Isolated diffusing capacity reduction in systemic sclerosis.

OBJECTIVE. To determine the long-term outcome of patients with systemic sclerosis (SSc) and an isolated reduction in the diffusing capacity for carbon monoxide (DLCO) at the time of initial evaluation. METHODS. Patients with an isolated reduction in DLCO (i.e., normal forced vital capacity [FVC] and normal ratio of the forced expiratory volume in one second [FEV1] to the FVC) on initial evaluation were identified from among 815 patients with SSc who were carefully followed up throughout their illness. We requested that patients have repeat pulmonary function testing (PFT), and the outcomes of these tests, as well as cardiopulmonary and survival outcomes, were determined. RESULTS. An isolated reduction in DLCO, with a normal FVC was detected in 152 (19%) of the 815 patients. A subset of those with an isolated reduction in DLCO (11%) developed isolated pulmonary hypertension and had severely reduced survival rates. Pulmonary hypertension was strongly associated with an initial DLCO of less than 55% of predicted normal and a FVC (% predicted)/DLCO (% predicted) ratio of greater than 1.4. Among all patients in whom this ratio was greater than 1.4, 22% developed isolated pulmonary hypertension, compared with only 2% of those whose ratio was less than 1.4 (P less than 0.01). Of the 152 patients with isolated DLCO reduction, 73 (48%) underwent PFTs a mean of 5.4 years (range 2.0-13.2) after the initial PFT. Only 6 (8%) of these 73 patients ever had serious pulmonary disease: 5 had isolated pulmonary hypertension, and 1 had severe pulmonary fibrosis. Half of the patients with a low initial DLCO demonstrated a significant improvement (greater than 20%) at followup testing that could not be explained by the demographic, clinical, or laboratory findings at the first visit. CONCLUSION. Isolated reduction in DLCO is a frequent abnormality in SSc. Overall, it is associated with a good prognosis for survival and for pulmonary morbidity. A small subset of patients (11%) who have a very low DLCO (less than 55% of predicted) have developed isolated pulmonary hypertension, all of whom had limited scleroderma.     

Therapy for severe interstitial lung disease in systemic sclerosis. a retrospective study

Objective. This retrospective observational study attempted to determine whether any of the therapies used in the management of systemic sclerosis (SSc) patients held potential benefit for patients with interstitial lung disease. Methods. All patients with SSc who had a pulmonary function test (PFT) showing a forced vital capacity (FVC) of <70% predicted and an additional PFT at least 4 months later were grouped according to the treatment they received. Changes in pulmonary function were analyzed by the mean percent predicted FVC from the initial and the final test, and by the rate of percent change in FVC (ml/year) in the first 2 years after therapy. Bronchoalveolar lavage was performed in a subset of these patients. Results. Of 363 SSc patients who had an FVC < 70% predicted, 122 had a second PFT and fulfilled the criteria for one of the following drug groups: high–dose prednisone (n = 21), immunosuppressive other than cyclophosphamide (CYC) (n = 16), CYC (n = 14), D–penicillamine (n = 37), or no drug (n = 34). In both analyses, the CYC–treated group showed significantly more improvement in FVC than did the other groups. Patients with early disease had the greatest likelihood of responding to any drug. Conclusion. This retrospective study shows that patients treated with CYC had a significant improvement in FVC over time. Prospective controlled studies of CYC treatment in early disease are necessary to determine if it can significantly alter the natural history of interstitial lung disease.     

Impact of anti-centromere antibodies on pulmonary function test results in patients with systemic sclerosis without established or suspected pulmonary disease

Pulmonary arterial hypertension (PAH) occurs in approximately 15 % of patients with systemic sclerosis (SSc). Annual screening with pulmonary function tests (PFT) is recommended to help identify those patients at risk of PAH. We have noted that patients with SSc who carry anti-centromere autoantibodies (ACA) often have PFT abnormalities, in the absence of clinical evidence of PAH. To evaluate this further, we undertook a retrospective case-control study evaluating PFT results in patients with SSc in whom pulmonary complications have neither been diagnosed nor suspected. Patients were divided according to ACA carriage and groups compared for PFT results. The median forced vital capacity (FVC) was higher in ACA-positive patients (106 vs. 93 %, p?=?0.004). The gas transfer factor (TLco) was significantly lower in the ACA group (62.5 vs. 71 %, p?=?0.013). The resulting FVC:TLco was significantly higher for ACA-positive vs. ACA-negative patients with SSc (1.70 vs. 1.29, p?<?0.001). Our findings suggest patients carrying ACA, without established or suspected pulmonary complications, have PFT abnormalities consistent with indolent increased pulmonary vascular resistance despite the majority of such patients not subsequently developing PAH. The long-term sequelae of PFT abnormalities in those patients with ACA who do not subsequently develop PAH are unknown.     

