Combined pulmonary fibrosis and emphysema characteristics in a Greek cohort.

BACKGROUND: Combined pulmonary fibrosis and emphysema (CPFE) has recently received great attention, with studies suggesting that it presents a distinct clinical entity while others have challenged this hypothesis. This nationwide study aimed to describe a large cohort of Greek CPFE patients and to examine potential prognostic factors for survival. METHODS: This retrospective study included 97 patients with CPFE. Demographic and clinical data, pulmonary function tests, echocardiography results and bronchoalveolar lavage analysis were recorded. RESULTS: Most patients were male (94.8%) and 92% were current or ex-smokers. Spirometry results were abnormal (forced vital capacity (FVC) 72.9±19.9% pred and forced expiratory volume in 1 s/FVC 82.9±9.7%) with reduced diffusing capacity of the lung for carbon monoxide (D LCO) (42.3±17.4% pred). Mean systolic pulmonary arterial pressure was 41.9±19.7 mmHg and pulmonary hypertension was present in 58.8% of patients. Mean 6-min walk distance was 335.4±159.4 m. Mean emphysema score was 14.23±8.69% and mean interstitial lung disease (ILD) extent was 39.58±19.82%. Mean survival was 84 months (95% CI 72-96 months). Patients with D LCO ≥39% pred had better survival than patients with D LCO <39% pred (p=0.031). Patients with ILD extent ≥30% had worse survival than patients with ILD extent <30% (p=0.037). CONCLUSIONS: Our results indicate that CPFE patients have preserved lung volumes associated with disproportionately reduced D LCO, while reduced D LCO and increased ILD extent was associated with worse prognosis.

Prediction of hospitalization stay in COPD exacerbations: the AECOPD-F score.

BACKGROUND: Hospital admissions for COPD exacerbations account for 70% of total costs of COPD treatment, and the duration of hospital stay is directly related to this cost. The aim of this study was to investigate possible associations of demographic, clinical, laboratory, and functional parameters with stay of subjects admitted for COPD exacerbations and to provide a score for the prediction of the need for prolonged hospitalization. METHODS: We included 164 consecutive subjects admitted to 2 respiratory medicine departments of 2 tertiary hospitals for a COPD exacerbation, and we evaluated laboratory, clinical, and functional parameters possibly related to the duration of hospital stay. RESULTS: Seven parameters evaluated on subject admission (Antonisen type of exacerbation, number of Exacerbations in the previous year, Charlson index of comorbidities, Oxygenation, Partial pressure of P(aCO2) in arterial blood gases, Dyspnea according to the Borg dyspnea scale, and history of chronic respiratory Failure) were able to predict stay and were included in a simple score named AECOPD-F. The area under the curve of the score for the prediction of prolonged hospital stay is 0.960, and a cutoff point ≥ 3 predicts prolonged stay with a sensitivity of 84.5% and a specificity of 92.5% (95% CI 0.917-0.984). The AECOPD-F score was validated in a second group of 88 subjects admitted to the hospital for a COPD exacerbation. In the validation group, subjects with a score ≥ 3 required prolonged stay compared with those with a score < 3 (8.0 [6.0-10.0] vs 6.5 [4.0-9.0] d, respectively, P = .007). CONCLUSION: The AECOPD-F score could accurately predict stay in hospitalized COPD subjects. The implementation of this score in clinical practice could be useful in the discharge planning of such subjects.

Collateral damage: depressive symptoms in the partners of COPD patients.

BACKGROUND: Depression is a frequent comorbidity in COPD patients and is associated with greater physical impairment, increased health-care utilization, and worse outcomes. The presence of depressive symptoms in the partners of COPD patients has not been evaluated. METHODS: We evaluated the partners of 230 consecutive COPD patients included in a prospective study. Depressive symptoms were evaluated using Beck's Depression Inventory (BDI) on the first day of admission for COPD exacerbation. Patients were followed-up for 1 year. RESULTS: Significant depressive symptoms were present in 39.6 % of the COPD patients and in 40.9 % of their partners. Beck scores were higher in the partners of patients with severe airflow obstruction and in those with ≥2 exacerbations and ≥1 hospitalizations for COPD exacerbation during the 1-year follow-up. The BDI score of the patients' partners was significantly correlated with the BDI score of the COPD patients (r s = 0.422). In multivariate analysis, depressive symptoms in the COPD patients were an independent predictor of depressive symptoms in their partners (OR 4.136, 95 % CI 1.991-8.594; p < 0.001). CONCLUSIONS: A large proportion of the partners of COPD patients present significant depressive symptoms. The identification of those patients and their partners represents a possible target for intervention.

Concomitant sarcoidosis and a connective tissue disease: review of the clinical findings and postulations concerning their association.

