Reduced Diffusion Capacity Research Articles

Severe Pulmonary Hypertension Associated With Emphysema: A New Phenotype?

Mild to moderate precapillary pulmonary hypertension (PH) is a common complication of COPD and has typically been related to severe airflow limitation associated with chronic hypoxemia. Previous studies focusing specifically on patients with emphysema found that worsening PH was associated with progression of airflow obstruction. In the present report, we describe a new phenotype of COPD with severe precapillary PH in patients presenting with progressive dyspnea, normal spirometry, severely reduced diffusion capacity of the lung for carbon monoxide, and high-resolution CT scans of the chest showing diffuse centrilobular emphysema.

Pulmonary Emboli From Therapeutic Sodium Hyaluronate

A patient presented with shortness of breath and pleuritic pain shortly after bilateral knee synovial injections with sodium hyaluronate (HA). He was discharged after a brief hospitalization without a diagnosis when no Doppler or radiologic evidence of deep vein thrombosis or pulmonary emboli was found. Radiologic studies found patchy ground glass opacities that were predominantly peripheral in disposition, with prominent septal lines in the lungs; a subsequent pulmonary function test showed a reduced diffusing capacity of the lung for carbon monoxide (D(LCO)). These results prompted a lung biopsy that revealed multiple emboli composed of HA and fibrin in medium size pulmonary arteries, enlarged lymphatic vessels, and a bone marrow embolus. This is the first report of HA emboli following therapeutic HA injections and demonstrates that pulmonary function tests can be used to infer the reduction in pulmonary vascular area consequent to pulmonary emboli, and so can contribute to the detection of pulmonary emboli in unusual presentations.

Combined pulmonary fibrosis and emphysema associated with microscopic polyangiitis

To the Editors: Microscopic polyangiitis (MPA) is a necrotising multiorgan vasculitis associated with a variety of circulating autoantibodies, such as anti-neutrophil cytoplasm antibodies (ANCAs) against myeloperoxidase (MPO) [1]. Typical and most common pulmonary involvement comprises of alveolar haemorrhage secondary to pulmonary capillaritis as well as interstitial lung fibrosis [2] and progressive obstructive lung disease [1, 3, 4]. The combination of pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome, encompassing a distinct radiology, revealing both upper-lobe emphysema and lower-lobe fibrosis on high-resolution computed tomography (HRCT) of the chest, as well as lung function profile, with apparently preserved lung volumes contrasting with disproportionally impaired gas exchange, as assessed by reduced diffusing capacity of the lung for carbon monoxide [5, 6]. CPFE has been recently described in the context of connective tissue diseases [7]. Nevertheless, it is still debatable whether CPFE represents a distinct syndrome or it is just a phenotype of pulmonary fibrosis with coincidental emphysema. Here, we describe for the first time, in a male patient, a novel type of pulmonary manifestation of MPA, the combination of pulmonary fibrosis and emphysema. In 2008, an 80-yr-old Greek-Caucasian male, heavy ex-smoker (80 pack-yrs), ex-farmer and coal worker with a history of idiopathic pulmonary fibrosis, based …

Obstructive lung function in sarcoidosis may be missed, especially in older white patients

To the Editors: Lung function abnormalities are often present when patients are first diagnosed with pulmonary sarcoidosis, and most studies to date indicate that the predominant pattern is of restriction or normal airways, with a truly obstructive pattern in a minority [1]. The largest case–control study of sarcoidosis to date, the ACCESS (A Case Control Etiologic Study of Sarcoidosis) trial, showed that the biggest group of patients in that particular cohort (46.9%) presented with a forced expiratory volume in 1 s (FEV1)/forced vital capacity (FVC) ratio of >80% and only 13.2% presented with a ratio of 50–69% [2]. Of the 215 patients from the ACCESS trial who were followed up for a further 2 yrs, the majority showed no change in pulmonary function, radiographic stage or dyspnoea score [3]. A more recent European study showed that patients with sarcoidosis had impaired lung compliance and a reduced diffusing capacity of the lung for carbon monoxide ( D L,CO) and, while there was no predominance of restrictive lung defects, airway obstruction was only present in 11.7% of patients [4]. Over the past 3 yrs, we have noticed that an increasing number of patients who present to our clinics in West London, UK with a new diagnosis of pulmonary sarcoidosis have a predominantly obstructive lung defect. A detailed analysis of lung function in sarcoidosis with respect to stratification by patient demographics has, to our knowledge, not been previously reported. We present the results of a systematic retrospective analysis of 164 consecutive patients presenting with a new diagnosis of pulmonary sarcoidosis over a 10-yr period and in whom formal lung function was recorded at the time of presentation. All patients had a clinically definite diagnosis, with supportive histology …

Erratum to: Impaired diffusing capacity for carbon monoxide in children with type 1 diabetes: is this the first sign of long-term complications?

