scholarly journals AB0597 PULMONARY FUNCTION IN PATIENTS DIAGNOSED OF EARLY SYSTEMIC SCLEROSIS: A NEW TOOL FOR SYSTEMIC SCLEROSIS CLASIFFICATION?

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1595.1-1595
Author(s):  
F. M. Ortiz Sanjuan ◽  
C. Pávez Perales ◽  
E. Vicens Bernabeu ◽  
C. Alcañiz Escandell ◽  
I. Cánovas Olmos ◽  
...  

Background:Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc) and is often progressive and has a poor prognosis. A restrictive ventilatory defect could suggest ILD either alone or in combination with pulmonary arterial hypertension.Nowadays, Early-SSc is well defined as preliminary stage of SSc. Patients who meet criteria for Early-SSc could benefit from an early diagnosis of pulmonary involvement.Objectives:Our aim was to assess the pulmonary function in patients diagnosed of Early SSc.Methods:Retrospective observational study of a wide and unselected series of patients diagnosed as Early-SSc from a single university hospital from 2012 to 2019. Patients were classified as Early-SSc following Le Roy criteria. Despite this, patients already did not meet 2013 ACR/EULAR classification criteria for SSc. We reviewed pulmonary function through conventional spirometry and diffusing capacity of lung for carbon monoxide (DLCO).Results:We included 56 patients with a mean age of 52.3±12.1 years (96.4% women; 3.6% men).At the diagnosis of Early-SSc, no one of our patients evidenced a restrictive ventilatory pattern. DLCO was below normal limits in 18 patients (32.1%). Small airway obstruction expressed according decreased maximal (mid-) expiratory flow (MMEF) 25-75 was present in 24 patients (42.8%).After a mean follow-up period of 38.3±2.4 months, 29 (51.8%) patients fulfilled 2013 ACR/EULAR criteria. The average time between diagnosis of Early-SSc and achieve SSc classification was 24.4±1.8 months. The remaining 27 patients continued classified as Early-SSc.An analysis of the subgroup of patients which progressed to SSc showed that DLCO was decreased in 15 of those 29 patients (51.7%) and 18 of 29 patients (62.1%) presented decreased MMEF 25-75. Comparing with the subgroup of patients which not progressed to SSc were significant differences (Decreased DLCO: 51.7% vs 11.1%; p=0.02 and decreased MMEF 25-75: 42.8% vs 22.2%; p=0.05).The analysis of pulmonary function of the subgroup of patients continued classified as Early-SSc after follow-up period did not show significative changes after follow-up.Conclusion:In our study, a third of the patients classified as Early-SSc presented at diagnosis abnormal values of DLCO and/or signs of small airway obstruction without the presence of a restrictive ventilatory pattern. Moreover, this pulmonary disfunction was significantly more frequent in patients who progressed to definitive SSc. Patients which remains classified as Early-SSc did not experience significative changes.Our results support the concept that pulmonary function was impaired in Early-SSc and that I should probably be considered for future Early-SSc classification criteria.Disclosure of Interests:None declared

Author(s):  
Ahmed Jalwa ◽  
Rahul Magazine ◽  
ROHIT SINGH ◽  
Shama Shetty

Introduction Allergic rhinitis, beginning from childhood, is a global health problem. According to the literature, allergic rhinitis has been found to be associated with asthma and other allergic manifestations. In this study we like to find out the significance and prognostic importance of pulmonary function test (PFT) in allergic rhinitis. Design and setting: The study was carried out over a period of 2 years, with 63 cases and controls each. Subjects in the age of 20-55 years with allergic rhinitis and SFAR score of >/=7 were included as a case. Participants were interviewed and sent for PFT. Controls were recruited from the retrospective data of healthy individuals with pulmonary function parameters done for health checkup. These controls had an SFAR score of <7. Main outcome measures: Clinical data and PFT of cases and controls was used for the study. SFAR score was used to recruit the cases and controls. All the data obtained were analyzed and compared between cases and controls. Results The mean age of the cases and controls were 33.17 +/- 10.817 and 44.41 +/- 7.4, respectively. Majority of cases and controls were males (60.3% and 57.1%). A statistically significant difference in FEF25-75% among cases and controls was noted (p=0.00), thus proving probability of developing small airway obstruction in subjects with allergic rhinitis. Conclusion Subjects with allergic rhinitis have a probability of developing small airway obstruction with subclinical changes, hence necessitating the need of regular follow-up.


