Abnormal Pulmonary Function Tests Research Articles

Interstitial Lung Disease in Patients with Primary Biliary Cirrhosis

Primary biliary cirrhosis (PBC) is a chronic autoimmune disorder of unknown etiology. The disease affects middle-aged women and is characterized by the destruction of the intralobular bile ducts that causes consequent cholestasis. AMA is a hallmark of PBC, composed mostly of IgG and IgM class. The M2 antibody is the most specific one, with sensitivity range of 54-98% depending on type of test used. PBC is often accompanied by other autoimmune diseases, such as Sjögrens syndrome, thyroiditis, rheumatoid arthritis, dermatomyositis, polymyositis. Interstitial lung disease (ILD) has been reported in patients with primary biliary cirrhosis but its frequency and nature are poorly understood. We report pulmonary involvement in the course of PBC in 4 middle-aged women. Histopatological examination of lung specimens was available in three patients: two presented with sarcoid - like granulomas, one with lymphocytic interstitial pneumonia (LIP). In one patient the diagnosis of pulmonary fibrosis was based on clinical and radiological features. Because of abnormal pulmonary function tests (PFT) results all the patients were treated with prednisone, one, additionally with azathioprine. The treatment was successful in all of the patients.

Shrinking lung syndrome masked by pleuropericarditis: a case report and review of the literature

The purpose of this article is to present an unusual case of shrinking lung syndrome (SLS) masked by pleuropericarditis with a review of the literature. We report a case of SLS in a 44-year-old woman in which the diagnosis was initially confounded by concurrent pleuropericarditis. The English medical literature was comprehensively reviewed for SLS for its presentation, clinical findings, diagnosis, treatment, with specific focus on its pathogenesis. SLS is a rare respiratory complication associated with systemic lupus erythematosus (SLE). The main manifestation of the disease is unexplained dyspnea, chest pain, and orthopnea. Lung volume reduction without parenchymal abnormalities along with restrictive ventilatory defect on pulmonary function test (PFT) is the hallmarks of this condition. Pathogenesis, treatment, and prognosis of SLS are not well described due to the small number of reported cases. The diagnosis of SLS in our patient was made based on imaging, PFT, and the exclusion of other respiratory diseases associated with SLE. Treatment with corticosteroid and intravenous cyclophosphamide was initiated due to simultaneously diagnosed renal involvement. Our case demonstrates the salient features of SLS. It emphasizes that although SLS is a rare disease limited to small subset of patients with SLE, it should be considered in patients with SLE with unexplained dyspnea. Moreover, symptoms of pleuropericarditis can mask and delay the diagnosis of SLS. Prompt diagnosis and treatment can lead to a decrease in morbidity and stabilization of pulmonary function test abnormalities.

Assessment of the Level of Asthma Control among Adult Patients in Two Tertiary Care Centers in Nigeria

Background. According to the Global Initiative for Asthma (GINA) guidelines, the goal of asthma management is to achieve clinical control. Uncontrolled asthma places a significant social and economic burden on patients. Objective. The two aims of this study were to (1) assess the level of asthma control (according to the GINA definition of “control”) among adult patients attending two tertiary care centers in Nigeria and (2) to determine the predictors associated with uncontrolled asthma. Methods. This cross-sectional study was carried out from June 2009 to December 2010. The participants were all 18 years old or older with physician-diagnosed asthma. First, the participants completed an interviewer-administered questionnaire, which included items that collected their socio-demographic information and clinical data. Second, anthropometric indices were measured and spirometry was conducted to determine each participant’s lung function. Finally, the researcher team members assessed each participant’s inhaler device technique. The outcome measures were (1) uncontrolled asthma, (2) partly controlled asthma, and (3) controlled asthma. Results. One hundred and twenty-four asthma patients participated in the study. Of these, 69.3% had uncontrolled asthma, 22.6% had partly controlled asthma, and 8.1% had controlled asthma. Multivariate analysis showed that uncontrolled asthma was strongly associated with asthma severity based on clinical features, the incorrect use of an inhaler device, the use of oral corticosteroids, an abnormal pulmonary function test, the presence of comorbidity, and the lack of adherence to inhaled corticosteroids (ICSs). The results also revealed that increasing age and a lack of tertiary education were weakly associated with asthma control. In this study, gender, marriage, smoking status, occupation, socioeconomic status, income, and the duration of the asthma were not associated with asthma control. Conclusion. A significant proportion of the sampled patients had uncontrolled asthma. To reduce this number, health care providers must reinforce the education of each asthma patient and promote the regular assessment of asthma control at every clinic visit, identify those with poor control, and institute the appropriate therapy needed to achieve clinical control.

