The concept on idiopathic pulmonary fibrosis (IPF) pathogenesis has progressed from chronic inflammation to aberrant wounding healing and even more to the current paradigms of a multifactorial and heterogeneous disease process. Despite the growth of clinical trials for IPF, most of the results, including N-acetylcysteine combination, warfarin, and bosentan, were disappointing. On the other hand, there have been a number of important developments; the foremost is the licensing of pirfenidone in Europe and Asia. In this article, we briefly review the recent knowledge of pathogenesis of IPF. We also summarize the recent clinical trials regarding the management of IPF.
Background: Fractional exhaled nitric oxide (FeNO) can be measured easily, rapidly, and noninvasively for the assessment of airway inflammation, particularly mediated by eosinophil, such as asthma. In bronchiectasis (BE),the pathogenesis has been known as chronic airway inflammation and infection with abnormal airway dilatation; however, there are little studies to evaluate the role of FeNO in BE. Methods: From March 2010 to February 2012, 47 patients with BE, diagnosed by high resolution computed tomography (HRCT), performed FeNO, compared with asthma and chronic obstructive pulmonary disease (COPD). All patients carried out a complete blood count including eosinophil count, chemistry, sputum examination, and spirometry, if indicated. A retrospective analysis was performed to elucidate the clinical role of FeNO in BE patients. Results: The mean FeNO levels in patients with BE was 18.8±1.5 part per billion (ppb), compared to 48.0±6.4 and 31.0±4.3 in those with asthma and COPD, respectively (p＜0.001). The FeNO levels tended to increase along with the disease severity scores by HRCT; however, it was statistically not significant. FeNO in BE with a co-infection of nontuberculous mycobacteria was the lowest at 17.0±3.5 ppb among the study population. Conclusion: FeNO in BE was lower than other chronic inflammatory airway diseases, particularly compared with asthma. For clinical application of FeNO in BE, more large-scaled, prospective studies should be considered.
Background: Differentiating cardiogenic pulmonary edema from other bilateral lung diseases such as pneumonia is frequently difficult. We conducted a retrospective study to identify predictors for cardiogenic pulmonary edema and non-cardiogenic causes of bilateral lung infiltrates in chest radiographs. Methods: The study included patients who had newly developed bilateral lung infiltrates in chest radiographs and patients who underwent echocardiography. Cases were divided into two groups based on the echocardiographic findings: the cardiogenic pulmonary edema group and the non-cardiogenic group. Clinical characteristics and basic laboratory findings were analyzed to identify predictors for differential diagnosis between cardiogenic and non-cardiogenic causes of bilateral chest infiltrates. Results: We analyzed 110 subjects. Predictors of cardiogenic pulmonary edema were higher brain natriuretic peptide (BNP) levels, lower C-reactive protein (CRP) levels on the day of the event (＜7 mg/dL), age over 60 years, history of heart disease, and absence of fever and sputum. CRP on the day of the event was an independent factor to differentiate cardiogenic and non-cardiogenic causes of newly developed bilateral chest infiltrates. Also, the validity was comparable to BNP. Conclusion: Clinical symptoms (sputum and fever), medical history (dyslipidemia and heart disease), and laboratory findings (BNP and CRP) could be helpful in the differential diagnosis of patients with acute bilateral lung infiltrates in chest radiographs.
Multicentric Castleman's disease (CD) is a rare atypical lymphoproliferative disorder, which is characterized by various systemic manifestations. Some patients with multicentric CD may have concomitant lung parenchymal lesions, for which lymphoid interstitial pneumonia (LIP) is known to be the most common pathologic finding. Follicular bronchiolitis and LIP are considered to be on the same spectrum of the disease. We describe a case of multicentric CD with pulmonary involvement, which was pathologically proven as follicular bronchiolitis.
We present a case of congenital cystic adenomatoid malformation (CCAM) in a 25-year-old male who was presented with chronic cough. Chest radiography revealed an abnormal mass-like shadow in the right lower pulmonary zone. A contrast enhanced computed tomography showed an 11 cm solid, cystic mixed mass on the right lower lobe. A right lower lobectomy was performed by video-assisted thoracoscopic surgery without complications. The gross specimen showed a massive cavitation with multiloculated cysts of varying size, consistent with CCAM, along with noticeable granulomatous inflammation. Non-tuberculosis mycobacteria were isolated from a bronchial wash specimen, and the resected tissue homogenates were positive for Mycobacterium avium-intracellulare complex by polymerase chain reaction.
A 43-year-old woman with breast cancer who was on neoadjuvant chemotherapy presented with cough, sputum and mild fever. High-resolution computed tomography showed diffuse ground glass opacities in bilateral lungs and subpleural patchy consolidations. Initially, she was thought to have pneumonia or interstitial lung diseases such as drug-induced pneumonitis and treated with antibiotics and steroids. She subsequently got breast cancer surgery because of disease progression, and concurrent thoracoscopic lung biopsy revealed metastatic carcinoma of the lung from breast cancer. The diagnosis of suspected interstitial lung disease can be made without lung biopsy, but malignancy should always be considered and lung biopsy should be performed in the absence of a definitive clinical diagnosis.
Epithelial growth factor receptor (EGFR) tyrosine kinase inhibitors (TKIs) have been widely used for non-small-cell lung cancer patients. Its untoward cutaneous effects are largely well known and developed in many patients treated with EGFR TKIs. However trichomegaly of eyelash is rarely reported. Although trichomegaly is not a drug-limiting side effect, it could be troublesome of continuing the treatment because of cosmetic issue or eyeball irritation by long eyelashes. Therefore clinicians are needed to pay attention to this uncommon effect. We herein describe erlotinib induced trichomegaly of eyelashes in a woman with adenocarcinoma of the lung.