Association of Gastroesophageal Factors and Worsening of Forced Vital Capacity in Systemic Sclerosis

2013 ◽  
Vol 40 (6) ◽  
pp. 850-858 ◽  
Author(s):  
Xuli Jerry Zhang ◽  
Ashley Bonner ◽  
Marie Hudson ◽  
Murray Baron ◽  
Janet Pope ◽  
...  

Objective.Interstitial lung disease (ILD) is a common complication of systemic sclerosis (SSc) and causes death. Once lung fibrosis occurs, disease course may become stable or decline. Little is known about risks for progression. We studied SSc–gastroesophageal (GE) involvement in relation to worsening forced vital capacity (FVC) on pulmonary function tests (PFT) to investigate whether it was related to progression. Our objective was to determine whether GE reflux and dysphagia are associated with progressive moderate/severe ILD as measured by PFT over 3 years.Methods.The Canadian Scleroderma Research Group is a multicenter SSc database that collects data annually. Using indicators of GE involvement and annual PFT, comparisons were made between no/mild ILD, stable moderate/severe ILD, and progressive moderate/severe ILD groups based on changes of FVC. Multivariate analyses determined associations between GE factors and ILD development and progression.Results.There were 1043 patients with SSc (mean age 55.7 yrs, mean disease duration 10.8 yrs); one-quarter had pulmonary fibrosis on chest radiograph that was related to FVC percentage predicted (Spearman’s rho −0.39; p < 0.01). Physician indicators such as esophageal dysmotility (p = 0.009) and postesophageal dilatation (p = 0.041), and patient indicators such as difficulty swallowing (p = 0.016) and waking up choking (p = 0.026) were associated with low FVC. In comparing progressive and stable moderate/severe FVC (< 70% predicted), early satiety (p = 0.018) and a combination term of postdilatation and choking (p = 0.042) increased risk of progression of ILD. Topoisomerase I was not associated with progression over followup.Conclusion.Symptoms of esophageal dysmotility were associated with worsening FVC in SSc, especially if both need for esophageal dilatation and choking were present.

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Svetlana I Nihtyanova ◽  
Emma C Derrett-Smith ◽  
Carmen Fonseca ◽  
Voon H Ong ◽  
Christopher P Denton

Abstract Background Pulmonary fibrosis (PF) is common in systemic sclerosis (SSc) and serial pulmonary function tests (PFTs) are used for routine PF monitoring. Forced vital capacity (FVC) decline reflects progression in PF and FVC is frequently used as an endpoint in clinical trials. We explore the changes in FVC over time in patients with SSc-PF, receiving standard management, including immunosuppression. Methods Only SSc patients with CT-confirmed PF were included. FVC changes over the first 10 years from onset and their associations were assessed using linear mixed effects models. Predictors of time from first PFT to development of threshold FVC (FVC&lt;70% and FVC&lt;50%) were analysed using Cox regression. Results We identified 505 SSc-PF subjects, 21.6% were male, average age at onset was 47 years and 49.3% had diffuse cutaneous subset (dcSSc). The most common autoantibody was anti-Scl70 in 40.4%, followed by anti-RNA polymerase (ARA) in 11.7% and anti-centromere (ACA) in 7.1%. In 16.4% of the patients, ANA was positive, but no ENAs were identified (ANA+ENA-). Average FVC at 12 months from onset was 80.1% (SD 19.3) and this declined by 0.32% per year (p = 0.007) at a group level. There was no significant correlation between baseline FVC and subsequent change (correlation coefficient -0.13; 95%CI -0.26, 0.01). For every year older age at onset, average FVC increased by 0.32%, p &lt; 0.001. Males had 3.3% lower FVC at 1 year from onset (p &lt; 0.001) and 0.6% faster decline per year (p = 0.034) compared to females. DcSSc subjects had 5.6% lower FVC compared to limited disease (p = 0.003). Average FVC at 1 year from onset in ARA+ subjects were higher than any other antibody (15.1% v. ANA+ENA-, p &lt; 0.001; 14.6% v. ATA, p &lt; 0.001 and 12.5% v. ACA, p = 0.010). Nevertheless, ARA+ subjects had FVC decline rates similar to ATA+ and ANA+ENA- subjects, while ACA+ patients had a small but significant increase in FVC over time. Factors that increased the risk for FVC drop below the thresholds were male sex, ATA and low baseline FVC (Table 1). Conclusion This study provides insight into long-term patterns of FVC change and develops a model that may help predict those most at risk of significant decline. Disclosures S.I. Nihtyanova None. E.C. Derrett-Smith None. C. Fonseca None. V.H. Ong None. C.P. Denton None.