Lung Function Tests in Sickle-Cell Patients in Benin City

Background. Sickle-cell disease (SCD) is a life-long haematological disorder characterized by red blood cells that assume an abnormal, rigid, sickle shape and with high prevalence in West Africa. Sickle cell disease (SCD) is one of the most common genetic disorders among Africans as indicated in Akinyanju (1989). 61.4% of the children born annually with haemoglobinopathies worldwide have been reported to have SCD and they are from Africa as shown in World Health Organization Report (1987). Hypoxaemia is the hallmark of pulmonary abnormality in SCD patients of all age groups. This was first documented by Klinefelter (1942), and has since been corroborated by other workers. The hypoxaemia of stable SCD patients is said to result from the combined effects of perfusion and diffusion defect. Materials and Methods. This study was carried out to determine the changes in lung functions in relation to gender, age, and body mass index (BMI) of sickle-cell patients in a stable state. 60 subjects made up of 30 patients and 30 control groups were used in this study. Both test and control groups were 13 men and 17 women, aged 19–35 years. Lung Function Test was done with the aid of a digital spirometer (Spirolab II, Italy). Weight was measured using Avery scales (Avery Berkel, 2003, UK). Height was measured using a wall-mounted stadiometer. Data were collected and analyzed using Student's t-test and Pearson correlation. was statistically significant. Results. The results revealed that adults with SCD had a significantly lower mean forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) compared to the control. The lung function indices were lower in females than males in the sickle cell patients. The FVC, FEV1, and FEV1/FVC correlated positively with BMI statistically in the patients. The lung function declined with age. This work has also shown that the most common pulmonary function test (PFT) abnormality was restrictive disease pattern (76.7%). Conclusion. The result of this work suggested that lung function differs significantly in subjects with SCD compared with matched controls of a similar age and gender.

Lethal Pulmonary Complications After Pediatric Allogeneic Hematopoietic Stem Cell Transplantation

Hematopoietic stem cell transplantation (HSCT) in children is accompanied by a transplant-related mortality of 10% to 30%, which is the result of lethal pulmonary complications (LPCs) in many cases. We retrospectively assessed prevalence and risk factors of LPC following 234 allogeneic HSCTs in 228 patients for malignant or nonmalignant diseases at a single institution. Pulmonary complications (PCs) were observed following 81 of 234 transplants (35%). LPCs were observed in 4% of HSCT within 100 days and in 14% within 5 years after HSCT. Late PCs after day 100 were lethal in 56% (22/39) of the patients with PCs, who are 11% (22/202) of all evaluable patients still alive after day +100. Causes of LPC after day 100 were viral (10 cases), bacterial (1 case), fungal (5 cases) pulmonary infection, or noninfectious (6 cases) PCs. Abnormal pretransplant pulmonary function test was not associated with an increased risk of LPC. Children older than 10 years and those undergoing a second HSCT had an increased incidence of overall LPC. T-cell depletion and mismatched donor HSCT (P = 0.001), but not age, were associated with an increased risk of lethal viral pneumonia. Transplant-related mortality up to 5 years after HSCT in children was associated with LPC in 14%. There were more late (>100 days) than early LPCs, predominantly due to infectious etiologies and affecting children >10 years of age.

Extent of Subclinical Pulmonary Involvement in Childhood Onset SystemicLupus Erythematosus in the Sultanate of Oman

The aim of this study was to investigate the frequency of pulmonary function abnormalities in clinically asymptomatic children with Systemic Lupus Erythematosus and to determine the relationship of these abnormalities to clinical, laboratory, and immunological parameters as well as to disease activity. Forty-two children with childhood onset Systemic Lupus Erythematosus were included in this study. Demographic, clinical, laboratory and immunological parameters, as well as disease activity were assessed. Pulmonary function tests (PFT) were performed routinely to screen for subclinical lung disease. Out of the 42 children, 19% (n=8) had clinical evidence of pulmonary involvement. The patients with no clinical evidence of pulmonary involvement (n=34) represent the study cohort. From our cohort of patients with no clinical evidence of pulmonary involvement 79% (n=27) had PFT abnormality; including 62% (n=21) had reduced FVC, 71% (n=24) had reduced FEV1, and 67% (n=12) had reduced DLCO. Similarly, 56% (n=15) had a restrictive PFT pattern, and 2.6% (n=2) had an obstructive PFT pattern, while 33% (n=7) had an isolated impairment of diffusion capacity. Due to small sample size; it was not possible to find a statistically significant difference between the cohort of asymptomatic SLE patients with abnormal PFT findings (n=27) and those with normal PFT findings (n=7) in terms of clinical, laboratory, immunological or disease activity index score. Subclinical lung disease, as demonstrated by abnormal PFT in patients with normal radiographs, may be common but should be interpreted with caution as an early sign of lung disease. Although PFT studies do not correlate well with pulmonary symptoms in patients with childhood onset SLE, they nevertheless provide objective quantification of the type and severity of the functional lesions.