INTRODUCTION: Known causes of granulomatous inflammation must be excluded before the diagnosis of sarcoidosis can be secured. We explored the possibility that connective tissue diseases (CTDs) could be a cause of granulomatous inflammation through an analysis of patients cared for in 2 medical centers and a review of the medical literature. METHODS: Patients with both a diagnosis of sarcoidosis and a CTD were identified at two medical centers. In addition, a literature search identified reported cases of patients with both diagnoses. RESULTS: We identified 15 patients at 2 medical centers plus 53 previous reported patients in the medical literature (total=68) with diagnoses of both sarcoidosis and a CTD. The patients were predominantly female. Scleroderma was the most common CTD. Only 2/59 (3%) with chest radiographs recorded had fibrocystic (stage 4) disease. In 48/67 (72%) patients where the time of diagnosis was recorded, sarcoidosis was diagnosed simultaneously or after the CTD. Sarcoidosis in only one organ ("isolated sarcoidosis") was associated with sarcoidosis being diagnosed simultaneously or after the CTD (p=0.0001). CONCLUSIONS: These data suggest that a significant portion of patients with CTDs and sarcoidosis may actually not have the latter disease. Rather, the CTD may "cause" granulomatous inflammation. Alternate explanations for these findings include that sarcoidosis and/or the connective tissue disease was misdiagnosed in these patients. The diagnosis of a concomitant connective tissue disease and sarcoidosis must be made with extreme caution.

The impact of depressive symptoms on recovery and outcome of hospitalised COPD exacerbations.

The impact of depressive symptoms on outcomes of acute exacerbations of chronic obstructive pulmonary disease (AECOPD) has not been thoroughly evaluated in prospective studies. We prospectively enrolled 230 consecutive patients hospitalised for AECOPD, without previous diagnosis of depression. Depressive symptoms were evaluated with Beck's depression inventory. Pulmonary function tests, arterial blood gases, COPD assessment test (CAT) and Borg dyspnoea scale were recorded on admission and on days 3, 10 and 40. Patients were evaluated monthly for 1 year. Patients with depressive symptoms required longer hospitalisation (mean ± sd 11.6 ± 3.7 versus 5.6 ± 4.1 days, p<0.001). Clinical variables improved during the course of AECOPD, but depressive symptoms on admission had a significant impact on dyspnoea (p<0.001) and CAT score (p = 0.012) improvement. Patients with depressive symptoms presented more AECOPD (p<0.001) and more hospitalisations for AECOPD (p<0.001) in 1 year. In multivariate analysis, depressive symptoms were an independent predictor of mortality (hazard ratio 3.568, 95% CI 1.302-9.780) and risk for AECOPD (incidence rate ratio (IRR) 2.221, 95% CI 1.573-3.135) and AECOPD hospitalisations (IRR 3.589, 95% CI 2.319-5.556) in 1 year. The presence of depressive symptoms in patients admitted for AECOPD has a significant impact on recovery and is related to worse survival and increased risk for subsequent COPD exacerbations and hospitalisations in 1 year.

Expression of hypoxia-inducible factor (HIF)-1a-vascular endothelial growth factor (VEGF)-inhibitory growth factor (ING)-4- axis in sarcoidosis patients.

BACKGROUND: Sarcoidosis is a granulomatous disorder of unknown etiology. The term of immunoangiostasis has been addressed by various studies as potentially involved in the disease pathogenesis. The aim of the study was to investigate the expression of the master regulator of angiogenesis hypoxia inducible factor (HIF)-1a - vascular endothelial growth factor (VEGF)- inhibitor of growth factor 4-(ING4) - axis within sarcoid granuloma. METHODS: A total of 37 patients with sarcoidosis stages II-III were recruited in our study. Tissue microarray technology coupled with immunohistochemistry analysis were applied to video-assisted thoracoscopic surgery (VATS) lung biopsy samples collected from 37 sarcoidosis patients and 24 controls underwent surgery for benign lesions of the lung. Computerized image analysis was used to quantify immunohistochemistry results. qRT-PCR was used to assess HIF-1a and ING4 expression in 10 sarcoidosis mediastinal lymph node and 10 control lung samples. RESULTS: HIF-1a and VEGF-ING4 expression, both in protein and mRNA level, was found to be downregulated and upregulated, respectively, in sarcoidosis samples compared to controls. Immunohistochemistry coupled with computerized image analysis revealed minimal expression of HIF-1a within sarcoid granulomas whereas an abundant staining of ING4 and VEGF in epithelioid cells was also visualized. CONCLUSIONS: Our data suggest an impairment of the HIF-1a - VEGF axis, potentialy arising by ING4 overexpression and ultimately resulting in angiostasis and monocyte recruitment within granulomas. The concept of immunoangiostasis as a possible protection mechanism against antigens of infectious origin needs further research to be verified.