2018 ◽  
Vol 45 (11) ◽  
pp. 1572-1576 ◽  
Author(s):  
Kimberly Showalter ◽  
Aileen Hoffmann ◽  
Gerald Rouleau ◽  
David Aaby ◽  
Jungwha Lee ◽  
...  

Objective.Forced vital capacity (FVC) and DLCO are used for screening of systemic sclerosis–associated interstitial lung disease (SSc-ILD). The study purpose was to determine the sensitivity, specificity, and negative predictive value (NPV) (proportion of true negative screening tests) of FVC and DLCO thresholds for SSc-ILD on chest high-resolution computed tomography (HRCT) scans.Methods.Patients fulfilling American College of Rheumatology 2013 SSc criteria with a chest HRCT scan and pulmonary function tests (PFT) were studied. A thoracic radiologist quantified radiographic ILD. Optimal FVC and DLCO % predicted thresholds for ILD were identified using receiver-operating characteristic curves. The FVC and DLCO combinations with greatest sensitivity and specificity were also determined. Subanalysis was performed in patients with positive Scl-70 autoantibodies.Results.The study included 265 patients. Of 188 (71%) with radiographic ILD, 59 (31%) had “normal” FVC (≥ 80% predicted), and 65 out of 151 (43%) had “normal” DLCO (≥ 60% predicted). FVC < 80% (sensitivity 0.69, specificity 0.73), and DLCO < 62% (sensitivity 0.60, specificity 0.70) were optimal thresholds for radiographic SSc-ILD. All FVC and DLCO threshold combinations evaluated had NPV < 0.70. The NPV for radiographic ILD for FVC < 80% was lower in patients with positive Scl-70 autoantibody (NPV = 0.05) compared to negative Scl-70 autoantibody (NPV = 0.57).Conclusion.Radiographic ILD is prevalent in SSc despite “normal” PFT. No % predicted FVC or DLCO threshold combinations yielded high NPV for SSc-ILD screening. “Normal” FVC and DLCO in patients with SSc, especially those with positive Scl-70 autoantibodies, should not obviate consideration of HRCT for ILD evaluation.


2020 ◽  
Vol 7 (1) ◽  
pp. e000598
Author(s):  
Michael T Durheim ◽  
Anna-Maria Hoffmann-Vold ◽  
Tomas M Eagan ◽  
Arnt-Ove Hovden ◽  
May Brit Lund ◽  
...  

IntroductionIdiopathic pulmonary fibrosis (IPF) and systemic sclerosis-associated interstitial lung disease (SSc-ILD) are fibrotic ILDs with divergent disease populations. Little is known about health-related quality of life (HRQL) in SSc-ILD relative to IPF.MethodsWe used the Kings Brief Interstitial Lung Disease Questionnaire (K-BILD) to compare HRQL in a cross-sectional study of 57 patients with IPF and 29 patients with SSc-ILD. Analysis of covariance was used to adjust for age, gender and lung function.ResultsThe unadjusted mean K-BILD score was 63.1 (95% CI 57.1 to 69.1) among patients with SSc-ILD, as compared with 54.7 (51.8–57.5) among those with IPF (p=0.005). However, this difference in HRQL was attenuated after adjustment for age, gender and lung function. In a multivariable model, only forced vital capacity was associated with K-BILD scores. K-BILD scores were correlated with both forced vital capacity and with other relevant HRQL measures, regardless of ILD diagnosis.DiscussionPatients with SSc-ILD may have better ILD-specific quality of life than patients with IPF, but this difference appears to be driven primarily by better lung function. These results underscore the impact of lung function on HRQL in fibrotic ILD and the utility of K-BILD to assess HRQL in SSc-ILD.