The Sensitivity of Lung Disease Surrogates in Detecting Chest CT Abnormalities in Children With Cystic Fibrosis

Chest CT scans detect structural abnormalities in children with cystic fibrosis (CF), even when pulmonary function tests (PFTs) are normal. The use of chest CT is limited in clinical practice, because of concerns over expense, increased resource utilization, and radiation exposure. Quantitative chest radiography scores are useful in detecting mild lung disease, but whether they are sensitive to the presence of CT scan abnormalities has not been evaluated. To determine in a cross-sectional study if quantitative chest radiography is a more sensitive marker of chest CT abnormalities than other lung disease surrogates. Brody chest CT scores were calculated for 81 children enrolled in the Wisconsin CF Neonatal Screening Project. We determined the sensitivity for Wisconsin (WCXR) and Brasfield (BCXR) chest radiography scores, PFTs, positive cultures for P. aeruginosa (PA), and parental report of symptoms to detect a Brody score worse than the median score for study participants. The mean FEV(1) for the study population was 91% predicted. Abnormal WCXR and BCXR scores had the highest sensitivity to detect a chest CT score worse than the median; abnormal PFTs, parental report of symptoms, and the presence of PA had much lower sensitivity (P < 0.001). In this cross sectional study, quantitative chest radiography has excellent sensitivity to detect an abnormal chest CT and may have a role in monitoring lung disease progression in children with CF.

Respiratory Involvement in Children with Inflammatory Bowel Disease.

Inflammatory bowel diseases (IBDs), including Crohn's disease and ulcerative colitis, are systemic diseases with a variety of extra-intestinal manifestations. Respiratory involvement, whether clinically symptomatic or latent, may be more common than previously thought. As opposed to adults, most of the cases in children involve Crohn's disease. The pathogeneses of the pulmonary manifestations are obscure. The inflammatory process is not restricted to the bowel as has been suggested by findings of high levels of fractional exhaled nitric oxide (FeNO) in the airways. Increased FeNO has been reported even during clinical remission, while increasing further during exacerbations. Pulmonary manifestations develop usually after the onset of the bowel disease; however, they may emerge after colectomy and cessation of therapy. Pulmonary lesions in children usually involve the lung parenchyma with granulomatous infiltrates. Other less common injuries involve the airways and the pleura. The most prevalent pathologies in adults are bronchiectasis, cryptogenic organizing pneumonia, and small airway disease. Pulmonary involvement in IBD is often latent or sub-clinical and may be detected solely by laboratory or imaging techniques. Abnormalities in pulmonary function tests (PFTs) are not consistent except for low-diffusion capacity for carbon monoxide, which was found in both children and adults. High-resolution computed tomography (CT) is a sensitive tool for detecting lung involvement and may reveal abnormalities even when PFTs are normal and the patient is asymptomatic. Medications used for IBD, especially nonsteroidal anti-inflammatory drugs, are also a risk factor for lung injury. Treatment of pulmonary involvement often includes systemic corticosteroids with subsequent prolonged treatment of inhaled steroids in appropriate selected cases. Other immune-modulators have been tried. Pediatricians, especially those treating children with IBD, pediatric gastroenterologists, and pediatric pulmonologists should have a high index of suspicion and be aware of tools to be used in the assessment and treatment of pulmonary complications in IBD.