Severe restrictive lung disease in systemic sclerosis

Objective. We sought to identify risk factors for developing severe restrictive lung disease and to determine the time of onset and rate of progression in patients with systemic sclerosis (SSc). Methods. Using the University of Pittsburgh Scleroderma Databank, we grouped patients according to their lowest forced vital capacity (FVC) value: <75% predicted, 50—75% predicted, and > 50% predicted. In patients with severe restrictive disease, we examined serial pulmonary function test (PFT) results to determine the rate of loss of lung volume over time. Results. Of 890 SSc patients, 60% (n = 531) never had an FVC ⩽75% predicted; 27% (n = 243) had moderate restrictive disease, with an FVC value of 50—75% predicted; and only 13% (n = 116) of the patients had severe restrictive disease, with FVC ⩽50% predicted. Black race, male sex, early disease, and primary cardiac involvement due to SSc were the features most frequently associated with severe restrictive lung disease (by multiple logistic regression). Fifty–five patients with severe restrictive lung disease had their first of at least 2 PFTs during the first 5 years after onset of any SSc (not pulmonary) symptoms. In 30 patients, the FVC declined by 32% per year in the first 2 years of illness, in 16 patients the annual loss was 12% in years 2—4 after disease onset, and in 9 patients annual loss was 3% during years 4—6 of disease (P < 0.005 by 1–way analysis of variance). Conclusion. In SSc patients, black men with early disease who have cardiac involvement are the most likely to have factors associated with the development of severe restrictive lung disease (which is increasingly becoming a major cause of death). Disease subtype (diffuse versus limited cutaneous) and serum antitopoisomerase I antibody do not differentiate between moderate and severe restrictive disease. Careful monitoring of pulmonary function early in the disease, when the greatest loss of lung function occurs, may help identify patients likely to respond to new therapy.     

Rheumatoid arthritis lung disease. Determinants of radiographic and physiologic abnormalities

Objective. To determine the prevalence and important clinical predictors of radiographic and physiologic abnormalities indicative of rheumatoid arthritis interstitial lung disease (RA-ILD). Methods. An unselected cohort of patients with a confirmed diagnosis of RA and known lung disease were identified (n = 336) and evaluated for RA disease activity and severity. Outcomes included abnormalities determined by the pulmonary function tests of forced vital capacity (FVC) and diffusion capacity for carbon monoxide (DLco), and/or chest radiographic findings of interstitial infiltrates. We used multivariable statistical modeling to determine the independent significance of cigarette smoking and other RA-specific factors on the pulmonary abnormalities of interest. Results. At least 1 of the 3 abnormal findings was identified by pulmonary tests in 32.4% of all patients. These abnormal findings included an FVC <80% of predicted in 42 patients, a DLco <80% of predicted in 64 patients, and evidence of radiographic interstitial infiltrates in 40 patients. After statistical adjustment for confounding factors, pack-years of cigarette smoking remained a significant predictor of low DLco (β = −0.07, 95% confidence interval [95% CI] −0.09, −0.04), low FVC (β = −0.003, 95% CI −0.006, −0.0004), and interstitial abnormalities on chest radiograph (odds ratio for ⩾25 pack-years = 3.76, 95% CI 1.59, 8.88). The Health Assessment Questionnaire (HAQ) Disability Index (DI) was also an important risk factor for the decline in both the DLco (β = −1.15, 95% CI −2.00, −0.30) and FVC (β = −0.23, 95% CI −0.32, −0.13). Conclusion. Although RA disease activity/severity (particularly as defined by the HAQ DI) was important, smoking was the most consistent independent predictor of radiographic and physiologic abnormalities suggestive of ILD in RA.     