We assessed the presence of lung dysfunction in children with type 1 diabetes, evaluated as reduced diffusing capacity of the lung for carbon monoxide (DLCO), and its components: membrane diffusing capacity (DM) and pulmonary capillary blood volume (Vc). A total of 42 children, aged 15.6 ± 3.8 years, with type 1 diabetes for 8.3 ± 5.5 years, and 30 healthy age and sex-matched peers were recruited for the study. Lung volumes and spirometric dynamic parameters were assessed by plethysmography. Single-breath DLCO was measured according to international recommendation. DM and Vc volume were calculated. Lung volumes were significantly reduced in young patients with type 1 diabetes when compared to controls. Moreover, DLCO was reduced in patients compared to controls (78% ± 16% vs. 120% ± 1%, P = 0.0001). However, when differentiating DM and Vc compartments, we observed a significant impairment only about Vc (34 ± 20 ml vs. 88 ± 18 ml; P = 0.0001), while no difference was observed about DM compartment (23 ± 4 vs. 26 ± 3 ml/min/mmHg, P = 0.798). Whether this might be seen as the “first” sign of microangiopathic involvement in patients with type 1 diabetes has to be confirmed on larger groups but is still fascinating. Meanwhile, we suggest to screen DLCO in all patients with type 1 diabetes.

Silicosis with splenic involvement

A 58-year-old woman presented with progressive breathlessness over several years. She worked in the ceramic industry with crystalline silica exposure for 14 years but had never smoked. The lung function tests revealed a moderate obstructive ventilatory defect and a severe reduced diffusing capacity of carbon monoxide. A chest radiograph revealed calcified antracosilicotic nodules within …

Pulmonary sequelae in a patient recovered from swine flu

The pandemic of swine flu (H1N1) influenza spread to involve the whole world rapidly. Many patients manifested a mild clinical illness but some developed pneumonia and respiratory failure. High mortality was observed in patients with severe disease. Among survivors, studies are limited. Ground-glass opacities on a high-resolution computerized tomography scan and reduced diffusion capacity were noted after 3 months in a study. But long-term complications in patients with swine flu pneumonia have not been studied well. We are presenting an unusual case of swine flu pneumonia who developed interstitial lung disease after recovery.

Impaired diffusing capacity for carbon monoxide in children with type 1 diabetes: is this the first sign of long-term complications?

We assessed the presence of lung dysfunction in children with type 1 diabetes, evaluated as reduced diffusing capacity of the lung for carbon monoxide (DLCO), and its components: membrane diffusing capacity (DM) and pulmonary capillary blood volume (Vc). A total of 42 children, aged 15.6±3.8 years, with type 1 diabetes for 8.3±5.5 years, and 30 healthy age and sex-matched peers were recruited for the study. Lung volumes and spirometric dynamic parameters were assessed by plethysmography. Single-breath DLCO was measured according to international recommendation. DM and Vc volume were calculated. Lung volumes were significantly reduced in young patients with type 1 diabetes when compared to controls. Moreover, DLCO was reduced in patients compared to controls (78%±16% vs. 120%±1%, P=0.0001). However, when differentiating DM and Vc compartments, we observed a significant impairment only about Vc (34±20ml vs. 88±18ml; P=0.0001), while no difference was observed about DM compartment (23±4 vs. 26±3ml/min/mmHg, P=0.798). Whether this might be seen as the "first" sign of microangiopathic involvement in patients with type 1 diabetes has to be confirmed on larger groups but is still fascinating. Meanwhile, we suggest to screen DLCO in all patients with type 1 diabetes.

Lung function and quality of life in survivors of the acute respiratory distress syndrome (ARDS)

Recent studies have begun to describe the long-term outcomes of acute respiratory distress syndrome (ARDS) survivors. These patients experience a number of physical, mental and psychological morbidities that significantly impair their health-related quality of life (HRQL). The trajectory of pulmonary recovery in survivors of ARDS, as it relates to lung function, structure and health-related quality of life (HRQL), is predictable and often persists years after hospital discharge. True pulmonary parenchymal morbidity is uncommon and when present, persistent restrictive disease is likely related to diaphragmatic weakness with a mild reduction in diffusion capacity (DLCO). Future research should focus on identifying patients at risk for long-term functional limitations and the design of rehabilitation interventions tailored to individual patient needs.