Reduction of Maximal Oxygen Uptake in Sarcoidosis: Relationship with Disease Severity

Background: Patients with sarcoidosis frequently exhibit exertional dyspnea. Although the functional limitation might be related to ventilatory problems, some patients exhibited exertional dyspnea without significant abnormalities of pulmonary function tests (PFT). Therefore, the mechanisms responsible for exercise intolerance remain unclear. Objectives: The aim of this retrospective study was to determine the mechanisms responsible for exercise intolerance. Methods: Cardiopulmonary exercise testing (CPET) was performed in 157 dyspneic sarcoid patients (stage I: 29; stage II–III: 95; stage IV: 33) and VO₂ peak was correlated with radiological stage and resting PFT. Results: VO₂ peak was decreased in 73% patients, did not differ according to radiological stage and was correlated with VC, FVC, FEV₁, TLC and DLCO. FVC was the major significant predictor of VO₂ peak explaining 17% of VO₂ variation. Among CPET variables, peak heart rate and VE/VO₂ at ventilatory threshold explained 22% of VO₂ alteration in stage I and stage II–III, and 9% in stage IV. In stage IV, V_D/V_T peak explained 41% of VO₂ alteration. Conclusion: In conclusion, in the lower stages circulatory impairment and impaired heart rate response to exercise are involved in the exercise capacity limitation, whereas in stage IV the ventilatory and gas exchange impairment may be more important. CPET must be performed to accurately characterize the mechanisms responsible for exercise limitation and help the clinician in the management of the disease.

Pleuropulmonary manifestations in juvenile onset systemic lupus erythematosus: Assessment by pulmonary function tests and multidetector computed tomography

IntroductionPulmonary involvement is a common finding in adults with systemic lupus erythematosus (SLE) also it is one of the most important systems that can be affected in Juvenile onset SLE (JOSLE). Early detection and evaluation of the extent and severity of pulmonary involvement are quite critical for disease prognosis and patients management. Aim of the workTo determine the frequency and type of pleuropulmonary involvement in JOSLE using pulmonary function tests (PFTs) and multidetector CT (MDCT). Patients and methodsTwenty five patients with JOSLE were evaluated for the detection of pleuropulmonary affection in them. The evaluation included clinical, functional & radiological examination using MDCT as a recent & accurate modality for chest imaging. Based on clinical evaluation, patients were divided into two groups; group A (No=16) and group B (No=9), consisting of those asymptomatic and symptomatic as regard pleuropulmonary symptoms, respectively. ResultsThis study revealed that PFT abnormalities were detected in 60% of all studied JOSLE patients while MDCT abnormalities were detected in 52% of them. 37.5% of the asymptomatic patients had abnormal PFTs & 31.25% of them had abnormal findings on MDCT. There was statistically significant difference between patients groups regarding SLEDAI, percentages of abnormal PFTs, abnormalities in plain X-ray and MDCT. With the exception of forced expiratory flow at 25–75% of forced vital capacity (FEF25–75%), the study revealed statistically significant lower values of mean±SD of all measured PFTs in group B compared to group A. The most frequent MDCT findings in all studied patients were pleural effusion and pleural thickening in 16% of all findings, also ground glass opacities found in 16% of all abnormalities suggesting early interstitial lung disease. ConclusionClinical assessment and PFTs revealed a significant percentage of pleuropulmonary involvement in JOSLE patients. MDCT can be helpful in diagnosing the pulmonary involvement in asymptomatic JOSLE patients with normal chest X-ray and uncertain PFT.

Pulmonary manifestations in inflammatory bowel disease: a prospective study

Although pulmonary abnormalities have been recognized in patients with inflammatory bowel diseases (IBD), their prevalence and clinical significance are not known. To study the prevalence and clinical significance of pulmonary abnormalities in patients with IBD. Ninety-five non-consecutive patients with IBD (12 Crohn's disease, 83 ulcerative colitis; mean age 41.9 [SD 13] years; 47 women) were prospectively studied from January 2007 to March 2010. Pulmonary function tests (PFT) and high-resolution CT (HRCT) chest were performed in them. PFT were compared to those in 270 healthy (control) subjects matched for age, sex and smoking status. Twenty-seven (28.5%) patients and 11 (4%) control subjects had abnormal PFT (p < 0.0001). Small airway obstruction was seen in 18 patients, restrictive defect in six and mixed defect in three. Twenty-one (22%) patients had abnormal HRCT findings - bronchiectasis and nodules (nine patients each, including one with nodules who later developed active tuberculosis after infliximab therapy), parenchymal bands (8), mediastinal lymphadenopathy (five, including two with tuberculosis on histology and culture), emphysema (5), brochiolitis (2), pleural effusion or thickening (2), pericardial effusion (2), patchy consolidation (1), ground-glass opacities (1) and lung metastasis (1). Three patients had symptoms (one asthma, two cough). PFT and HRCT chest showed abnormality in about one-quarter of patients with IBD. A majority of patients with these abnormalities were asymptomatic.

