The aim of the present study was to describe the prevalence of clinical pulmonary manifestations in primary SS (pSS), and based on registry data, to assess quality of life (QoL) and mortality in ...these patients.
Patients with pSS consecutively included in the Norwegian systemic CTD and vasculitis registry (NOSVAR) were investigated for pulmonary manifestations when presenting with clinical pulmonary symptoms. Pulmonary involvement was defined as typical abnormalities identified with high-resolution CT (HRCT) and/or pulmonary function tests (PFTs).
Among patients referred from our primary area, Oslo (n = 117), lung involvement was found in 26 patients (22%). In our total cohort (n = 216), 59 patients (27%) were affected. A higher rate of pulmonary complications and trends towards longer disease duration and higher age indicated a selection of more complicated cases referred from outside our primary area. Abnormal HRCTs were found in 50 patients (23%) and PFTs in 34 patients (16%). The Medical Outcomes Study 36-Item Short-Form Health Survey Physical Functioning subscore, was significantly reduced in patients with lung involvement (P = 0.03). Furthermore, a 4-fold increased risk of dying after 10 years of disease among patients with lung involvement (n = 10, 17%) compared with those without lung involvement (n = 7, 4.5%) (P = 0.002) was found.
We found a high population-based prevalence of clinical pulmonary involvement (22%) among patients with pSS. Moreover, patients with lung involvement had reduced QoL represented by the subscale Physical Functioning, and mortality was increased.
To compare pulmonary function in patients with juvenile dermatomyositis (JDM) with that of matched controls; and to examine associations between pulmonary function impairment, high-resolution CT ...(HRCT) abnormalities and other disease variables in patients with JDM.
A total of 59 patients with JDM clinically examined a median 16.8 years (range 2-38 years) after disease onset were compared with 59 age-matched and sex-matched controls. Pulmonary function tests included spirometry, diffusing capacity for carbon monoxide (DLCO) and body plethysmography. In patients with JDM, HRCT scans were performed and cumulative organ damage and patient-reported health status assessed.
Patients with JDM had lower total lung capacity (TLC) and DLCO compared to controls (p=0.003 and <0.001, respectively). A low TLC was found in 26% of patients versus 9% of controls (p=0.026), and a low DLCO in 49% of patients versus 9% of controls (p<0.001). HRCT abnormalities were found in 37% of patients, and included interstitial lung disease (ILD) (14%), chest wall calcinosis (14%) and airway disease (15%). Three patients were diagnosed as having ILD prior to the follow-up visit. A low TLC was more often found in patients with than without abnormal HRCT (50% vs 12%, p=0.002). HRCT abnormality correlated with cumulative organ damage (r(s)=0.346, p=0.008) and patient-reported health status at follow-up (p<0.005).
Patients with JDM had smaller lung volumes than controls; a restrictive ventilatory defect was found in 26% and HRCT abnormality in 37% of the patients, and these findings were associated. Although mostly subclinical, the relatively high frequency of pulmonary involvement highlights the systemic nature of JDM.
The aim of this study was to assess the overall prevalence of pulmonary hypertension (PH) in an unselected MCTD cohort and review the current knowledge with a systematic database search.
A nationwide ...multicentre cohort of 147 adult MCTD patients were initially screened for PH by echocardiography, high-resolution computed tomography (HRCT), pulmonary function tests and N-terminal pro-brain natriuretic peptide (NT-proBNP) and then followed up for a mean of 5.6 years. Right-sided heart catheterization was performed when estimated pulmonary artery systolic pressure was >40 mmHg on echocardiography. PH was diagnosed according to the 2009 European Society of Cardiology and European Respiratory Society guidelines.
At inclusion, 2.0% (3/147) had established PH. Two additional PH patients were identified during follow-up, giving a total PH frequency in the cohort of 3.4% (5/147). All five had elevated serum NT-proBNP. Two had isolated pulmonary arterial hypertension (PAH) and three PH associated with interstitial lung disease (PH-ILD). Three PH patients died during follow-up. Nine other patients in the cohort also died, but none of them had echocardiographic signs of PH prior to death.
The data from the current unselected MCTD cohort suggest that the prevalence of PH is much lower than expected from previous studies but confirm the seriousness of the disease complication.
A low-dose CT of the paranasal sinuses was designed with few, thin sections, non-uniform intersection gaps, low milliampere settings and avoidance of direct radiation to the eye lens. The low-dose CT ...was prospectively compared with standard-dose CT in patients with suspicion of acute sinusitis. Forty-seven patients were examined with low-dose CT immediately after standard-dose CT. The effective dose and the lens dose were calculated and compared. Using standard-dose CT as a gold standard the sensitivity and specificity of low-dose CT was calculated for each sinus group. The effective dose and the lens dose of the low-dose CT were reduced to, respectively, 3 and 2% of the standard-dose CT. The diagnostic yield of the low-dose CT with regard to acute sinusitis was good with a high specificity (> or =96%) for all sinus groups. The sensitivity was also high (> or =95%) except for the frontal sinus where the sensitivity was 83%. Low-dose CT offers considerable dose reduction and should be the standard for imaging patients with suspected acute inflammatory paranasal disease.
