Idiopathic pulmonary fibrosis (IPF) is the most common interstitial lung disease (ILD) of unknown causes, which is characterized by pulmonary fibrosis. The median survival period after diagnosis is about 2-4 years. In recent years, the incidence rate of lung cancer associated with IPF (IPF-LC) is increasing, and the prognosis is worse than that of IPF alone. Pulmonary fibrosis may be closely associated with the occurrence and development of lung cancer. Although the pathogenesis of IPF-LC is still unclear, the current research shows that there are similarities between the pathogenesis of these two diseases at molecular and cellular levels. At present, the research on the cellular and molecular mechanism of lung cancer related to pulmonary fibrosis has become the focus of researchers' attention. This article reviews the related literature, focusing on the latest status of the cellular and molecular mechanisms and treatment of IPF-LC, hoping to help clinicians understand IPF-LC.
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Humans ; Idiopathic Pulmonary Fibrosis/complications* ; Lung Neoplasms/complications*

BACKGROUNDCombined emphysema and pulmonary fibrosis, including idiopathic pulmonary fibrosis (IPF), is a distinct disorder described with upper-lobe emphysema and lower-lobe fibrosis on chest computed tomography. Smoking appears to be the predominant risk factor for this disorder. We aimed to compare clinical features, smoking history, physiological and radiological findings between IPF with and without emphysema.

METHODSA sample of 125 IPF patients over a period of 48 months were evaluated. High resolution CT scans were reviewed blinded to clinical data. The IPF patients with or without emphysema were classified accordingly.

RESULTSThe prevalence of emphysema in this IPF sample was 70/125. IPF with emphysema was significantly associated with smoking status (OR 63; 95% CI 4.4 to 915; P = 0.002) and smoking pack year (OR 1.1; 95% CI 1.05 to 1.13; P = 0.000). The patients with IPF and emphysema had a higher decrease in carbon monoxide diffusing capacity adjusted for alveolar volume ((58±19)% pred vs. (66±21)% pred; P = 0.021) and a higher prevalence of pulmonary hypertension (24/70 vs. 7/55; P = 0.006). The two groups of patients had similar forced and residual volumes. No significant differences were found in cell differentials of bronchoalveolar lavage or the scores of fibrosis on chest CT. Survival of the patients with emphysema was significantly less than that of patients with IPF alone.

CONCLUSIONSCigarette smoking induces IPF combined with emphysema. Emphysema further impairs physiological function and increases the prevalence of pulmonary hypertension that leads to poor prognosis. The inclusion of the patients with combined pulmonary fibrosis and emphysema in IPF clinical trials may lead to under evaluation of the effect of treatment in patients.

Aged ; Female ; Humans ; Idiopathic Pulmonary Fibrosis ; etiology ; physiopathology ; Male ; Middle Aged ; Pulmonary Emphysema ; complications ; physiopathology ; Smoking ; adverse effects

OBJECTIVETo review the studies investigating the increased risk of lung cancer in patients with idiopathic pulmonary fibrosis (IPF).

DATA SOURCESData cited in this review were obtained mainly from PubMed and Medline from 1999 to 2013 and highly regarded older publications were also included.

STUDY SELECTIONWe identified, retrieved and reviewed the information on the frequency, risk factors, anatomical features, histological types, clinical manifestations, computed tomography findings and underlying mechanisms of lung cancer in IPF patients.

RESULTSThe prevalence rates of lung cancer in patients with IPF (4.8% to 48%) are much higher than patients without IPF (2.0% to 6.4%). The risk factors for lung cancer in IPF include smoking, male gender, and age. Lung cancers often occur in the peripheral lung zones where fibrotic changes are predominant. Adenocarcinoma and squamous cell carcinoma are the most common types of lung cancer in patients with IPF. Radiologic features of these patients include peripherally located, ill-defined mass mimicking air-space disease. The underlying mechanisms of the development of lung cancer in patients with IPF have not been fully understood, but may include the inflammatory response, epithelial injury and/or abnormalities, aberrant fibroblast proliferation, epigenetic and genetic changes, reduced cell-to-cell communication, and activation of specific signaling pathways.