Pathologic diagnosis and clinical analysis of chronic extrinsic allergic alveolitis

To study the clinicopathologic features and diagnostic approach of chronic extrinsic allergic alveolitis (EAA). Seven cases of chronic EAA diagnosed by open lung biopsy or lung transplant were enrolled into the study. The clinical and pathologic features were analyzed and the literature was reviewed. There were altogether 4 men and 3 women. The age of the patients ranged from 30 to 65 years (mean = 48 years). All cases represented chronic form and five cases diagnosed by open lung biopsy also showed features of recent aggravation, leading to hospitalization. Four cases had known history of exposure to inciting gases, pollens and pets, and only 2 cases were positive for allergens. High-resolution CT scan showed ground-glass attenuation and reticular pattern that often had a patchy distribution and central predominance. Bronchoalveolar lavage analysis showed marked lymphocytosis, with CD4(+)/CD8(+) ratio less than 1. Lung function test demonstrated a restrictive ventilatory defect, with decreased compliance, reduced diffusion capacity and high airway obstruction. Five cases had open lung biopsy performed and two cases had undergone lung transplantation. Pathologic examination showed bronchiolocentric cellular interstitial pneumonia, interstitial fibrosis, non-caseating epithelioid granulomas, epithelioid histiocytic infiltrate in the respiratory bronchioles and intraluminal budding fibrosis. The five cases with open lung biopsy performed also showed neutrophilic infiltrate in the alveoli. The two lung transplant cases were complicated by severe fibrotic changes. Chronic EAA demonstrates characteristic pathologic features. Definitive diagnosis requires correlation with clinical and radiologic findings due to possible morphologic mimicry of other diffuse parenchymal lung diseases.

Pneumoconiosis and emphysema in construction workers: results of HRCT and lung function findings

ObjectivesTo evaluate the prevalence of HRCT findings in construction workers previously surveyed by chest radiographs classified according to ILO guidelines. To examine the association between HRCT findings and exposure to...

A Case of Pulmonary Fibrosis with Microscopic Polyangiitis

A 65-year-old woman was admitted due to poor oral intake and a dry cough over the previous 3 months. The physical examination was remarkable for bibasilar crackles, and plain chest radiography showed reticulation in both lower lung fields. A pulmonary function test demonstrated a restrictive pattern with a reduced diffusing capacity of the lung for carbon monoxide. High resolution computed tomography showed reticulation and honey-combing in both peripheral lung zones, which was consistent with usual interstitial pneumonia pattern. Her skin showed livedo reticularis. The erythrocyte sedimentation rate and C-reactive protein level were elevated, and hematuria was noted on urinary analysis. A serologic test for auto-antibodies showed seropositivity for Myeloperoxidase-Anti-neutrophil cytoplasmic antibody (MPO-ANCA). A kidney biopsy was performed and showed focal segmental glomerulosclerosis. She was diagnosed as having pulmonary fibrosis with microscopic polyangiitis (MPA) and treated with high dose steroids. Here we report a case of pulmonary fibrosis coexistent with microscopic polyangiitis.

Associations with digital ulcers in a large cohort of systemic sclerosis: Results from the Canadian Scleroderma Research Group registry

Digital ulcers are a common complication of systemic sclerosis (SSc; scleroderma). Approximately half of SSc patients have had a digital ulcer, but information is lacking on whether digital ulcers are associated with patient demographics and clinical outcomes. We wanted to determine the associations between digital ulcers and other SSc vascular complications and organ involvement. Data from the Canadian Scleroderma Research Group are collected annually on SSc patients, including presence, location, and number of digital ulcers; complications from digital ulcers; internal organ involvement; skin score; and laboratory results. Correlation coefficients, chi-square test, and logistic regression modeling were done to determine the associations of digital ulcers with other factors, including internal organ complications. A total of 938 patients were included; 86% were women, the mean age was 55 years, the mean disease duration was 13.6 years, and 50% had limited cutaneous SSc. Eight percent had a digital ulcer currently and 44% had a digital ulcer ever; 53.1% had digital pitting scars. Digital ulcers were associated with increased modified Rodnan skin score (P = 0.0001), hand and finger skin score (P = 0.0001), Health Assessment Questionnaire score (P = 0.0001) and disease duration (P = 0.001), younger age of SSc onset (P = 0.0001), interstitial lung disease (ILD; P = 0.0001), and topoisomerase I (Scl-70) antibodies (P = 0.0001) in limited and diffuse cutaneous SSc subsets. Digital ulcers were not associated with sex (P = 0.95), smoking (P = 0.9), pulmonary arterial hypertension (PAH; P = 0.35), and scleroderma renal crisis (SRC; P = 0.569). Digital ulcers were further associated with reduced diffusing capacity for carbon monoxide (DLCO; P = 0.0001) and esophageal involvement (P = 0.0001) in diffuse cutaneous SSc only. Digital ulcers are associated with worse disease, including skin and lung involvement, but are not associated with PAH and SRC. However, the low DLCO that is associated with SRC could represent ILD or microvasculopathy.

