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Dyspnoea is a cardinal symptom of fibrotic interstitial lung disease (ILD), with a lack of proven effective therapies. With emerging evidence of the role of facial and nasal airflow for relieving breathlessness, this pilot study was conducted to examine the feasibility of conducting a clinical trial of a handheld fan (HHF) for dyspnoea management in patients with fibrotic ILD. In this mixed-methods, randomised, assessor-blinded, controlled trial, 30 participants with fibrotic ILD who were dyspnoeic with a modified Medical Research Council Dyspnoea grade ≥ 2 were randomised to a HHF for symptom control or no intervention for 2 weeks. Primary outcomes were trial feasibility, change in Dyspnoea-12 scores at Week 2, and participants' perspectives on using a HHF for dyspnoea management. Study recruitment was completed within nine months at a single site. Successful assessor blinding was achieved in the fan group [Bang's Blinding Index - 0.08 (95% CI - 0.45, 0.30)] but not the control group [0.47 (0.12, 0.81)]. There were no significant between-group differences for the change in Dyspnoea-12 or secondary efficacy outcomes. During qualitative interviews, participants reported that using the HHF relieved breathlessness and provided relaxation, despite initial scepticism about its therapeutic benefit. Oxygen-experienced participants described the HHF being easier to use, but not as effective for symptomatic relief, compared to oxygen therapy. Our results confirmed the feasibility of a clinical trial of a HHF in fibrotic ILD. There was a high level of patient acceptance of a HHF for managing dyspnoea, with patients reporting both symptomatic benefits and ease of use.

In the 2013 updated classification of the American Thoracic Society/European Respiratory Society, airway-centered interstitial fibrosis (ACIF) is included as a bronchiolocentric pattern of interstitial pneumonia (IP) among idiopathic IPs. We encountered a case of severe pulmonary hypertension (PH) with chronic IP. The patient initially presented with shortness of breath and often lost consciousness due to PH, and seven years after his first visit, he ultimately died. An autopsy revealed ACIF and usual IP. In particular, the ACIF comprised non-atypical smooth muscle hyperplasia, and pulmonary hypertensive vascular degeneration was detected. This case may represent a new pathological feature of ACIF.

Introduction: Interstitial lung diseases (ILDs) include a wide variety of chronic progressive pulmonary diseases characterized by lung inflammation, fibrosis and hypoxemia and can progress to respiratory failure and even death. ILDs are associated with varying degrees of quality of life impairments in affected people. Studies on the quality of life in patients with ILDs are still limited, and there are few studies with long-term follow-up periods in these patients., Methods: Data from patients who were clinically diagnosed with ILDs in the Respiratory Department, Beijing Chaoyang Hospital, Capital Medical University from January 2017 to February 2018 were collected. Clinical status and HRQoL were assessed at baseline and subsequently at 6- and 12-month intervals with the LCQ, mMRC, HADS, SF-36, and SGRQ. Multivariate linear regression was used to evaluate the determinants of the decline in HRQoL., Results: A total of 139 patients with idiopathic interstitial pneumonia (IIP) and 30 with connective tissue disease-associated ILD (CTD-ILD) were enrolled, 140 of whom completed the follow-up. The mean age was 63.7 years, and 92 patients were men. At baseline, the decline in HRQoL assessed by the SF-36 and SGRQ was significantly associated with the mMRC, LCQ and HADS depression score. In the follow-up, changes in FVC%, DLco%, mMRC and LCQ were significantly associated with changes in HRQoL., Conclusions: HRQoL in both IIP and CTD-ILD patients deteriorates to varying degrees, and the trend suggests that poor HRQoL in these patients is associated with many determinants, primarily dyspnea, cough and depression. Improving HRQoL is the main aim when treating patients living with ILDs., Competing Interests: The authors have declared that no competing interests exist.

An 81-year-old woman with rheumatoid arthritis (RA) who had been treated with bucillamine presented with dyspnea. Computed tomography of the chest showed ground-glass opacities and consolidations in both lungs and honeycombing in both basal lung areas. An elevation of the serum Krebs von den Lungen-6 level and hypoxemia were seen. Lymphocytosis with a decreased CD4/CD8 ratio was seen in the bronchoalveolar lavage fluid. A transbronchial lung biopsy specimen showed organizing pneumonia. Based on a diagnosis of bucillamine-induced pneumonitis (BIP) with RA-associated pre-existing interstitial pneumonia, she was successfully treated with the cessation of bucillamine and systemic corticosteroid therapy. The risk factors and prognosis of BIP are discussed.