Esophageal involvement and pulmonary manifestations in systemic sclerosis

OBJECTIVE: To assess whether esophageal manometric motor disturbances are associated with abnormalities consistent with interstitial lung disease (ILD) on both pulmonary function tests (PFT) and high resolution computerized tomography (HRCT) scans in patients with systemic sclerosis (SSc), during initial evaluation and at 2 years followup. METHODS: Esophageal manometry, PFT, and HRCT scans were performed in 43 consecutive SSc patients. PFT and HRCT scan parameters were compared between patients with severe esophageal motor dysfunction (i.e., aperistalsis and decreased low esophageal sphincter pressure), patients with moderate esophageal motor dysfunction (hypoperistalsis), and patients without esophageal motor dysfunction on manometry. RESULTS: During initial evaluation of SSc, patients with severe esophageal motor impairment, compared with those with moderate and without esophageal dysmotility, exhibited significantly decreased median values of diffusing capacity for carbon monoxide (DLco) (68% vs. 94% vs. 104%) and exhibited higher prevalence of evidence for ILD on HRCT scan (57% vs. 27% vs. 18%). At 2 years followup of SSc, patients with severe esophageal motor disturbances, compared with those without, had faster deterioration of DLco median values (-16% vs. +1%) and higher frequency of ILD on HRCT scan (70% vs. 25%). CONCLUSION: Our series underscores a correlation between the degree of esophageal manometric motor disturbances and evidence for ILD on PFT and HRCT scan in SSc patients, suggesting that gastroesophageal reflux (GER) may be one of the contributing factors of ILD in SSc. Our findings further indicate that patients with severe esophageal impairment may require closer followup of lung parameters. In turn, it suggests that aggressive therapy of GER should be initiated in these SSc patients, as it may result in decreased deterioration of pulmonary function.     

A longitudinal study of pulmonary function in Danish patients with systemic sclerosis

Summary Objective: To determine the types, prevalence and development of respiratory abnormalities in patients with systemic sclerosis (SSc), and to correlate the results with clinical and serological findings. Methods: 176 patients with SSc observed longitudinally were retrospectively included in the study. The change per year of vital capacity (VC), forced expiratory volume in one second/vital capacity (FEV₁/VC), diffusing capacity (DLco) and diffusing constant (Kco) of carbon monoxide from the first till the latest pulmonary function test were correlated to clinical and serological findings, including anti-centromere, anti-Scl-70, and antinucleolar antibodies. Results: An isolated reduction of DLco was seen in 47% and a restrictive ventilatory pattern in 25% of the patients. Restrictive ventilatory pattern correlated to pulmonary fibrosis, dyspnoea, a low prevalence (13%) of anti-centromere antibodies and a high prevalence of anti-Scl-70 antibodies (36%). Progression of DLco reduction was related to long disease duration, presence of anti-centromere antibodies and absence of treatment with penicillamine. Conclusion: Pulmonary involvement is common in patients with SSc. The occurrence of different serological abnormalities in patients with restrictive disease and in patients with progressive isolated reduction of DLco, suggests that the two types of pulmonary damage may have different pathogeneses rather than being different stages in the progression of pulmonary damage.     

Isolated DLco/VA reduction in systemic sclerosis patients: a new patient subset?

Clinical Rheumatology - Diffusing capacity for carbon monoxide (DLco) reduction is the first detectable pulmonary functional test (PFT) change in systemic sclerosis (SSc)–related pulmonary...     

Immunomodulatory treatment in unclassifiable interstitial lung disease: A retrospective study of treatment response

Background and Objective

The optimal management of unclassifiable Interstitial lung disease (ILD) remains a challenge. The aim of this study was to describe pulmonary function trajectories for patients treated with immunomodulatory therapy and for untreated patients.

Methods

Clinical information and treatment data were obtained retrospectively at two ILD centres. Pulmonary function data were analysed using (1) mixed effects linear regression models with and without clinical covariates and (2) propensity score matching using gender, age, physiology (GAP) stage, smoking and presence of ground glass opacities.