Rheumatology ◽  
2020 ◽  
Vol 60 (1) ◽  
pp. 250-255
Author(s):  
Takashi Nawata ◽  
Yuichiro Shirai ◽  
Mikito Suzuki ◽  
Masataka Kuwana

Abstract Objective To investigate the potential contribution of accessory respiratory muscle atrophy to the decline of forced vital capacity (FVC) in patients with SSc-associated interstitial lung disease (ILD). Methods This single-centre, retrospective study enrolled 36 patients with SSc-ILD who underwent serial pulmonary function tests and chest high-resolution CT (HRCT) simultaneously at an interval of 1–3 years. The total extent of ILD and chest wall muscle area at the level of the ninth thoracic vertebra on CT images were evaluated by two independent evaluators blinded to the patient information. Changes in the FVC, ILD extent, and chest wall muscle area between the two measurements were assessed in terms of their correlations. Multiple regression analysis was conducted to identify the independent contributors to FVC decline. Results Interval changes in FVC and total ILD extent were variable among patients, whereas chest wall muscle area decreased significantly with time (P=0.0008). The FVC change was negatively correlated with the change in ILD extent (r=−0.48, P=0.003) and was positively correlated with the change in the chest wall muscle area (r = 0.53, P=0.001). Multivariate analysis revealed that changes in total ILD extent and chest wall muscle area were independent contributors to FVC decline. Conclusion In patients with SSc-ILD, FVC decline is attributable not only to the progression of ILD but also to the atrophy of accessory respiratory muscles. Our findings call attention to the interpretation of FVC changes in patients with SSc-ILD.


2019 ◽  
Vol 11 (2) ◽  
Author(s):  
Herlina Yani ◽  
Sumartini Dewi ◽  
Andri Reza Rahmadi

Background Pulmonary fibrosis / intersitial lung disease (ILD) in systemic sclerosis (SSc) is a complicated restrictive pulmonary disease and the leading cause of disease-related mortality. Progressive skin fibrosis in diffuse-type SSc (dSSc) is associated with decreased forced vital capacity (FVC). Modified Rodnan Skin Score (mRSS) examination is used as a parameter to assess skin fibrosis, while high-resolution computed tomography (HRCT) and pulmonary function tests (PFTs) are used to assess pulmonary fibrosis. The HRCT test remains as the gold standard in diagnosing ILD. However, it costs a lot and is not available in all healthcare facilities. Krebs Von den Lungen-6 (KL-6) is a biomarker to evaluate pulmonary fibrosis. The aim of this study was to analyze the correlation of serum KL-6 levels with FVC and mRSS value of patients with restrictive lung disease in dSSc. Method This was a cross-sectional study that used primary data from dSSc patients who visited rheumatology outpatient clinic in Hasan Sadikin Hospital Bandung, Indonesia, during the period of June-July 2019. History taking, physical examination, mRSS, spirometry, and serum KL-6 levels were performed. Data were analyzed using the Rank Spearman correlation test.  Result There were 27 subjects with the mean age of 42 ± 12 years. Based on FVC (%) restrictive lung disease criteria, the majority of subjects (74.1%) had severe restrictive lung disease and the rest of all subjects (25.9%) were non severe restrictive lung disease. Serum KL-6 levels ranged from 0.545 to 8.138 ng/ml. The results showed that there was no correlation between serum KL-6 levels and FVC values (r = -0.118, p = 0.279) and mRSS (r = 0.101, p = 0.312 ). Conclusion There is no correlation between serum KL-6 levels with FVC and mRSS value of patient with restritive lung disease in diffuse type systemic sclerosis. Keywords : diffuse type systemic sclerosis, Forced Vital Capacity, KL-6, mRSS, restrictive lung disease.      