Lung Pathologic Findings in a Local Residential and Working Community Exposed to World Trade Center Dust, Gas, and Fumes

To describe pathologic findings in symptomatic World Trade Center-exposed local workers, residents, and cleanup workers enrolled in a treatment program. Twelve patients underwent surgical lung biopsy for suspected interstitial lung disease (group 1, n = 6) or abnormal pulmonary function tests (group 2, n = 6). High-resolution computed axial tomography and pathologic findings were coded. Scanning electron microscopy with energy-dispersive x-ray spectroscopy was performed. High-resolution computed axial tomography showed reticular findings (group 1) or normal or airway-related findings (group 2). Pulmonary function tests were predominantly restrictive. Interstitial fibrosis, emphysematous change, and small airway abnormalities were seen. All cases had opaque and birefringent particles within macrophages, and examined particles contained silica, aluminum silicates, titanium dioxide, talc, and metals. In symptomatic World Trade Center-exposed individuals, pathologic findings suggest a common exposure resulting in alveolar loss and a diverse response to injury.

Pulmonary function after whole lung irradiation in pediatric patients with solid malignancies

Although whole lung irradiation is used to treat pulmonary metastases of pediatric solid malignancies, few studies have addressed its long-term pulmonary consequences. The authors conducted a retrospective study of longitudinal changes in 171 pulmonary function tests (PFTs) and their relation with clinical features in 48 survivors of pediatric malignant solid tumors treated with whole lung irradiation. Although active respiratory symptoms were seen in only 9 patients (18.8%), abnormalities in forced vital capacity (FVC; 58.3%), forced expiratory volume in 1 second (FEV(1) ; 64.6%), total lung capacity (TLC; 72.9%), and diffusion capacity of the lung for carbon monoxide corrected for hemoglobin (DLCO(corr) ; 70.8%) were common. At a median follow-up of 9.7 years after whole lung irradiation, FVC, FEV(1) , and TLC significantly declined longitudinally (P = .04, .03, and .02, respectively). Focal pulmonary boost irradiation was significantly associated with abnormal FEV(1) /FVC (P = .03), forced expiratory flow between 25% and 75% forced vital capacity (P = .005), residual volume (RV; P = .005), and RV/TLC (P = .002). Ten patients had baseline PFTs, and FVC, FEV(1) , TLC, and DLCO(corr) worsened immediately after radiation, followed by transient improvement but subsequent decline. Thirteen of 32 (40.6%) patients aged >18 years were smokers. Pulmonary dysfunction was prevalent after whole lung irradiation and worsened over time, although most patients were asymptomatic. Boost irradiation impaired pulmonary function, and a significant proportion of patients were smokers. Further studies are planned to assess the predictors and clinical consequences of progressive PFT abnormalities and to evaluate educational interventions.

Pulmonary Complications in Survivors of Childhood Hematological Malignancies: Single-Center Experience

Children treated for cancer face the risk of complications later in life, including pulmonary dysfunction. The objective of this study was to evaluate frequency and severity of pulmonary complications in survivors of childhood leukemia and lymphoma treated with chemotherapy alone or combined with radiotherapy. Seventy cancer survivors of hematological malignancies were evaluated for pulmonary complications through history taking, chest examination, high-resolution computed tomography (HRCT) chest, and pulmonary function testing (PFTs). Although most survivors were not clinically compromised, the spectrum of impaired PFTs included obstructive pattern (14.3%), restrictive pattern (5.7%), and mixed pattern (20%). Significant pulmonary dysfunction was seen in children older than 10 years of age (P = .003), and in patients treated with combined chemotherapy and radiotherapy (72.7%) compared with those treated with chemotherapy alone (25%) (P = .001). Cumulative dose of bleomycin was significantly associated with abnormal PFTs (P = .04). Multivariate analysis revealed methotrexate therapy as significant predictor of abnormal PFTs (P = .002). Male patients who received combined therapy showed higher frequency of restrictive, obstructive lung disease, abnormal respiratory reactance, and peripheral airway disease (P = .007, P = .04, P = .002, P = .003, P = .05, respectively). Those with abnormal CT findings (n = 14) had lower forced vital capacity (FVC%), forced expiratory volume in 1 second (FEV1%), and peak expiratory flow (PEF%) when compared to cases with normal CT (P = .001, P <0.001, P = .001, respectively). Subclinical pulmonary function abnormalities are found in survivors of childhood hematological malignancies previously treated and off therapy. Pulmonary dysfunction is more evident with combined chemotherapy and radiotherapy, bleomycin, and methotrexate are the most incriminated chemotherapeutic agents, and males are at higher risk than females; therefore a specific and extended follow-up is warranted.