Results

Sixty-five percent of the 249 patients included received corticosteroids and/or other immunomodulators. Treated patients had lower forced vital capacity (FVC) (72% vs. 83% predicted) and diffusing capacity for carbon monoxide (DLco) (44% vs. 60% predicted). In mixed effects linear regression, the adjusted change in FVC was −0.22%, [−0.34; −0.11], and −0.15% [−0.28;-0.012] for DLco. The difference in pulmonary function decline between treated and untreated patients was insignificant, −0.082% per month, [−0.28; 0.11], p = 0.10 for FVC and −0.14% per month, [−0.36; 0.079], p = 0.15, for DLco. In propensity score matched analysis, the difference in change in FVC was 0.039% per month, p = 0.12, and for DLco, 0.0085% per month, p = 0.7.

Conclusion

The pulmonary function trajectories for treated and untreated patients were parallel, despite treated patients having more severe disease at baseline. The persisting differences between the groups suggest no overall effect, although improvement or stabilization may be seen in some patients. Prospective studies are needed to define subsets of patients with unclassifiable interstitial lung disease and their optimal management.     

Evaluating factors influencing screening for pulmonary hypertension in systemic sclerosis: does disparity between available guidelines influence clinical practice?

Pulmonary arterial hypertension (PAH) is one of the leading causes of mortality in systemic sclerosis (SSc). We audited adherence with available recommendations regarding cardiopulmonary screening for PAH in SSc and explored potential factors influencing clinical practice. A retrospective case note review of 108 patients with SSc who had attended outpatient clinic over the previous year was undertaken. Records were scrutinised for evidence of previous assessment with trans-thoracic echocardiography (TTE) and pulmonary function tests (PFT), along with information regarding clinical phenotype and serological subset. The proportion of patients for whom screening had been undertaken within the previous 12 months was low, with significantly fewer having TTE compared with PFT assessment (34.7% vs. 53.1%, p = 0.014). The majority of patients had undergone TTE and PFT assessment within the previous 2 years, but a lower proportion had undergone TTE compared with PFT (69.4% vs. 82.7%, p = 0.044). There were strong trends for more frequent PFT assessment in younger patients, limited cutaneous SSc and worse previous PFT results. In contrast, the frequency of TTE assessment was not associated with previous investigation results or disease subtype. Serological profile did not influence the frequency of either TTE or PFT assessments. Disparity between available published guidelines may influence both the frequency and preference of PAH screening in SSc in clinical practice. The higher frequency of PFT assessment might reflect a perceived superiority amongst clinicians of PFT over TTE in the early identification of SSc-PAH. SSc-specific guidelines, possibly incorporating additional independent risk factors, may improve the cost-effectiveness and clinical efficacy of screening recommendations designed to ensure the early identification of PAH in patients with SSc.     

Two‐year experience with mycophenolate mofetil in patients with scleroderma lung disease: a case series

To assess the effect of mycophenolate mofetil (MMF) on pulmonary functions in patients with systemic sclerosis‐associated lung disease (SSc‐ILD) who experienced an inadequate response to first line cyclophosphamide (CYC) therapy. Twelve consecutive SSc‐ILD patients who received MMF due to inadequate response to CYC as a first line agent, were retrospectively reviewed. Over the course of 2 years, pulmonary function tests (PFT) and high‐resolution computed tomography (HRCT) scans were performed. Following initial baseline tests, PFTs were continued at a frequency of every 6 months and HRCT scans were performed every 12 months. After MMF treatment, values of forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO) improved in three (25%) and two (16.6%) patients, respectively. It is also noted that the evaluation of serial HCRT scans showed no change in 54.5% of patients. Our case series suggested that PFT and imaging scores seemed to be stabilized by MMF in SSc‐ILD patients who were inadequate responders to CYC.     