Correlation of pulmonary function variables with hemodynamic measurements in patients with pulmonary arterial hypertension

A reduced diffusing capacity of the lung for carbon monoxide (DLCO) measured during a pulmonary function test can suggest pulmonary arterial hypertension (PAH). The DLCO has been reported to weakly correlate with pulmonary hemodynamics. To determine whether the relationship between the DLCO and pulmonary arterial pressures can be strengthened by normalizing the DLCO to spirometric variables. Patients were seen at a tertiary care referral center. Consecutive subjects who underwent right heart catheterization (RHC) for the evaluation of suspected PAH from 01 January 1991 through 01 October 2006 were identified. Pulmonary function testing (PFT) data performed within 60 days of the RHC was collected. Spearman rank correlation between PFT and RHC variables was calculated. One hundred thirty-eight patients who had an RHC performed had complete PFTs available. No significant correlation was identified between the mean pulmonary artery pressure and the pulmonary vascular resistance against the DLCO, nor the DLCO when normalized to: forced expiratory volume in 1 s, forced vital capacity, total lung capacity or alveolar volume. Spirometric subgroups were identified by standard definitions of restrictive and/or obstructive ventilatory defects. Clinical subgroups were classified based on the clinically diagnosed cause of the patient's PAH. Again, no significant correlation was identified between the PFT variables and RHC measurements in these stratified subgroups. In patients with suspected PAH, invasive hemodynamic measurements of PAH do not correlate with PFT variables, even when corrected for spirometric volumes, and regardless of the subgroup of ventilatory physiology or clinical diagnosis.

Pulmonary hypertension in a patient with Abt-Letterer-Siwe syndrome and episodes of HAPE

To the Editors: Abt–Letterer–Siwe syndrome, which is a disseminated form of Langerhans' cell histiocytosis (LCH), was diagnosed in the patient at the age of 6 months, affecting the inguinal lymph nodes and skin, and recurred at the age of 21 yrs, affecting lungs and arcus pubis 1. After two chemotherapy sessions, including vinblastine, etoposide and corticosteroids, complete remission was reached. At the age of 25 yrs the first high-altitude oedema occurred during a ski vacation at 2,400 m followed by the second episode 3 yrs later at an altitude of 2,800 m, which was confirmed by chest radiograph. After the second episode of high-altitude pulmonary oedema (HAPE) the patient was referred to the Division of Sports Medicine at the University Hospital of Heidelberg, Heidelberg, Germany, where he was assessed as described before 2. Estimated systolic pulmonary artery pressure ( P pa) was normal at rest (25 mmHg), increased to 50 mmHg after 30 min (oxygen saturation 74%) and to 60 mmHg (saturation 65%) after 90 min of exposure to normobaric hypoxia (inspiratory oxygen fraction ( F I,O2) of 12%). Stress Doppler echocadiography revealed systolic P pa values up to 95 mmHg during normoxic exercise of 125 W. The patient had a normal systemic blood pressure at rest and during exercise and no signs of left ventricular dysfunction. Maximum exercise capacity was 1.95 W·kg−1 (70% of normal), maximum oxygen consumption ( V ′O2) was 2.6 L·min−1 (80% of normal), body plethysmography showed patterns of obstructive and restrictive lung disease (airway resistance 158% of normal, forced expiratory volume in 1 s/forced vital capacity (FEV1/FVC) ratio was 60% (70% predicted), vital capacity was 70%, residual volume 166%, total lung capacity 95%) and a reduced diffusion capacity for carbon monoxide (48% pred). Despite shortness of breath, the …

Outcomes Study: 8-Year Experience of Patients With Isolated Reduction in Diffusion Capacity (Dlco)

Outcomes Study: 8-Year Experience of Patients With Isolated Reduction in Diffusion Capacity (Dlco)

Early Right Ventricular Dysfunction in Smokers With COPD and Reduced Diffusion Capacity for Carbon Monoxide (Dlco)

