Airway obstruction can be better predicted using Global Lung Function Initiative spirometry reference equations in Marfan syndrome.

A M Kolonics-Farkas,Z Kovats,Z Szabolcs,B Agg,B Odler,V Müller,K Benke,A Bohacs

doi:10.1556/2060.2021.00002

Abstract

Marfan syndrome is a genetic disorder of the connective tissue, including involvement of the lungs.Pulmonary function test was performed in 32 asymptomatic adult Marfan patients using European Community for Coal and Steel (ECCS) and Global Lung Function Initiative (GLI) reference values.Using GLI equations for reference, significantly lower lung function values were noted for forced vital capacity (FVC) (87.0 ± 16.6% vs. 97.1 ± 16.9%; P < 0.01) and forced expiratory volume in the first second (FEV1) (79.6 ± 18.9% vs. 88.0 ± 19.1%; P < 0.01) predicted compared to ECCS. Obstructive ventilatory pattern was present in 25% of the cases when calculating with GLI lower limit of normal (LLN), and it was significantly more common in men as compared to women (n = 6, 50% vs. n = 2, 10%; P = 0.03).GLI is more suitable to detect early ventilatory changes including airway obstruction in young patients with special anatomic features, and should be used as a standard way of evaluation in asymptomatic Marfan population.

Highlights

Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder, mainly characterized by vascular and skeletal manifestations, including common involvement of the lungs [1]
Pulmonary function test was performed in 32 asymptomatic adult Marfan patients using European Community for Coal and Steel (ECCS) and Global Lung Function Initiative (GLI) reference values
Using GLI equations for reference, significantly lower lung function values were noted for forced vital capacity (FVC) (87.0 ± 16.6% vs. 97.1 ± 16.9%; P < 0.01) and forced expiratory volume in the first second (FEV1) (79.6 ± 18.9% vs. 88.0 ± 19.1%; P < 0.01) predicted compared to ECCS

Summary

Introduction

Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder, mainly characterized by vascular and skeletal manifestations, including common involvement of the lungs [1]. In 1960 the European Community for Coal and Steel (ECCS) was the first organization to issue recommendations for spirometry and released equations for calculations of reference values [5]. In Hungary the ECCS calculations were used until recently, where height and age are major determinants of LF reference equations, and corrections are necessary for height in special patient populations [3]. Several data supported the need for more appropriate reference values for spirometry; in 2012, Quanjer et al published new spirometric prediction equations that include appropriate age-dependent lower limits of normal (LLN), which can be applied to both sexes and different ethnic groups (Global Lung Function Initiative 2012-GLI) [6]

Methods

Results

Discussion

Conclusion