Pulmonary survival study in 91 patients with systemic sclerosis

In systemic sclerosis (SSc), major determinant of morbidity and mortality is pulmonary complication including pulmonary interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). In this study, the natural course of pulmonary involvement in SSc patients was investigated. This was a historical cohort study of SSc patients at a referral center for SSc in Iran between February 1998 and December 2007. Patients had a standardized initial evaluation, and interstitial pulmonary involvement was established by high-resolution CT scan (HRCT). Pulmonary hypertension was assessed by tricuspid gradient on echocardiography. Development of abnormal FVC or DLCO was considered as secondary outcome. Analysis of pulmonary survival was performed for primary and secondary outcomes. Ninety-one SSc patients were included in the study with the mean age of 44.1 (14.8). Among these, 65 (71.4%) patients were classified as limited subtype (lcSSc) and 84 (93.3%) were women. PAH was investigated in 8 (8.2%) patients, 1 (6.7%) in dcSSc and 7 (15.9%) in lcSSc subtype of disease. ILD had developed after a median of 107 (SE = 24.4) months after the first symptom of SSc, and 29 patients (31.9%) developed pulmonary fibrosis. Alveolitis and fibrosis had developed after a median of 129.0 (22.9) and 259.0 (74.2) months, respectively. There was a significant difference in Alveolitis-free pulmonary survival between two subgroups of the disease, which showed pulmonary alveolitis developed later in limited SSc (P = 0.03). The difference was not significant in two subtypes when Cox regression model was used to identify the effect of other prognostic factors on pulmonary survival in patients. In the present study, clinical manifestations of two subtypes of disease were divergent at first; however they became convergent in late stages, and this was the same as results in previous studies. Echocardiography for evaluation of pulmonary hypertension and pulmonary function tests for early detection of ILD and PAH is recommended for SSc patients to detect early stages of pulmonary involvement before significant vascular and fibrotic changes occur.     

Clinical characteristics of non-idiopathic pulmonary fibrosis,progressive fibrosing interstitial lung diseases: A single-center retrospective study

Progressive fibrosing interstitial lung disease (PF-ILD) is a progressive phenotype of fibrosing ILDs with varying definitions and elusive clinical characteristics. We aimed to clarify the clinical features and prognosis of PF-ILD cases based on the deterioration of pulmonary function.Altogether, 91 consecutive ILD patients who underwent at least 2 pulmonary function tests (PFTs) with an interval of at least 24 months, as the screening period, between January 2009 and December 2015 were retrospectively reviewed. The deterioration of forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DLco) was calculated based on PFT data and screening period. The definition of PF-ILD was

• 1.relative decline of 10% or more in FVC per 24 months or
• 2.relative decline in FVC of 5% or more with decline in DLco of 15% or more per 24 months.

Medical records of 34 patients with idiopathic pulmonary fibrosis (IPF), 11 patients with non-IPF, PF-ILD, and 46 patients with non-IPF, non-PF-ILD were retrospectively analyzed. Patient characteristics, pharmacologic or non-pharmacologic treatment status, and prognosis were compared between the IPF and non-IPF groups and between the non-IPF, PF-ILD and non-IPF, non-PF-ILD groups.Eleven patients (19.3%) showed a progressive phenotype in the non-IPF group. The pulmonary function data at the first PFT were worse in non-IPF, PF-ILD patients than in non-IPF, non-PF-ILD patients. There were no differences in the proportion of patients who were observed without pharmacologic treatment or of those receiving pharmacologic treatment between the non-IPF, PF-ILD and non-IPF, non-PF-ILD groups. Low %FVC at the first PFT and the usual interstitial pneumonia-like fibrotic pattern on high-resolution computed tomography were risk factors for PF-ILD in the non-IPF group. The mortality in the non-IPF, PF-ILD group was significantly worse than that of the non-IPF, non-PF-ILD group and was as poor as that of the IPF group. Multivariate logistic analysis showed that aging and low %DLco at the first PFT were risk factors for mortality within the non-IPF group.The prognosis of non-IPF, PF-ILD patients was as poor as that of IPF patients. Non-IPF, PF-ILD patients require more intensive treatment before disease progression.     

Intravenous cyclophosphamide pulse therapy in interstitial lung disease associated with systemic sclerosis in a retrospective open-label study: influence of the extent of inflammation on pulmonary function