A 63-year-old man presented with persistent cough and progressive dyspnea. Computed tomography showed irregular pleural thickening and fibrotic changes with volume loss in the upper lobes, and subtle reticulation in the lower lobes. Pleuroparenchymal fibroelastosis (PPFE) was diagnosed based on the findings of a surgical lung biopsy. Bronchiolar lesions, including proliferative bronchiolitis, constrictive bronchiolitis obliterans, and peribronchiolar metaplasia were evident on pathology. A usual interstitial pneumonia (UIP) pattern was also observed in the lower lobes. Three weeks after the biopsy, an acute exacerbation occurred. We herein describe a rare case of idiopathic PPFE with various bronchiolar lesions and a UIP pattern in which an acute exacerbation developed.

Pulmonary rehabilitation is recommended for patients with chronic lung diseases including idiopathic pulmonary fibrosis according to international guidelines. However, data for patients with interstitial lung disease (ILD) are limited. We examined the effect of an inpatient pulmonary rehabilitation on functional status and quality of life in ILD patients. We evaluated 402 consecutive ILD patients who were admitted to a specialised pulmonary rehabilitation centre (1999-2010). All patients performed a standardised pulmonary rehabilitation programme including pulmonary function tests, blood-gas analysis, 6-min walk test (6MWT), dyspnoea rating and health-related quality of life questionnaire (the 36-item short-form health survey; SF-36) on admission and discharge. Mean duration of pulmonary rehabilitation was 30±1 days. 6MWT distance improved by 46±3 m (308±6 m versus 354±6 m; p<0.001). Dyspnoea rating did not change. Lung function testing showed marginal improvement of vital capacity (+1±0%; p = 0.002). The SF-36 questionnaire demonstrated an increase in all eight sub-scores as well as in the physical and mental health summary scores (physical 6±1 points, p<0.001; mental health 10±1 points, p<0.001). Moreover, patients with signs of pulmonary hypertension also benefited from pulmonary rehabilitation. In a large cohort of patients with ILD, pulmonary rehabilitation had a positive impact on functional status and quality of life. Considering the limited treatment options in this patient population pulmonary rehabilitation appears to be a valuable adjunct therapy.

Objective: To report a case of pulmonary surfactant protein (SP) gene mutation associated with pediatric interstitial lung disease, and study the clinical diagnosis process and review of related literature, to understand the relationship between interstitial lung disease and SP gene mutation in infants and children., Method: The clinical, radiological, histological, and genetic testing information of a case of SP gene mutation related pediatric interstitial lung disease were analyzed and related literature was reviewed., Result: A 2-year-old girl without a history of serious illness was hospitalized because of the shortness of breath, cough, excessive sputum, and the progressive dyspnea. Physical examination on admission revealed tachypnea, slight cyanosis, and the retraction signs were positive, respiratory rate of 60 times/minute, fine crackles could be heard through the lower lobe of both lungs; heart rate was 132 beats/minute. No other abnormalities were noted, no clubbing was found. Laboratory test results: pathologic examination was negative, multiple blood gas analysis suggested hypoxemia. Chest CT showed ground-glass like opacity, diffused patchy infiltration. Bronchoalveolar lavage fluid had a large number of neutrophils, and a few tissue cells. Eosinophil staining: negative. Fluconazole and methylprednisolone were given after admission, pulmonary symptoms and signs did not improve, reexamination showed no change in chest CT. Then lung biopsy was carried out through thoracoscopy. Histopathology suggested chronic interstitial pneumonia with fibrosis. The heterozygous mutation of R219W in the SFPTA1 and the S186N in SFTPC were identified by SP-related gene sequencing. The review of related literature showed that polymorphisms at the 219th amino acid in SP-A1 allele were found in adults with idiopathic pulmonary fibrosis (IPF), but there is no related literature in pediatric cases. The patient in this report had a mutation at the SP-A1 allele consistent with related literature. Data of 17 young children with mutation in SP-C gene showed that all the 17 cases had dyspnea and tachypnea, chest CT revealed diffuse opacities in lungs, the pathology of lungs was NSIP and CPI. There were 17 kinds of mutation and the common mutation was I73T. The mutation of S186N in SFTPC in our case has never been shown in previously published literature., Conclusion: A case of interstitial lung disease with S186N gene mutation in SFTPC was preliminarily diagnosed in an infant. The SP-C gene mutations and polymorphisms are associated with pediatric interstitial lung disease.

Interstitial lung diseases (ILD) constitute a large group of disorders characterized by various etiology and pathogenesis. Inflammation and pulmonary fibrosis are the most important processes in the course of ILD. Disease causes the decrease of the gas diffusion in the lungs and provokes hypoxia. Chronic inflammation and hypoxia are strong stimulus of neovascularization. Neoangiogenesis is a principal response of vessels to inflammation. The critical importance of tumor angiogenesis in the development and metastatic spread of tumors is proved. Relations of ILD with neoplasma have been observed. Neovascularization takes an important role in wound healing allowing the cells to flow into damaged structures. Recently, pulmonary fibrosis has been deemed to result from abnormal wound healing in the lung in response to injury to the alveolar epithelium. Angiogenesis participates in pathogenesis of idiopathic pulmonary fibrosis. More and more data suggest the role of angiogenesis in pathogenesis of other ILDs, such as granulomatosis, fibrosis and vasculitis. The mechanism of angiogenesis in ILD is not clear yet. New data concerning participation of neoangiogenesis in pathogenesis of ILD created target for new drugs. Thalidomide, a strong antiangiogenic drug was used successfully in the some cases of ILD.