Alterations in the pulmonary function tests of inflammatory bowel diseases

We aimed to determine the changes in the pulmonary function tests of the patients with inflammatory bowel diseases. Forty inflammatory bowel dieases patients; 30 ulcerative colitis and 10 Crohn's disease, and ageand sex-matched control group, consisting of 30 healthy persons, were included in the study. Disease activity in patients with ulcerative colitis was assessed by Truelove and Witts Criteria and in Crohn's disease patients by Chron's Disease Activity Index. Pulmonary function tests were found abnormal at least in one parameter in 17/30 ulcerative colitis patients (56%) and in 5/10 Crohn's disease patients (50%) in the activation period and in 5/30 ulcerative colitis patients (17%) and in 2/10 Crohn's disease patients (20%) in the remission period of the diseases of the same patients. Forced vital capacity, first second, residual volume/total lung capacity, diffusing capacity of the lung for carbon monoxide and diffusing capacity of the lung for carbon monoxide per liter alveolar volume values were found significantly impaired in the activation period in comparison with the values of the same patients in the remission period (p<0.01). It was found that pulmonary function test values in patients with inflammatory bowel dieases were not affected by either the type of disease or treatment with 5-aminosalicylic acid. However, they were affected notably by the disease activity. Pulmonary function test abnormalities were found frequently in patients with inflammatory bowel dieases without presence of any respiratory symptoms and lung radiograph findings. The severity and frequency of these pulmonary function test abnormalities which were detected even in the remission periods increase with the activation of the disease. Therefore, pulmonary function test may be used as a non-invasive diagnostic procedure in determining the activation of inflammatory bowel dieases and might aid to the early diagnosis of the latent respiratory.

Clinical features of pulmonary involvement in patients with microscopic polyangiitis

To explore the clinical features of pulmonary involvement in patients with microscopic polyangiitis (MPA). We retrospectively investigated the clinical data of 50 patients hospitalized with MPA in Peking Union Medical College Hospital from January 2008 to December 2009, the data included clinical manifestation, laboratory parameters, echocardiography, pulmonary function test, chest computed tomography, and histopathology of kidney. Pulmonary involvements were observed in 46 patients, common symptoms include cough (34/46), expectoration (30/46), dyspnea (19/46) and hemoptysis (16/46). Pulmonary involvement was the initial manifestation in 14 patients, five cases had radiographic evidences of usual interstitial pneumonia before MPA was diagnosed. The prevalence of positive MPO-ANCA antibodies in MPA patients was 96%. The prevalence of positive PR3-ANCA antibodies was 6%. Radiographic manifestations included ground glass attenuation (16/37), interstitial changes (16/37), infiltrates (12/37) and pleural effusion (7/37). The most frequent abnormality in pulmonary function test was reduced carbon monoxide diffusing capacity (12/15) and restrictive ventilation dysfunction (4/15). The incidences of pulmonary hypertension was 33% (13/39), the average pulmonary artery systolic pressure was (48 ± 8) mm Hg (1 mm Hg = 0.133 kPa). The prevalence of pulmonary involvement in patients with MPA was high, pulmonary involvement was the initial manifestation in 28% patients. The clinical manifestations were nonspecific, radiographic manifestations included ground glass attenuation, interstitial changes, infiltrates and pleural effusion. The short term prognosis was well in patients with pulmonary involvement treated with systemic corticosteroids and cyclophosphamide, infection was a leading cause of death in patients with pulmonary involvement.

An implication of the short physical performance battery (SPPB) as a predictor of abnormal pulmonary function in aging people

If association between the decline in physical performance and the decline in pulmonary function is confirmed, the SPPB could be used as a predictor for pulmonary functional declines in aging people because of its convenient use. This study aimed to elucidate the association of the SPPB with the pulmonary function test (PFT) to determine the usefulness of the SPPB as a predictor of PFT decline. The SPPB and PFT were performed on random sample nested in the Korean Longitudinal Study of Aging (KLoSA) panel, a national representative sample of aging people in Korea. Comparisons of adjusted means of forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), forced expiratory ratio (FER) defined as FEV1/FVC between normal and abnormal SPPB groups were performed using the t-test. The association between PFT and SPPB abnormality was examined using multiple logistic regression analysis. Additionally, the associations of gait speed and chair stand time with FEV1 and FVC were examined using multiple linear regression analysis. Five hundred and eighteen subjects were included in analysis. Approximately 43% (222/518) of the subjects were male and 65% (338/518) were 60 years or older. Adjusted means of FEV1 and FER were significantly or marginally lower when SPPB score was abnormal in both overall and non-smoking men (p=0.009 and 0.053 for overall, p<0.001 and p<0.080 for non-smokers), but FVC was lower only in non-smoking men (p=0.024). Abnormal SPPB score was significantly associated with abnormal PFT regardless of sex. (adjusted odds ratio=OR=3.76, 95%CI=1.96–7.22 for men, adjusted OR=2.11, 95%CI 1.28–3.47 for women). Gait speed was significantly or marginally associated with FEV1 and FVC in participants 60 years or older, regardless of sex. We conclude that abnormal SPPB score was associated with abnormal pulmonary function. Thus, the SPPB has the potential to be used as an early predictor of abnormal pulmonary function in clinical settings and epidemiological study.