Interstitial lung disease (ILD) is the primary cause of death in patients with systemic sclerosis (SSc). It is thought that chronic inflammation is a key component in SSc-ILD. Treatment, such as cyclophosphamide (CYC), targets this inflammation. We hypothesized that treatment with CYC might be more effective in the inflammatory phase. Therefore, we analyzed whether the extent of inflammation, as assessed by the proportion of ground glass compared to fibrosis, SSc disease duration, the extent of ILD, or baseline diffusion capacity of the lungs (DLCO) <?60%, modifies the effect of intravenous CYC pulse therapy (750 mg/m²) on pulmonary function (as measured by FVC, DLCO) in SSc-ILD patients, after 12, 24, and 36 months. Consecutive patients with SSc-ILD receiving CYC pulses between 2003 and 2015 were included. Pulmonary function tests were performed at 0, 6, 12, 24, and 36 months. There were 75 patients included. Forced vital capacity (FVC) (86% of predicted) and DLCO (42% of predicted) were stable after 12, 24 and 36 months of follow-up (p?>?0.05). Forty-four patients completed 12 cycles of CYC. For the extent of ILD, proportion of ground glass compared to fibrosis, SSc disease duration, and baseline DLCO, there were no differences (all p?>?0.05) in the course of FVC and DLCO. Treatment with CYC followed by maintenance therapy stabilizes pulmonary function in patients with SSc-ILD over a 3-year period. The extent of ILD, proportion of ground glass, SSc disease duration, and baseline DLCO <?60% did not influence the effect of CYC on pulmonary function.     

Long-term iloprost infusion therapy for severe pulmonary hypertension in patients with connective tissue diseases

Objective. To determine the effects of short-term, maximum-tolerated-dose and long-term, optimum-dose iloprost treatment of severe pulmonary hypertension associated with systemic sclerosis (SSc) and the primary antiphospholipid syndrome (APS). Methods. Three patients with SSc and 2 with APS who had failed to respond to oral vasodilator therapy for pulmonary hypertension were enrolled in a 32-week, open, prospective trial. Short-term infusion of maximum-tolerated doses and continuous infusion of optimum doses of iloprost were carried out following baseline cardiac catheterization. Catheterization was repeated at 2 and 32 weeks. All 5 patients completed the study and continued therapy for an average of 82 weeks (range 58–103). Results. Acute infusion of maximum tolerated doses significantly ameliorated the cardiac index (0.92 liters/minute/m²; P < 0.01), pulmonary artery O₂ saturation (10.6%; P < 0.05), and pulmonary resistance (−6.7 units; P < 0.05). After 2 weeks of continuous infusion of optimum doses, there was improvement in pulmonary resistance (⩾16%) and pulmonary artery O₂ saturation ( > 30%) in the 2 patients with primary APS. After 2 and 32 weeks, the 3 SSc patients showed variable hemodynamic responses. New York Heart Association functional class and exercise tolerance improved in all patients. There was 1 episode of bacteremia, and 1 patient died after 72 weeks of study. Conclusion. Continuous iloprost infusion may improve exercise tolerance and quality of life in patients with severe pulmonary hypertension associated with SSc and primary APS.     

Coexisting COPD in elderly asthma with fixed airflow limitation: Assessment by DLco %predicted and HRCT

Background: Asthma patients with fixed airflow limitation (FL) are theoretically classified into two phenotypes, that is, coexisting chronic obstructive pulmonary disease (COPD) and asthmatic airway remodeling. However, the precise percentages of such patients are not known. Objective: To assess the prevalence of patients with both FL and COPD components in elderly asthma. Methods: We evaluated patients by lung diffusion impairment and emphysematous findings in high-resolution computed tomography (HRCT) as candidates for COPD components, as a multicenter, cross-sectional survey. Asthma outpatients ≥ 50 years of age were enrolled from Tohoku University Hospital, Sendai, Japan, and four hospitals (Tohoku Medical and Pharmaceutical University Wakabayashi Hospital, Sendai, JAPAN; Wakayama Medical University Hospital, Kimiidera, Japan; Hiraka General Hospital, Yokote, Japan; Iwate Prefectural Isawa Hospital, Oshu, Japan) with pulmonary physicians from March 1, 2013 to November 30, 2014. Results: The prevalence of patients with FEV₁/FVC <70% was 31.0% of those in their 50s, 40.2% of those in their 60s and 61.9% of those in their 70s or older. The prevalence of those patients with lung diffusion impairment (i.e. the percent predicted values of diffusing capacity of the lung for carbon monoxide (DLco %predicted) <80%) or emphysematous findings in HRCT (i.e. the appearance of low attenuation area (LAA)) was 18.3% of those in their 50s, 13.8% of those in their 60s and 35.7% of those in their 70s or older. Conclusions: Nearly half of the patients with FL in elderly asthma show coexisting COPD components when assessed by DLco %predicted and LAA in HRCT.     