CONCLUSIONSThese findings suggest that IPF is associated with increased lung cancer risk. It is necessary to raise the awareness of lung cancer risk in IPF patients among physicians and patients.

Age Factors ; Female ; Humans ; Idiopathic Pulmonary Fibrosis ; complications ; epidemiology ; genetics ; Lung Neoplasms ; epidemiology ; etiology ; genetics ; Male ; Risk Factors ; Sex Factors

BACKGROUNDThe factors affecting the outcome of patients referred for lung transplantation (LTx) still have not been investigated extensively. The aim of this study was to characterize the patient outcomes and identify the prognostic factors for death while awaiting the LTx.

METHODSFrom January 2003 to November 2013, the clinical data of 103 patients with end-stage lung disease that had been referred for LTx to Department of Thoracic Surgery, Shanghai Pulmonary Hospital were analyzed retrospectively. The relationship between predictors and survival was evaluated using the Kaplan-Meier method and the Cox proportional hazards model.

RESULTSTwenty-five patients (24.3%) died while awaiting the LTx. Fifty patients (48.5%) underwent LTx, and 28 patients (27.2%) were still on the waitlist. Compared to the candidates with chronic obstructive pulmonary disease (COPD), patients with idiopathic pulmonary fibrosis (IPF) had a higher mortality while awaiting the LTx (40.0% vs. 12.3%, P = 0.003). Patients requiring mechanical ventilation (MV) had a higher mortality while waiting than others (50.0% vs. 20.2%, P = 0.038). Two variables, using MV and IPF but not COPD as primary disease, emerged as significant independent risk factors for death on the waitlist (hazard ratio [HR] = 56.048, 95% confidence interval [CI]: 3.935-798.263, P = 0.003 and HR = 14.859, 95% CI: 2.695-81.932, P = 0.002, respectively).

CONCLUSIONThe type of end-stage lung disease, pulmonary hypertension, and MV may be distinctive prognostic factors for death while awaiting the LTx.

Adult ; Aged ; Female ; Humans ; Hypertension, Pulmonary ; complications ; Idiopathic Pulmonary Fibrosis ; mortality ; Lung Diseases ; mortality ; Lung Transplantation ; statistics & numerical data ; Male ; Middle Aged ; Pulmonary Disease, Chronic Obstructive ; mortality ; Retrospective Studies ; Waiting Lists

OBJECTIVETo study the pathologic characteristics of chronic hypersensitivity pneumonitis, especially the pattern of pulmonary interstitial fibrosis; and to compare the histologic features with those of idiopathic interstitial pneumonitis.

METHODSThe HE-stained paraffin sections of 10 cases of chronic hypersensitivity pneumonitis encountered during the period from 2000 to 2008 were retrospectively analyzed.

RESULTSThere were altogether 6 males and 4 females, with age of patients ranging from 23 to 59 years (mean=47.2 years). Clinically, the patients presented with chronic cough and shortness of breath for 4 months to 6 years. Histologically, 7 cases showed usual interstitial pneumonitis (UIP)-like fibrosis. Patchy fibrosis was observed under the pleura, adjacent to interlobular septa and around bronchioles. In all of the 7 cases, foci of fibroblastic proliferation, as well as bronchiolar metaplasia of peribronchiolar alveoli and mild bronchiolitis, were noted. Three cases presented with mild honeycomb changes of lung and 3 cases showed non-specific interstitial pneumonitis (NSIP)-like fibrosis, in which the alveolar septa were expanded by fibrous tissue and collagen, with relative preservation of alveolar architecture. Bronchiolitis and lymphocytic infiltrates in alveolar septa were seen. Schaumann bodies were identified in 1 case. In general, patients with chronic hypersensitivity pneumonitis were younger than patients with idiopathic UIP. Computed tomography often showed upper and middle lobar involvement and mosaic attenuation. Compared with idiopathic UIP, the UIP-like fibrosis of chronic hypersensitivity pneumonitis often occurred not only under the pleura and adjacent to interlobular septa, but also around bronchioles and was accompanied by bronchiolar metaplasia.