Airway involvement in sarcoidosis.

Sarcoidosis is a common disease and affects the respiratory system in > 90% of cases, most commonly the intrathoracic lymph nodes and the respiratory parenchyma. Less commonly, the airways are involved, and the disease is manifested as mucosal erythema, edema, granularity and cobblestoning, plaques, nodules, and bronchial stenosis, airway distortion, traction bronchiectasis, and bronchiolitis. Airway involvement may lead to airflow limitation. Involvement of oral, nasal, and pharyngeal mucosa may cause hoarseness, dysphagia, laryngeal paralysis, and upper airway obstruction. Airway symptoms are important indicators of airway involvement in sarcoidosis. Pulmonary function testing, radiologic imaging, and bronchoscopy occupy a significant role in the diagnosis and management of airway involvement in patients with sarcoidosis.

A massive hemorrhagic pleural effusion does not exclude the diagnosis of tuberculosis: a case report.

We report a case of an immunocompetent 18-year-old man with a massive hemorrhagic, exudative, lymphocytic pleural effusion. Blind transthoracic pleural biopsy showed granuloma formation, while the pleural fluid culture was positive for Mycobacterium tuberculosis, confirming the diagnosis of primary tuberculous pleuritis. A massive hemorrhagic pleural effusion is extremely rare in tuberculosis, but tuberculosis is a very protean disease and should always be included in the differential diagnosis of pleural effusions.

Primary monophasic synovial sarcoma presenting as a pulmonary mass: a case report.

INTRODUCTION: Primary pulmonary synovial sarcoma is an extremely rare tumor with only few case reports in the literature. CASE PRESENTATION: A healthy 67-year-old woman was admitted for investigation of a pulmonary mass found on a routine X-ray. She had a history of breast cancer diagnosed and treated 13 years previously with left mastectomy followed by adjuvant endocrine therapy. No progression of the disease was reported. Thoracic computer tomography disclosed a soft-tissue mass in the lower lobe of the left lung arising in the vicinity of the pleura. No abnormal lymph nodes were noted. Further work-up for metastases was negative. Subsequently, the lower lobe of the left lung was removed and the diagnosis was a monophasic synovial sarcoma. CONCLUSION: The diagnosis of monophasic primary pulmonary synovial sarcoma requires clinical, imaging and immunohistochemical investigation to exclude alternative primary sources. The treatment of choice is excision (lobectomy or pneumonectomy), which in most of cases is helpful for diagnosis. The prognosis is usually poor.

Airway involvement in Wegener's granulomatosis.

Wegener's granulomatosis is characterized by necrotizing granulomatous inflammation and necrotizing vasculitis affecting predominantly small arteries, arterioles, capillaries, and venules. In contrast to the well-described pulmonary parenchymal involvement of Wegener's granulomatosis, the lower airway (tracheobronchial) disease manifestations are less well recognized by clinicians. Consequently, mild disease of the airways is easily missed. There is a relative paucity of published information on various tracheobronchial manifestations of Wegener's granulomatosis. This article provides a comprehensive review of the diagnosis and management of the infraglottic tracheobronchial disease manifestations.

Sleep-related breathing disorders in patients with idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a chronic and usually fatal lung disease of unknown etiology. The aim of this study was to describe clinical and polysomnographic features of sleep-related breathing disorders (SRBD) and to identify predictors of obstructive sleep apnea (OSA) in IPF patients. Eight hundred fifty-seven patients with IPF were admitted to the Cleveland Clinic from 2001 to 2005. An all-night polysomnogram (PSG) was performed in 18 of them to investigate complaints suggestive of sleep-disordered breathing. OSA was confirmed in 11 of the 18 IPF patients with complaints suggestive of sleep apnea, while the remain 7 patients had a diagnosis of primary snoring or upper airway resistance syndrome (UARS). All patients showed a reduction in sleep efficiency, REM sleep, and slow wave sleep. The apnea-hypopnea index (AHI) was positively correlated with body mass index (p < 0.0001, r = 0.80). The REM AHI and overall AHI were negatively correlated with FEV(1) (p = 0.008, r = -0.59 and p = 0.04, r = -0.49, respectively) and FVC percentages (p = 0.03, r = -0.50 and p = 0.08, r = -0.42, respectively). Our study is the first describing SRBD in IPF patients. An increased BMI and a significant impairment in pulmonary function testing may be predictors of OSA in this population. In the absence of effective treatments for IPF, the diagnosis and treatment of comorbid SRBD may lead to improvements in quality of life.