Rheumatology ◽  
2019 ◽  
Vol 59 (7) ◽  
pp. 1684-1694 ◽  
Author(s):  
Veronika K Jaeger ◽  
Mohammed Tikly ◽  
Dong Xu ◽  
Elise Siegert ◽  
Eric Hachulla ◽  
...  

Abstract Objectives Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations. Methods SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses. Results The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P &lt; 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P &lt; 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P &lt; 0.001) diffuse skin involvement than had WP. AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P &lt; 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P &lt; 0.001; OR(BP) = 2.4, P &lt; 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P &lt; 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P &lt; 0.001]. Conclusion Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality.


2017 ◽  
Vol 6 (1) ◽  
pp. 21
Author(s):  
N B Mahotra ◽  
T M Amatya ◽  
B SJB Rana ◽  
D Banstola

<p> Due to regular exercises, athletes tend to have an increase in pulmonary functions when compared to non exercising individuals, especially when the exercise is strenuous. Intensity and severity of sports engaged in by the athletes determines the extent of strengthening of the inspiratory muscles with a resultant increase in the lung volumes and capacities. Pulmonary parameters like tidal volume and forced vital capacity are significantly higher in athletes than in non athletes. A cross sectional comparative study was carried out in national sports council, Tribhuvan army club and institute of medicine, Kathmandu, Nepal. The athletes were footballers from Tribhuvan army club and sprinters from national sports council. The non-athletes were medical students from the institute of medicine, Kathmandu. Data were collected after performing spirometry and mean values were compared between athletes and non-athletes. Pulmonary functions were assessed based on forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1) and peak expiratory flow rate (PEFR) expressed as percent predicted for the age, sex, height, weight and race. Spirometry was performed in 169 subjects, out of which 84 were athletes and 85 were non-athletes. Athletes had a significantly superior FVC (P =0.00) compared to non-athletes. FEV1 was also recorded significantly higher in athletes than in non-athletes (P=0.023). Athletes have better pulmonary function tests than non-athletes because exercise in athletes strengthens the muscles of respiration. </p>


Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4594-4594
Author(s):  
Tomasz Czerw ◽  
Malgorzata Widuchowska ◽  
Malgorzata Krawczyk-Kulis ◽  
Dariusz Kata ◽  
Magdalena Kopec-Medrek ◽  
...  