Respiratory symptoms in rheumatoid arthritis: relation to pulmonary abnormalities detected by high-resolution CT and pulmonary functional testing

Pulmonary disease is the most frequent and among the most severe extra-articular manifestation of rheumatoid arthritis (RA). However, this issue has not been sufficiently studied in Egyptian patients. The objectives of the present study are to investigate the prevalence and types of pulmonary involvement using high-resolution computed tomography scan (HRCT) and pulmonary function tests (PFT) and evaluate the association between respiratory symptoms and RA-lung disease in a group of Egyptian RA patients. Thirty-six RA patients were recruited; 34 females (94.4%) and 2 males (5.6%) with median age of 48.5 years, and none of them was smoker. Detailed medical and drug histories were obtained. PFT, plain X-ray of the chest, and HRCT were performed to all subjects involved. Nearly 64% of RA patients demonstrated abnormalities in PFT and 47% in HRCT. Mixed restrictive and obstructive pattern was the commonest. Nearly two-thirds of our patients reported one or more pulmonary symptom whether dyspnea, cough, wheezing, or phlegm. Dyspnea was the most frequent symptom. Respiratory symptoms were statistically more common in patients with lung disease. The advanced age, high radiological score, and severity of rheumatoid disease were found to be predictive of lung involvement. Among respiratory symptoms, dyspnea and cough were associated with any pulmonary abnormalities. When specific pulmonary abnormalities were considered, only dyspnea was identified as predictor for restriction. For obstructive abnormality, both cough and wheezing provided valid prediction. We conclude that pulmonary involvement is a common manifestation in Egyptian RA patients, and the pattern of involvement is generally consistent with other studies that were performed worldwide. Specific respiratory symptoms could be used as practical, easy, and cost-effective method, especially in older and with more severe RA patients, to discriminate patients in need of subsequent PFT and HRCT imaging.

Pulmonary Function Test in Transfusion-Dependent β-Thalassemia Major Patients: A Pilot Study

Lung involvement is one of known complications of thalassemia. The aim of this study was to determine predominant type of pulmonary dysfunction and its relationship to iron overload in β-thalassemia children. Fifty thalassemia major children with treatment of regular blood transfusion and desferrioxamine participated in the study. Thirty-three boys and 17 girls (median age 12.5 years) with β-thalassemia enrolled in the study. Other information including body mass index, hematocrit, and the number of years of blood transfusion were recorded. Serum ferritin level and hematocrit were 3346 ± 1667 mg/dL and 27.7 ± 2, respectively. Pulmonary function tests were performed in all subjects for detecting pulmonary dysfunction. Thirty-five patients (70%) with thalassemia had abnormal result of spirometry. Obstructive airway disease based on reduced forced expiratory volume in 1 second (FEV1) and FEV1/forced vital capacity (FVC) ratio <80% was detected in 4 patients (8%). Six patients (12%) showed restrictive pattern, as defined by a reduction FVC <80% and FEV1/FVC ratio ≥80%. In this study, small airway involvement based on presence of forced expiratory flow (FEF25%–75%) <60%, FEV1/FVC ratio >70%, and FVC >80% was detected in 25 subjects (50%). Decreased values of peak expiratory flow rate (PEF) were detected in 23 (46%) and low FEV1 in 10 (20%) subjects. There was no significant correlation between abnormal pulmonary function test and serum ferritin level in children with thalassemia. This study showed small airway disease was predominant abnormality in thalassemia patients, although additional larger studies are needed to evaluate underlying mechanisms and validate these findings.