Early Right Ventricular Dysfunction in Smokers With COPD and Reduced Diffusion Capacity for Carbon Monoxide (Dlco)

Convalescent Pulmonary Dysfunction Following Hantavirus Pulmonary Syndrome in Panama and the United States

The objective of this study was to document persistent pulmonary symptoms and pulmonary function abnormalities in adults surviving hantavirus pulmonary syndrome (HPS). Acute infection by most hantaviruses result in mortality rates of 25–35%, while in Panama the mortality rate of 10% is contrasted by an unusually high incidence. In all types of HPS, the viral prodrome, cardiopulmonary phase due to massive pulmonary capillary leak syndrome, and spontaneous diuresis are followed by a convalescent phase with exertional dyspnea for 3–4 weeks, but the frequency of persistent symptoms is not known. In this observational study of a convenience sample, 14 survivors of HPS caused by Choclo virus infection in Panama and 9 survivors of HPS caused by Sin Nombre virus infection in New Mexico completed a questionnaire and pulmonary function tests up to 8 years after infection. In both groups, exertional dyspnea persisted for 1–2 years after acute infection in 43% (Panama) and 77% (New Mexico) of survivors surveyed. Reduction in midexpiratory flows (FEF25–75%), increased residual volume (RV), and reduced diffusion capacity (DLCO/VA) also were common in both populations; but the severity of reduced expiratory flow did not correlate with exertional dyspnea. Symptoms referable to previous hantavirus infection had resolved within 3 years of acute infection in most but not all patients in the Panama group. Temporary exertional dyspnea and reduced expiratory flow are common in early convalescence after HPS but resolves in almost all patients.

Presenting Features of Feather Duvet Lung

Background: Feather duvet lung (FDL) is a rare subgroup of bird fancier’s lung. It is caused by inhalation of organic dust due to goose or duck feathers in duvets or pillows. Methods: A retrospective review of the medical records of 13 patients with FDL was performed to assess the specific history and review clinical characteristics of patients with this disease. Results: All patients were female with a mean age of 53 years (26–71). They were recently exposed to feather duvets (6), pillows (1) or both (6). Specific histories were duvets or pillows filled with raw goose feathers from their own farms (4), intensive contact with goose feathers in youth (3), and bird exposure prior to symptom onset (5). In all patients specific IgG antibodies to goose and/or duck feathers were detected. Pulmonary function tests revealed a moderate to severe reduced diffusion capacity and a mild restrictive pattern. High-resolution computed tomography was performed in 11 patients and demonstrated predominantly ground-glass opacities (10) and fibrosis (6). In bronchoalveolar lavage fluid, lymphocytic alveolitis was demonstrated in all patients. Lung biopsies were obtained in 9 patients and demonstrated lymphocytic alveolitis (8), granulomas (3), bronchiolitis obliterans organizing pneumonia pattern (2), and usual interstitial pneumonia pattern (1). Conclusions: The clinical findings of FDL are typical of extrinsic allergic alveolitis. Primary sensitization could be due to former exposure to bird antigens at home or goose/duck feather exposure in youth. In view of the increasing popularity of feather duvets, FDL should be considered in the differential diagnosis of patients with extrinsic allergic alveolitis.

Exercise tolerance and dyspnea in patients with chronic obstructive pulmonary disease

Peripheral muscle weakness and nutritional disorders, firstly loss of body weight, are common findings in patients with chronic obstructive pulmonary disease (COPD). The aim of this study was to analyse the impact of pulmonary function parameters, nutritional status and state of peripheral skeletal muscles on exercise tolerance and development of dyspnea in COPD patients. Thirty COPD patients in stable state of disease were analyzed. Standard pulmonary function tests, including spirometry, body pletysmography, and measurements of diffusion capacity were performed. The 6-minute walking distance test (6MWD) was done in order to assess exercise tolerance. Level of dyspnea was measured with Borg scale. In all patients midthigh muscle cross-sectional area (MTCSA) was measured by computerized tomography scan. Nutritional status of patients was estimated according to body mass index (BMI). Statisticaly significant correlations were found between parameters of pulmonary function and exercise tolerance. Level of airflow limitation and lung hyperinflation had significant impact on development of dyspnea at rest and especially after exercise. Significant positive correlation was found between MTCSA and exercise tolerance. Patients with more severe airflow limitation, lung hyperinflation and reduced diffusion capacity had significantly lower MTCSA. Exercise tolerance in COPD patients depends on severity of bronchoobstruction, lung hyperinflation and MTCSA. Severity of bronchoobstruction and lung hyperinflation have significant impact on dyspnea level.