Abstract Abstract 4594 Systemic sclerosis (SSc) is an autoimmune disease characterized by vasculopathy and fibrosis. Patients with diffuse cutaneous subset and organ involvement have poor prognosis and require more effective therapeutic strategies. Recent data show that high-dose immunosuppressive therapy and autologous hematopoietic stem cell transplantation (autoHSCT) may result in clinical improvement in two thirds of those patients when applied in the early course of disease. Between 2003 and 2010 six patients suffering from severe SSc were treated with autoHSCT in Hematology and BMT Department of Medical University of Silesia (SUM) in Katowice, Poland in cooperation with Department of Internal Medicine and Rheumatology of SUM in Katowice. Patients with diffuse SSc (modified Rodnan skin score (mRSS) 16 or greater) and organ (respiratory or cardiac) involvement were enrolled. Patients with progressive pulmonary disease defined as at least 15% decrease in forced vital capacity (FVC) or diffusion capacity of the lung for carbon monoxide (DLCO) in the previous 6 months were also included. Study group characteristic before autoHSCT: 6 patients with diffuse subset of SSc (4 women, 2 men), median age 54 years (30-68), disease duration 24 months (10-87), mRSS 19 (17-34), DLCO 51% (32-77), forced vital capacity (FVC) 70% (62-101), left ventricle ejection fraction 61% (60-70), serum creatinine level 0.8 mg/dL (0.5-1.1), organ dysfunction: lung (4/6 pts); kidney (0/6); heart (3/6); gastrointestinal tract (3/6), autoantibodies: antinuclear (5/6), Scl-70 (5/6), prior therapy: corticosteroids (4/6), cyclophosphamide (5/6). Peripheral blood stem cells were mobilized with G-CSF (10μ g/kg/d) subcutaneously with the first apheresis scheduled on day 5. The number of aphereses required for collection of stem cells was 3.5 (1-5). Cell selection before transplantation was not performed. Conditioning regimen included cyclophosphamide at total dose 200mg/kg given on days -6 to -3 and Campath-1H at total dose 60mg on days -3 to -1 as iv vivo purging of lymphocytes. The median number of transplanted CD34+ cells was 2.6 (2.1-8.1). One patient (68 years old woman with cardiac and lung involvement before transplantation) died three days after autoHSCT because of circulatory insufficiency. All remaining five patients engrafted. Hematopoietic recovery was as follows: neutrophil count above 500/μ l 12 days (9-16), platelet count above 20 000/μ l 8 (7-14). Main early complications after transplantation were infections (bacterial pneumonia – 1 pt, CMV reactivation – 1, FUO – 3, mucositis - 3) and cardiac arrhythmias. Five of six patients are alive at a median observation time 2.5 years (0.5-7). All of them achieved disease improvement after autoHSCT. Major improvement was seen in skin (mRSS) and disability index. Organ function remained stable. Autoantibodies are still detectable. In one case disease progression occured 1.5 years after transplantation requiring systemic treatment. Remaining 4 patients have sustained response without post-transplant therapy. Despite small number of cases this report shows that durable responses can be achieved in the majority of patients with diffuse SSc after autoHSCT. Early qualification to the procedure before severe organ dysfunction occurs is essential to achieve sustained remissions and reduce transplant related mortality. Our findings are consistent with outcomes reported in the literature. Results from prospective randomized clinical trials, such as ASTIS and SCOT trials, comparing high-dose immunoablation and autoHSCT with standard cyclophosphamide therapy will finally determine efficacy of this new treatment modality. Disclosures: No relevant conflicts of interest to declare.


2010 ◽  
Vol 2010 ◽  
pp. 1-5 ◽  
Author(s):  
Antonio Aversa ◽  
Roberto Bruzziches ◽  
Davide Francomano ◽  
Edoardo Rosato ◽  
Felice Salsano ◽  
...  

Systemic Sclerosis (SSc) is a connective tissue disorder featuring vascular alterations and an immunological activation leading to a progressive and widespread fibrosis of several organs such as the skin, lung, gastrointestinal tract, heart, and kidney. Men with SSc are at increased risk of developing erectile dysfunction (ED) because of the evolution of early microvascular tissutal damage into corporeal fibrosis. The entity of penile vascular damage in SSc patients has been demonstrated by using Duplex ultrasonography and functional infra-red imaging and it is now clear that this is a true clinical entity invariably occurring irrespective of age and disease duration and constituting the ‘‘sclerodermic penis’’. Once-daily phosphodiesterase type-5 (PDE5) inhibitors improve both sexual function and vascular measures of cavernous arteries by improving surrogate markers of endothelial dysfunction, that is, plasma endothelin-1 and adrenomedullin levels, which may play a potential role in preventing progression of penile fibrosis and ED. Also, the beneficial effect of long-term PDE5i add-on therapy to SSc therapy in the treatment of Raynaud's phenomenon is described.


Rheumatology ◽  
2015 ◽  
Vol 54 (8) ◽  
pp. 1464-1471 ◽  
Author(s):  
Ada Man ◽  
Todd Davidyock ◽  
Laura T. Ferguson ◽  
Michael Ieong ◽  
Yuqing Zhang ◽  
...  

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