变（CTD-ILD）的临床特点和治疗效果。方法分析82例CTD-ILD患者的临床特征及胸部高分辨CT（HRCT）特点并观察治疗后HRCT的变化。结果（1）SSc—ILD的发生率最高，为70．0％。其次为pSS—ILD、PM／DM-ILD、RA—ILD、SLE—ILD，系统性红斑狼疮继发的ILD（SLE—ILD）发病年龄（26．2±7．8）较其他CTD-ILD早（P〈0．05）。RA—ILD、SSc—ILD病程较长，pSS—ILD、SLE一[LD和PM—ILl）病程较短。RA．ILD出现活动后气促少见（P〈0．05）。pSS—ILD出现呼吸道症状（40％）和体征（50％）最多见。85．7％SSc-ILD出现雷诺现象，支持雷诺现象与ILD相关。（2）SLE—ILD以磨玻璃影多见，丽蜂窝状改变较少；pSS-ILD以蜂窝状改变和纵隔淋巴结肿大相对较多。（3）大部分患者治疗后肺部间质病变吸收好转（56．7％），其中88．2％SLE—ILD吸收好转，28．4％患者肺部病变处于静止，14．9％患者间质病变较前进展。结论CTD—ILD患者一旦确诊，应尽早用激素联合免疫抑制剂治疗。