Fabry disease affects multiple organs, including the lungs. To date, there have been no reports of the effect of enzyme replacement therapy on patients with pulmonary manifestations of the disease. We report on a case of marked improvement of pulmonary signs and symptoms in a patient with Fabry disease following enzyme replacement therapy. A 38-year-old female with Fabry disease presented with dyspnea requiring constant supplemental oxygen. Following bi-weekly treatment with agalsidase beta, she demonstrated marked improvement in her pulmonary symptoms, eventually no longer needing supplemental oxygen and returning to an active lifestyle. Improvement was seen on pulmonary function tests and chest CT examinations. Enzyme replacement therapy may alleviate pulmonary dysfunction in patients with Fabry disease., ((c) 2007 Wiley-Liss, Inc.)

The objective of this study was to evaluate the risk factors associated with mortality in interstitial lung disease patients. We performed a retrospective study of 722 consecutive patients submitted for lung biopsy during the 1986-1990 period. Twenty-two (3%) died within the 30 days following surgery. Forty-four patients who survived after the surgery for the same time span were randomly chosen as control group. Dyspnea at rest was present in 18/44 of surviving group (SG) and in 18/22 of the nonsurviving group (NSG) (OR 6.5, 95% CI 1.8-22.4,p = .001). Systemic diseases (i.e., diabetes, systemic arterial hypertension)were mainly present in the NSG (OR 7.2, 95% CI 2.3-22.8, p < .001). The SG displayed significantly less respiratory insufficiency with a PaO2 of 52.2 + 8.4 versus 38.5 i 9.4 mm Hg, and PaCO2 of 28.8 i 4.5 versus 38.5 +/- 9.2 mm Hg, respectively (p < .001). Likewise, the SG exhibited a PaCO2/PaO2 ratio of 0.5 - 0.1, while in the NSG it was of 1 +/- 0.4 (p < .001), showing a sensitivity of 84% and specificity of 93% for mortality. Multiple logistic regression analysis for these variables showed that log likelihood was still significant for PaCO2 > 34 mm Hg, PaO2 <48 mm Hg, and comorbid diseases. Logistic regression analysis of these three variables showed the greatest sensitivity and specificity (84 and 750/0,respectively) for prediction of mortality. However, the strongest association was found when PaCO2/PaO2 ratio was analyzed alone (OR 21,073,CI 95% 28-15,946,357, p < .005). These data suggest that PaCO2/PaO2 ratio appears to be a predictor of mortality in this subset of patients. Its prospective use has reduced early mortality after surgery less than 1% in the last decade.

The aim of this study was to evaluate the safety and efficacy of azathioprine in the treatment of interstitial lung disease (ILD) associated with systemic sclerosis (SSc). The records of patients with SSc with ILD who were treated with azathioprine were reviewed. Patients were treated with azathioprine and low-dose prednisone if they had progressive pulmonary symptoms (deterioration in the dyspnea score) or poor or deteriorating lung function. Response was classified as improved if the FVC increased more than 10% from baseline, and stable if it remained within 10% of baseline. Serial dyspnea scores were recorded. Eleven patients were treated with azathioprine, three of whom received treatment for 6 months or less owing to adverse effects (nausea, leukopenia and pulmonary tuberculosis in one patient each). The remaining eight patients received at least 12 months' treatment and the results suggested an improvement in the mean percent predicted FVC from a baseline value of 54.25+/-3.53 to 63.38+/-6.15 after 12 months ( p=0.101). Overall, five patients improved and three remained stable. The mean dyspnea score ( n=8) improved from a baseline of 1.55+/-0.19 to 0.50+/-0.19 at 12 months ( p=0.011). This is the first case series of patients with SSc-associated ILD treated with azathioprine. Our results suggest that azathioprine may have a role in stabilizing lung function and improving symptoms in SSc, although this needs confirmation by a randomized controlled trial.

Eleven subjects, aged between 15 and 60 years, presenting with diffuse infiltrative lung disease (DILD) and progressive dyspnoea, underwent an open lung biopsy (OLB). The authors feel that OLB does give a confidence to the treating physician to begin with a specific therapy in the form of steroids. But, as a matter of fact, at most health care delivery centres in the country, facilities for OLB are not available. Hence, the specific therapy should be instituted presumptively following an overall suggestion of disease based upon the clinical, physiological (chiefly comprising the pulmonary function test or PFT) and the radiological criteria, so that progression of disease could be arrested at an early stage.