CONCLUSIONSChronic hypersensitivity pneumonitis can mimic other types of lung conditions with interstitial fibrosis, especially UIP and NSIP. As a result, some cases of chronic hypersensitivity pneumonitis may be misdiagnosed as such.

Adult ; Alveolitis, Extrinsic Allergic ; complications ; pathology ; Chronic Disease ; Diagnostic Errors ; Female ; Humans ; Idiopathic Pulmonary Fibrosis ; etiology ; pathology ; Lung Diseases, Interstitial ; pathology ; Male ; Middle Aged ; Pulmonary Alveoli ; pathology ; Young Adult

Evidence suggests that diabetes mellitus (DM) is associated with idiopathic pulmonary fibrosis (IPF). According to the new IPF guidelines, high-resolution computed tomography (HRCT) is an essential means of diagnosing IPF. We investigated the relationship between IPF and DM in patients treated between 2003 and 2007. Newly diagnosed IPF patients in large university teaching hospitals in Korea were enrolled from January 2003 to December 2007. We retrospectively analyzed 1,685 patients using the interstitial lung disease (ILD) registry. In total, 299 IPF patients (17.8%) also had DM. The mean age of our subjects was 68.0 +/- 9.4 yr. HRCT showed significantly more reticular and honeycomb patterns in IPF patients with DM than in IPF patients without DM (P = 0.014, P = 0.028, respectively). Furthermore, significantly higher incidences of hypertension, cardiovascular diseases, and other malignancies (except lung cancer) were found in IPF patients with DM than in IPF patients without DM. In conclusion, IPF patients with DM are more likely to have the usual interstitial pneumonia (UIP) pattern, including reticular and honeycomb patterns, on HRCT than are those without DM.
Aged ; Cardiovascular Diseases/epidemiology/etiology ; Diabetes Mellitus, Type 2/*complications ; Female ; Humans ; Hypertension/epidemiology/etiology ; Idiopathic Pulmonary Fibrosis/complications/*diagnosis/radiography ; Incidence ; Male ; Middle Aged ; Neoplasms/epidemiology/etiology ; Registries ; Republic of Korea/epidemiology ; Retrospective Studies ; Tomography, X-Ray Computed

Patients with idiopathic pulmonary fibrosis (IPF) have an increased risk for developing tuberculosis (TB). However, no studies have been reported regarding the development of nontuberculous mycobacterium (NTM) lung disease (NTMLD). We reviewed 795 patients with IPF from five university hospitals who were diagnosed by histological or radio-clinical criteria. In the 795 patients with IPF, pulmonary infections with mycobacterium tuberculosis (MTB) and NTM were found in 35 (4.4%) and 16 patients (2.0%), respectively, which was a higher frequency than that found in the general population. TB was more common in patients treated with immunosuppressants than in those who did not receive immunosuppressants (2.6% vs 1.4%, P = 0.12). Among the IPF patients who had mycobacterial infections,immunosuppressant users developed TB or NTMLD within 1 yr after treatment with immunosuppressants,while those occurred later than 2 yr after diagnosis of IPF in the subjects that did not receive immunosuppressants. Among 51 IPF patients who had mycobacterial infections, 9 (18%) died during follow-up. Of these, three died due to progression of pulmonary tuberculosis. TB and NTMLD is relatively common in patients with IPF in Korea and may be fatal in some groups. Careful evaluation of TB and NTMLD is necessary not only for immunosuppressant users, but also for nonusers with IPF.
Aged ; Cohort Studies ; Female ; Follow-Up Studies ; Hospitals, University ; Humans ; Idiopathic Pulmonary Fibrosis/complications/*diagnosis/pathology ; Immunosuppressive Agents/therapeutic use ; Male ; Middle Aged ; Mycobacterium Infections/complications/*diagnosis/drug therapy ; Mycobacterium Infections, Nontuberculous/*diagnosis/drug therapy/pathology ; Retrospective Studies ; Tuberculosis, Pulmonary/complications/*diagnosis/pathology