Pulmonary function changes in children after transcatheter closure of atrial septal defect

This study was performed to assess changes in pulmonary function test (PFT) and pulmonary outcome after transcatheter closure of atrial septal defect (ASD) in pediatric patients. A total 55 pediatric patients undergoing transcatheter ASD closure received PFT at baseline (day before ASD closure), and at 3 days and 6 months after procedure. Forced vital capacity (FVC), forced expired volume in 1 sec (FEV₁), FEV₁ to FVC ratio (FEV₁/FVC), peak expiratory flow (PEF), and mean forced expiratory flow during the middle half of FVC (FEF_25–75) were measured. Individually, subjects were classified by spirometry as normal, obstructive or restrictive, to evaluate the effect of transcatheter closure on pulmonary outcome. These 55 children had significantly reduced mean PEF and FEF_25–75 (84 ± 24%, P = 0.040 and 76 ± 22%, P = 0.010, respectively) at baseline, with FEF_25–75 reduced significantly at 3 days and 6 months (78 ± 24%, P = 0.010 and 81 ± 24%, P = 0.040, respectively) after transcatheter closure. Six months after transcatheter closure of ASD, significant improvement was observed in mean FVC (94 ± 19% vs. 98 ± 15%, P = 0.034) and FEV₁ (90 ± 20% vs. 96 ± 19%, P = 0.008). Assessed individually, better pulmonary outcome was found in patients without pulmonary hypertension (PH) (χ² = 8.333, P = 0.044). PFT disturbance was observed in significant flow limitation in the peripheral airway of ASD patients. Improved PFT was found after transcatheter closure and better pulmonary outcome was observed in patients without PH. ASD children need monitoring pulmonary function and should receive transcatheter closure before PH develops. Pediatr Pulmonol. 2009; 44:1025–1032. ©2009 Wiley‐Liss, Inc.     

The relationship between serum immunoglobulin levels and pulmonary involvement in systemic sclerosis

OBJECTIVE: To examine the relationship between serum immunoglobulin (Ig) levels and pulmonary function in patients with systemic sclerosis (SSc). METHODS: Twenty-four patients with SSc who had at least 2 sets of pulmonary function tests (PFT) at intervals of more than one year were eligible. Multiple linear regression models were constructed for prediction of the annualized rates of change of forced vital capacity (FVC), carbon monoxide diffusing capacity (DL(CO)), and DL(CO) per unit alveolar volume (K(CO)). RESULTS: The rates of change of FVC and K(CO) correlated with the annualized rate of change of IgG (p < 0.001 and p = 0.005, respectively), and the rate of change of DL(CO) correlated with the serum IgM level at the first PFT (p = 0.020) and with the annualized rate of change of IgG (p = 0.007). CONCLUSION: The rates of change of serum Ig levels are associated with those of pulmonary function in SSc. Use of this model may assist investigation of pulmonary involvement.     

INCREASE OF PULMONARY DIFFUSING CAPACITY IN SYSTEMIC SCLEROSIS

Pulmonary function tests and chest radiographs of 29 non-smokingsystemic sclerosis (SSc) patients were analysed, featuring anapparently paradoxic finding of an increased diffusing lungcapacity for carbon monoxide (DLCO). Twenty-one patients (72%)had abnormal pulmonary function, 11 of them had restrictivedisease (38%), six (21%) had isolated DLCO increase, four(14%)had isolated DLCO reduction, while two patients had obstructivedisease (7%). Chest X-ray revealed interstitial abnormalitiesconsistent with pulmonary fibrosis in all four patients withisolated DLCO reduction, in one obstructive patient and in sixrestrictive patients. Inpatients with DLCO increased steroidtreatment significantly reduced DLCO (P<0.05) and membraneDLCO component (Dm) (P<0.05). Hitherto unobserved findingof DLCO increase in SSc patients was associated with shorterduration of SSc (P<0.05), normal lungmechanics and roentgenogram(P<0.05) and absence of pulmonary symptoms (P<0.05). Thefindings that in some SSc patients DLCO increases suggest thatDLCO might prove to be an early and sensitive indicator of acutepulmonary involvement. KEY WORDS: Scleroderma, Pulmonary function tests, Pulmonary diffusing capacity, Memebrane diffusing capacity, Pulmonary capillary blood volume, Corticosteroids