PEDIATRICS ◽  
1975 ◽  
Vol 56 (5s) ◽  
pp. 868-879
Author(s):  
Gerd J. A. Cropp ◽  
I. J. Schmultzler

We evaluated clinical status and pulmonary function in 60 perennially asthmatic and 11 normal children before and repeatedly after incrementally increasing bicycle ergometer exercise. The changes in clinical status and pulmonary function which were elicited by strenuous exercise were graded by an air exchange and a physiological grading system respectively. Normal children showed no significant clinical or physiological changes after exercise. Strenuous exercise elicited significant deteriorations in clinical and physiological measurements in 36% to 77% of asthmatic girls and 46% to 90% of asthmatic boys, the frequency depending on the test used to determine exercise-induced abnormalities. The incidence of exercise-induced asthma (EIA) was statistically significantly higher in asthmatic boys than girls. The higher incidence of EIA in boys was primarily due to a larger number of very severe attacks in boys than girls; mild and moderate EIA was about equally common in the two sexes. Most patients with EIA developed large and small airway obstruction, although large airway obstruction tended to be the predominant and the more severe abnormality. Clinical and physiological abnormalities, regardless of severity, were usually most marked during the first ten minutes after exercise and lessened thereafter. Mild EIA usually lasted for only 15 minutes or less; severe EIA improved, but usually did not resolve within 35 minutes of exercise. There were three patients in whom the severity of EIA got worse after exercise and an additional seven in whom the improvement was minimal. In these ten patients isoproterenol aerosol terminated EIA, indicating that exercise-induced large and small airway obstruction in asthmatic children is primarily. if not solely. due to bronchospasm.


PEDIATRICS ◽  
1975 ◽  
Vol 56 (5s) ◽  
pp. 860-867
Author(s):  
Gerd J. A. Cropp ◽  
I. J. Schmultzler

Sixty asthmatic children were exercised on a bicycle ergometer and had pulmonary function tests performed before and repeatedly after exercise. Pulmonary function measurements included airway resistance (Raw), specific airway conductance (SGaw) functional residual capacity (FRC), peak expiratory flow rate (PEFR), maximum mid-expiratory flow (MMEF), forced expiratory volume during first second of expiration (FEV1), and forced vital capacity (FVC). At any one time during the post-exercise observation period decreases in SGaw were greater than changes in any other pulmonary function test, making SGaw the most sensitive test for the detection. of exercise-induced airway obstruction in asthmatics. Beyond five minutes after exercise PEFR and MMEF were reduced by exercise approximately equally, but somewhat less often and less markedly than SGaw. Exercise-induced reductions in FEV1 were less marked and less frequent than decreases in PEFR and MMEF, and reductions in FVC were the least severe and least often observed abnormality. Decreases in SGaw were significantly, but not linearly correlated with decreases in PEFR, MMEF, FEV1,, FVC, and FEV1/FVC. There were statistically significant linear correlations between exercise-induced increases in FRC and decreases in FVC and between increases in Raw and FRC. If we accept that increases in Raw and FRC indicate increases in large and small airway obstruction respectively, exercise-induced decreases in FVC may indirectly suggest acute hyperinflation and thus small airway obstruction. Although the positive correlation between Raw and FRC indicated that both large and small airway obstruction developed after exercise in many of our asthmatics, increases in Raw were usually greater than increases in FRC, suggesting that large airway obstruction tends to be greater than small airway obstruction in exercise-induced asthma.


1984 ◽  
Vol 143 (2) ◽  
pp. 197-204
Author(s):  
MASAHARU SUGIYAMA ◽  
HIDETADA SASAKI ◽  
HIROSHI INOUE ◽  
MASAO NAKAMURA ◽  
TAKAO SASAKI ◽  
...  

CHEST Journal ◽  
2015 ◽  
Vol 148 (4) ◽  
pp. 1075A
Author(s):  
Banu Salepci ◽  
Ali Fidan ◽  
Elif Torun Parmaksiz ◽  
Esma Coskun ◽  
Nesrin Kiral ◽  
...  

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Julien Guiot ◽  
Makon-Sébastien Njock ◽  
Béatrice André ◽  
Fanny Gester ◽  
Monique Henket ◽  
...  

AbstractSystemic sclerosis (SSc) is a rare connective tissue disease associated with rapid evolving interstitial lung disease (ILD), driving its mortality. Specific biomarkers associated with the progression of this lung disease are highly needed. We aimed to identify specific biomarkers of SSc-ILD to predict the evolution of the disease. For this, we compared prospectively serum levels of several biomarkers associated with lung fibrosis in SSc patients (n = 102), among which SSc-no ILD (n = 63) and SSc-ILD (n = 39), compared to healthy subjects (HS) (n = 39). We also performed a longitudinal study in a subgroup of 28 patients analyzing biomarkers variations and pulmonary function tests over a period of 2 years. Serum level of IGFBP-2 was significantly increased in SSc patients compared to HS, and negatively correlated with pulmonary function (assessed by carbon monoxide transfer coefficient (KCO)) (r = − 0.29, p < 0.01). Two-year longitudinal analysis in a subgroup of 28 SSc patients determined that IGFBP-2 variation was positively correlated with KCO at 2-year follow-up (r = 0.6, p < 0.001). SSc patients with a lower variation of IGFBP-2 (less than 22%) presented significant deterioration of pulmonary function at 2-year follow-up (p < 0.01). ROC curve analysis enabled us to identify that baseline IGFBP-2 > 105 ng/ml was associated with a poor outcome (KCO < 70% predicted) at 2-year follow-up (AUC = 0.75, p < 0.05). We showed for the first time that serum levels of IGFBP-2 might be a prognostic factor of the development of SSc-ILD.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 159.1-159
Author(s):  
R. Dobrota ◽  
S. Jordan ◽  
P. Juhl ◽  
B. Maurer ◽  
M. O. Becker ◽  
...  