Outcome of Hodgkin's Lymphoma Patients After Reduced-Intensity Allogeneic Stem Cell Transplantation: Complete Remission Status Has the Most Relevant Clinical Impact along with Extranodal Disease and C-Reactive Protein Before Transplant

AbstractAbstract 3542Patients affected by Hodgkin's lymphoma (HL) who are chemorecfractory or relapsed after an autologous transplantation (autoSCT) may be eligible to a reduced intensity allogeneic stem cell transplantation (RIC alloSCT). Patients in progressive (PD) or stable disease (SD) at transplant seem to do worse than those in complete (CR) or partial remission (PR). We reviewed 43 cases of HL patients treated with a RIC alloSCT in our Institute from 2001 to 2010, focusing on the outcome based on disease status, disease characteristics, and treatment used to induce remission before transplant. Thirty patients (70%) were affected by nodular sclerosis HL. Median age at transplant was 31 (range, 53-19), 22 patients (51%) were female. The median number of previous chemotherapy was 4 (range, 2–10). Seven patients (16%) underwent autoSCT followed by alloSCT and 3/7 of them (43%) were in CR before allografting. All but one of the other 36 patients (81%) relapsed after an autoSCT and before allografting, were treated as follows: 19 received hyperfractioned polichemotherapy (D-PACE n=16, HypercHidam n=2, EPOCH n=1), 8 patients received conventional chemotherapy (gemcytabin based-chemotherapy n=4, BEACOPP n=1, bendamustine n=2, mecloretamin n=1) and 12/27 (44%) of these patients achieved CR, 8 patients received non-chemotherapy drugs alone or combined with chemotherapy in the setting of phase I-II trials and 3/8 (37%) were in CR at transplant, only one patient was in untested relapse. Thirty two (74%) patients had previously received radiotherapy. Disease status at transplant was: CR n=18 (42%), PR n=16 (37%), PD/SD n=9 (21%). Sixteen patients (37%) had extranodal disease. C-reactive protein (PCR) value at admission was above the normal range in 14 patients (33%). In 40 patients HCT-CI was assessed and it was 0 in n= 5 (13%), 1–2 n= 11 (27%), and ≥3 n= 24 (60%), with almost all the patients having abnormal pulmonary function tests. The type of donor was: HLA identical sibling n= 15 (35%), haploidentical sibling, n= 16 (37%), matched unrelated (MUD) n= 12 (28%). The conditioning regimen was based on thiotepa, fludarabine and cyclophosphamide; 2Gy TBI and T-cell depletion with alemtuzumab or ATG were used in case of haploidentical donor. The median follow-up of living patients was 26 months (range, 2–70).All but one patients engrafted. The median PFS and OS were 10 months and 38 months, respectively. The estimated 3-year progression free survival (PFS) and 3-year overall survival (OS) were 37% and 52%, respectively. One year and 2-year transplant related mortality (TRM) was 7.6% and 10.8%, respectively. We performed an univariate analysis based on sex, histology, donor type (sibling versus MUD versus haploidentical), number of previous therapies (<=3 versus >3), extranodal disease, disease status before transplant (CR versus PR versus SD/PD), treatment received at relapse before alloSCT (chemotherapy versus autoSCT versus experimental drugs), previous radiotherapy, HCT-CI (0 versus ≥1), PCR value (normal versus abnormal). OS was influenced by sex (p=0.008, female patients did worse), disease status before alloSCT (p=0.025, both PR and PD/SD patients did worse than CR patients), extranodal disease (p=0.001), and elevated PCR (p=0.002). PFS was adversely correlated to female sex (p= 0.009), PR/PD/SD (p=0.0001), HCT-CI >=1 (p=0.03), extranodal disease (p=0.01), and abnormal PCR (p< 0.001). Haploidentical recipients showed a lower cumulative incidence of relapse (p=0.05), despite a higher NRM (0.02). Of note, the type of therapy received before transplant, either chemotherapy, autoSCT or experimental drugs, did not influence the outcome. In multivariate analysis including disease status, PCR value and extranodal disease, the latter was predictive of OS (p=0.04), disease status (CR versus PR/PD/SD) (p<0.001) and PCR value (p<0.01) were independent prognostic factors for PFS. PFS of patients with chronic graft-versus-host disease was not different compared to those without (p=0.29).In conclusion, RIC alloSCT may be a feasible and efficacious treatment option in chemorefractory and relapsed HL patients, but CR status has been confirmed as one of the strongest predictor of outcome independently of the therapy used to achieve the response. An abnormal PCR value at the time of admission for transplant and a history of extranodal disease are also adverse prognostic factors. Disclosures:No relevant conflicts of interest to declare.