To investigate serum levels of endostatin in a well characterised cohort of patients with primary Sjögren's syndrome (pSS) and healthy controls (HC) and assess associations between these mediators ...and clinical parameters.
All patients (n=144) were recruited from the Norwegian systemic connective tissue disease and vasculitis registry (NOSVAR) and fulfilled American-European classification criteria for pSS. Pulmonary involvement was based on clinical symptoms and abnormal findings on high-resolution computed tomography of the lungs. The controls were 100 healthy blood donors. Serum levels of endostatin was determined by enzyme immunoassay.
We found higher mean levels of serum endostatin in patients with pSS compared with the controls (p<0.001). The patients with interstitial lung disease (ILD) had higher levels compared with those without pulmonary involvement.
Our results indicate that endostatin is increased in patients with pSS and particularly in those with ILD.
Pulmonary involvement is a common finding in adults with systemic lupus erythematosus (SLE). The aim of this study was to investigate the frequency of pulmonary abnormalities in patients with ...childhood-onset SLE, with particular reference to interstitial lung disease (ILD), and to examine any association between pulmonary abnormalities and other disease-related variables.
A cohort of 60 Norwegian patients with childhood-onset SLE was examined in a cross-sectional study by high-resolution computed chest tomography (HRCT) and pulmonary function tests (PFT). Median disease duration was 11.2 years. Disease activity, cumulative organ damage and immunological markers were also assessed.
Five patients (8%) had abnormal HRCT findings, including micronodules in four patients and bronchiectasis in one. None of the patients had radiographic evidence of ILD. PFT results were impaired in 37% of the patients, the most frequent pulmonary dysfunction was reduced carbon monoxide diffusing capacity (26%). HRCT findings, disease activity or serology did not correlate with PFTs. Reduced diffusion capacity was associated with smoking (p-value < 0.05).
Lung function was moderately impaired, while the frequency of pulmonary parenchymal involvement was low. There was no radiographic evidence of ILD, which is an unexpected finding given the high frequencies reported in adult SLE patients assessed with HRCT. The results suggests that PFT values are often abnormal, but these are infrequently associated with development of ILD or other substantial parenchymal alterations in childhood-onset SLE, and do not require further HRCT investigation in asymptomatic patients.
The objective of this study was to investigate prospectively CT contrast medium enhancement curves of lymphomatous lymph nodes of the neck.
Eleven patients with biopsy-proven lymphoma had their ...enlarged cervical lymph nodes examined with dynamic CT and compared with a control group.
The mean contrast medium enhancement of the lymphomatous nodes was significantly lower than that of the control group in the time interval from 40 s to 180 s after injection.
The enhancement pattern previously suggested from studies of retroperitoneal lymph nodes was confirmed in this prospective study of cervical nodes.
The purpose of the study was to evaluate the ability of CT, including expiratory scans with minimum intensity projection in predicting the development of bronchiolitis obliterans syndrome after lung ...transplantation.
Forty consecutive patients, 29 bilateral and 11 single lung transplanted, were followed-up with regular scans for a median of 36 months. Air trapping was evaluated on expiratory scans constructed from two short spiral scans with minimum intensity projection-technique, one at the level of the carina and the other midway between the right diaphragm and the carina. Air trapping was scored on a 16-point scale. Bronchiolitis obliterans syndrome was diagnosed according to established clinical criteria and quantified spirometrically.
Bronchiolitis obliterans syndrome developed in 17 patients (43%) after a median of 12 months. Air trapping and bronchiectasis was seen before the diagnosis of bronchiolitis obliterans syndrome in only two and one patient, respectively. Interobserver agreement for air trapping score was good (kappa
=
0.65). Air trapping scores performed significantly better than that achieved by chance alone in determining the presence of bronchiolitis obliterans syndrome (
P
=
0.0025). An air trapping score of 4 or more provided the best results with regard to sensitivity and specificity in diagnosing bronchiolitis obliterans syndrome. The sensitivity, specificity, positive and negative predictive values of an air trapping of 4 or more in the diagnosis of bronchiolitis obliterans syndrome were 77, 74, 68 and 81%, respectively.
Expiratory CT scans with minimum intensity projection-reconstruction did not predict the development of bronchiolitis obliterans syndrome in most patients. The findings seriously limit the clinical usefulness of long-term CT monitoring for diagnosing bronchiolitis obliterans syndrome after lung transplantation.
A throat infection with rapid deterioration Zätterstrøm, Ulf K; Hokland, Bjørn M; Walberg, Mette ...
Tidsskrift for den Norske Lægeforening,
2002-Aug-20, 20020820, Letnik:
122, Številka:
19
Journal Article
En halsinfeksjon med rask forverring Zätterstrøm, Ulf K; Hokland, Bjørn M; Walberg, Mette ...
Tidsskrift for den Norske Lægeforening,
2002, Letnik:
122, Številka:
19
Journal Article