Reduced diffusing capacity for carbon monoxide predicts borderline pulmonary arterial pressure in patients with systemic sclerosis

This study included SSc patients who underwent right heart catheterization (RHC) in our hospital during 2010–2018. Following variables were assessed for performance to predict mPAP ≥ 25 mmHg or >  20 mmHg; anti-centromere or U1-RNP antibody, plasma BNP level, serum urate level, right axis deviation, forced vital capacity (FVC)/diffusing capacity for carbon monoxide (DLCO) ratio, and tricuspid regurgitation velocity. Of 58 patients enrolled in this study, 24 had mPAP of ≥ 25 mmHg an d 9 had mPAP of 21–24 mmHg. Among variables tested, only FVC/DLCO elevated similarly in patients with mPAP of ≥ 25 mmHg (median 2.5) and those with mPAP of 21–24 mmHg (median 2.5) compared to those with mPAP of ≤ 20 mmHg (median 1.5). Given the particularly good correlation betwe en DLCO and mPAP of >  20 mmHg, each variable was weighted according to its odds ratio and the total weighted score was calculated. The total weighted score exhibited a good predictive performance for mPAP of >  20 mmHg with its sensitivity of 87.5% and specificity of 92%. Among conventional risk factors for PAH, decreased DLCO may predict mPAP >  20 mmHg with priority in SSc patients. Weighting DLCO may improve the performance of screening algorithm for early SSc-PAH.
Source: Rheumatology International - Category: Rheumatology Source Type: research

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Abstract Phenotypic differences have been described between patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) and SSc-associated pulmonary hypertension, including performance differences in the 6-min walk test (6MWT). Moreover, the correlations between the 6MWT and traditional pulmonary function tests (PFTs) are weak, indicating the need to search for new parameters that explain exercise performance. Thus, our objective was to evaluate the impact of ventilation distribution heterogeneity assessed by the nitrogen single-breath washout (N2SBW) test and peripheral muscle dysfunction on...
Source: Braz J Med Biol Res - Category: Research Authors: Tags: Braz J Med Biol Res Source Type: research
Pulmonary arterial hypertension (PAH) is a dreaded complication of systemic sclerosis (SSc) that occurs in ~10% of patients. Most individuals present with severe symptoms, significant functional impairment and severe haemodynamics at diagnosis, and survival after PAH diagnosis is poor. Therefore, early diagnosis through systematic screening of asymptomatic patients has the potential to identify PAH at an early stage. Current evidence suggests that early diagnosis and treatment of PAH in patients with SSc may lead to better clinical outcomes. Annual screening may include echocardiography, but this can miss some patients due...
Source: European Respiratory Review - Category: Respiratory Medicine Authors: Tags: Pulmonary vascular disease Review Source Type: research
Lung diffusing capacity for nitric oxide may be of clinical value in systemic sclerosis because more sensitive to interstitial lung disease than standard lung diffusing capacity for carbon monoxide. AbstractA decreased lung diffusing capacity for carbon monoxide (DLCO) in systemic sclerosis (SSc) is considered to reflect losses of alveolar membrane diffusive conductance for CO (DMCO), due to interstitial lung disease, and/or pulmonary capillary blood volume (VC), due to vasculopathy. However, standard DLCO does not allow separate DMCO from VC. Lung diffusing capacity for nitric oxide (DLNO) is considered to be more sensiti...
Source: Physiological Reports - Category: Physiology Authors: Tags: Original Research Source Type: research
ConclusionsThe lung should be carefully studied during CTDs. PFTs can represent an important prognostic tool for diagnosis and follow up of RA-ILD, but, on their own, lack sufficient specificity or sensitivity to describe lung involvement in SSDs and IIMs. Several composite indexes potentially able to describe the evolution of lung damage and response to treatment in SSDs are under investigation. Considering the potential severity of these conditions, an HRCT jointly with PFTs should be performed in all new diagnoses of SSDs and IIMs. Moreover, follow up PFTs should be interpreted in the light of the risk factor for respir...
Source: Multidisciplinary Respiratory Medicine - Category: Respiratory Medicine Source Type: research
Authors: Rizzi M, Radovanovic D, Airoldi A, Cristiano A, Frassanito F, Gaboardi P, Saad M, Atzeni F, Sarzi-Puttini P, Santus P Abstract Systemic sclerosis (SSc) is an autoimmune disease characterised by tissue fibrosis leading to vascular injury. Nitric oxide (NO) has been implicated in the pathogenesis of autoimmune diseases. A deficiency in basal NO production by the constitutive endothelial isoform of nitric oxide synthase may promote vasoconstriction and vascular wall thickening. In January 2017, we searched the PubMed/Medline, Cochrane Library and Enbase/Medline databases for studies analysing physio-pathologi...
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
AbstractObjectiveLysyl oxidase (LOX) is an extracellular enzyme that cross-links collagen fibrils. LOX was found to be increased in serum of SSc patients and was suggested to be related to skin fibrosis, yet a vascular source of LOX has been demonstrated in idiopathic pulmonary arterial hypertension (iPAH). We aimed to validate elevated LOX serum levels in SSc and to study its correlation with clinical characteristics and investigate its main source at the tissue level.MethodsA total of 86 established SSc patients were compared with 86 patients with very early diagnosis of systemic sclerosis (VEDOSS), 110 patients with pri...
Source: Rheumatology - Category: Rheumatology Source Type: research
Introduction: Systemic sclerosis (SSc) is a chronic autoimmune disease with ongoing high disease related mortality. Three randomized controlled trials proofed efficacy of autologous stem cell transplantation (aHSCT) in patients with severe SSc and superiority over the standard cyclophosphamide pulse therapy. Treatment related mortality is a major point of criticism and different treatment regimen are used in different centers.Methods: This prospective, open, multi-center, non-interventional study of the European Group for Blood and Marrow Transplantation (EBMT) analyzed data for consecutive aHSCT performed in SSc. Every ce...
Source: Blood - Category: Hematology Authors: Tags: 731. Clinical Autologous Transplantation: Results: Poster II Source Type: research
Authors: Rizzi M, Radovanovic D, Santus P, Airoldi A, Frassanito F, Vanni S, Cristiano A, Sarzi-Puttini P, Atzeni F Abstract The 6-minute walk test (6MWT) is a standardised, feasible and reliable measure of sub-maximal exercise capacity that has never been fully validated in systemic sclerosis (SSc). A variety of data suggest that many non-pulmonary aspects of SSc contribute to the test results, thus blunting the ability of the 6MWT to measure changes in lung function. Sources of variability are a training effect, technician experience, subject encouragement, medication, other activities on day of testing, decondit...
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
CONCLUSION: Overall survival in PHAROS was higher than other SSc-PAH cohorts. PAH accounted for more than half of deaths and primarily within the first few years after PAH diagnosis. Optimization of treatment for those at greatest risk of early PAH-related death is crucial. PMID: 29777655 [PubMed - as supplied by publisher]
Source: Chest - Category: Respiratory Medicine Authors: Tags: Chest Source Type: research
The objective of this study was to evaluate the incidence of pulmonary hypertension (PH) and determining factors in patients with systemic sclerosis (SSc) and a diffusing capacity of the lung for carbon monoxide (DLCO)
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary vascular disease Original Articles: Pulmonary hypertension Source Type: research
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