Background:Timely diagnosis of patients with very early systemic sclerosis (veSSc) is essential for their personalized and optimal management. We hypothesise that changes in serum-based extracellular matrix (ECM) turnover biomarkers are already detectable in patients with veSSc, even before occurrence of specific clinical signs.Objectives:To investigate circulating ECM turnover markers as potential biomarkers for veSSc.Methods:Patients with veSSc, n=42, defined as presence of Raynaud’s syndrome and at least one of puffy fingers, positive antinuclear antibodies or pathological nailfold capillaroscopy, who did not meet any classification criteria for SSc, were compared to healthy controls (HC, n=29). Longitudinal assessment, data and sera collection were conducted by EUSTAR standards. ECM-degradation (BGM, C3M, C4M, C6M) and ECM-formation biomarkers (PRO-C3, PRO-C4, PRO-C5) were measured in serum using ELISA assays. The statistical analyses included Mann-Whitney U, Spearman correlation and ROC analysis. Using Kaplan-Meier plots and univariable Cox regression, we explored if biomarkers can predict progression towards definite SSc (fulfillment of ACR/EULAR criteria or minimum two points increase in the criteria score) during the longitudinal follow-up.Results:Compared to HC, veSSc patients showed a deregulated turnover of type III and IV collagen, with higher degradation (higher C3M, C4M, both p<0.0001 and PRO-C3, p=0.004, Figure 1a, resulting in lower turnover ratios PRO-C3/C3M and PRO-C4/C4M, both p<0.0001). The biglycan degradation biomarker BGM was also higher in veSSc (p=0.006), whereas the degradation biomarker for type VI collagen, C6M, was lower than in HC (p=0.002). In the ROC analysis, biomarkers of type III and IV collagen distinguished between veSSc and HC: C3M, AUC=0.95, p<0.0001; C4M, AUC=0.97, p<0.0001; turnover ratios PRO-C3/C3M, AUC=0.80, p<0.0001; PRO-C4/C4M, AUC=0.97; p<0.0001 (Figure 1b).Median follow up was 4.5 years (range 0.5-7.9 years), mean age was 50±2.2 years, 88% female gender, 24% with puffy fingers, 92% were ANA positive, 64% had an abnormal capillaroscopy, none had organ involvement or skin fibrosis. 14/42 veSSc patients fulfilled the ACR/EULAR classification criteria at follow-up (time to fulfilment of criteria ranged between 0.5 and 6.8 years from inclusion) and in addition, 18/42 veSSc patients gained at least two classification criteria-points. This resulted in 14, respectively 18 progressors for the longitudinal analysis. However, in univariable Cox regression, the baseline levels of the markers did not predict progression over time.Conclusion:ECM turnover is already altered in veSSc patients compared to HC. Biomarkes of type III and IV collagen distinguished between veSSc patients and HC, which may indicate them as potential biomarkers for the detection of veSSc in addition to the established immunological and capillaroscopic criteria.Disclosure of Interests:Rucsandra Dobrota: None declared, Suzana Jordan: None declared, Pernille Juhl Employee of: Nordic Bioscience, Britta Maurer Grant/research support from: AbbVie, Protagen, Novartis, congress support from Pfizer, Roche, Actelion, and MSD, Speakers bureau: Novartis, Mike O. Becker: None declared, Carina Mihai: None declared, Anne-Christine Bay-Jensen Shareholder of: Nordic Bioscience A/S, Employee of: Full time employee at Nordic Bioscience A/S., Morten Karsdal Shareholder of: Nordic Bioscience A/S., Employee of: Full time employee at Nordic Bioscience A/S., Anne Sofie Siebuhr Employee of: Nordic Bioscience, Oliver Distler Grant/research support from: Grants/Research support from Actelion, Bayer, Boehringer Ingelheim, Competitive Drug Development International Ltd. and Mitsubishi Tanabe; he also holds the issued Patent on mir-29 for the treatment of systemic sclerosis (US8247389, EP2331143)., Consultant of: Consultancy fees from Actelion, Acceleron Pharma, AnaMar, Bayer, Baecon Discovery, Blade Therapeutics, Boehringer, CSL Behring, Catenion, ChemomAb, Curzion Pharmaceuticals, Ergonex, Galapagos NV, GSK, Glenmark Pharmaceuticals, Inventiva, Italfarmaco, iQvia, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Roche, Sanofi and UCB, Speakers bureau: Speaker fees from Actelion, Bayer, Boehringer Ingelheim, Medscape, Pfizer and Roche


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