Exercise as it relates to Disease, Changes in Pulmonary Exercise Haemodynamics in Scleroderma

This is a critical analysis of the article "Changes in pulmonary exercise haemodynamics in scleroderma: a 4-year prospective study" by Gabor Kovacs, Alexander Avian, Nora Wutte, Franz Hafner, Florentine Moazedi-Fürst, Sonja Kielhauser, Elisabeth Aberer, Marianne Brodmann, Winfried Graninger, Vasile Foris, Andrea Olschewski and Horst Olschewski.

Background

Scleroderma is an autoimmune disease which causes a thickening and tightening of a variety of soft tissues, there are different classifications based on how the body is affected 1]. These classifications are broadly broken into limited and systemic scleroderma [1]. Limited scleroderma mainly effects the skin of the extremities, causing limited movement and poor circulation [1]. Systemic scleroderma (SSc) additionally affects the underlying soft tissues, including main organs such as the heart, lungs, and gastrointestinal tract [1]. Because of the nature of SSc, it causes serious complications which are often not diagnosed until after death [2]. Most SSc patients have a survival rate of up to 5 years from diagnosis; 14% of all deaths are caused by [pulmonary arterial hypertension (PAH)](https://www.webmd.com/lung/pulmonary-arterial-hypertension#:~:text=Having%20pulmonary%20arterial%20hypertension%20(PAH,lungs%20become%20narrow%20or%20blocked.), with 87% of all deaths attributed to scleroderma caused by heart complications [2] [3] [4]. Until recently there have been limited screening processes to detect possible PAH in SSc patients [2]. However, techniques with increased sensitivity have been developed with the potential to detect abnormalities in cardiac function which can cause serious health issues, even death [2]. Early diagnosis of cardiac involvement in SSc patients is important as it can lead to better therapy responses and may prevent life threatening complications for many years [2]. The article "[Changes in pulmonary exercise haemodynamics in scleroderma: a 4-year prospective study" is an important step in research as it looks at using exercise and non-invasive ways to screen for PAH [3].

Where is this research from?

"Changes in pulmonary exercise haemodynamics in scleroderma: a 4-year prospective study" was conducted by Gabor Kovacs et al. and included in the European Respiratory Journal (ERJ), which was published online on the 13th of July 2017 [3]. The ERJ is the lead scientific journal of the European Respiratory Society (ERS) and publishes a wide array of reliable up to date, high quality peer reviewed articles and research [5].

The authors of this study have a wide variety of expertise in numerous disciplines such as pulmonology, statistics, angiology, rheumatology, and physiology [3]. They are affiliated the Medical University of Graz, Graz, Austria and the Ludwig Boltzmann Institute for Lung Vascular Research, Graz, Austria [3].

What kind of research was this?

The research is a 4-year prospective cohort study which is longitudinal in design, which provides information about the relationship between exposure and outcome 3] [6]. Prospective cohort studies have several advantages over other types of observational studies and are considered the gold standard within this area of study [6]. When considering other forms of research designs such as [meta-analysis and systematic reviews, prospective cohort studies sit in the middle [6].

What did the research involve?

The research involved studying a sample of 99 individuals with systemic scleroderma to look for pulmonary arterial hypertension (PAH) 3]. Prior to commencing the study, written consent was obtained and the study was conducted in line with the [Helsinki Declaration 3].These individuals had previously been screened and participated if they were determined not to have any pre-existing diagnosis of any heart conditions [3]. Baseline rest and exercise data that looked at systolic pulmonary arterial pressure (sPAP), peak oxygen uptake, mean pulmonary arterial pressure, and heart rate was collected using [exercise echocardiography, cardiopulmonary exercise testing and in a subgroup right heart catheter (RHC) insertion [3]. The patients from this study where then followed up 4 years later on average, at this point data was collected again [3]. To collect the exercise induced responses all patients were asked to perform a symptom-limited cardiopulmonary exercise test on a variable-load, half-recumbent, left sloping echocardiography cycle ergometer using a standard protocol with a 25 watt increment increase every 2 minutes [3].

What were the basic results?

The results showed that scleroderma patients without previously diagnosed cardiac diseases developed a mild but significant deterioration of pulmonary exercise haemodynamics and exercise capacity over a 4-year period indicating a progression of pulmonary vascular disease [3]. Three out of the 99 patients developed RHC-confirmed PAH, however 41 patients did not complete the study [3]. Exercise testing results showed that systemic pulmonary arterial pressure at 50 watts (sPAP50) increased, mean pulmonary vascular resistance increased significantly as well, indicating a possible stiffening of the pulmonary arteries which have been shown to be a predictor of PAH and is associated with decreased exercise capacity and poorer outcomes [3]. There were significant decreases in peak oxygen uptake demonstrate a decrease in exercise capacity [3]. At rest there was no significant change in resting systemic pulmonary arterial pressure or mean pulmonary vascular resistance, indicating that exercise can induce symptoms of pulmonary arterial pressure and a change in pulmonary haemodynamics earlier than at rest [3].

What conclusions can we take from this research?

The study found that exercise induced pulmonary haemodynamics can be important for recognising pulmonary vascular disease in SSc [3]. Currently RHC insertions represent the most reliable method to assess pulmonary haemodynamics, however this procedure is invasive and cannot be performed in a healthy population for ethical reasons [3]. This study suggests that echocardiography, could be a reliable method to assess pulmonary haemodynamics in scleroderma patients [3]. It cannot be assumed that echocardiography is a reliable and valid tool in determining rest and exercise pulmonary haemodynamics outside of this study [3]. Additional research is required to attempt minimise some of the limitations of the study such as using a small sample size, loss to follow up, heavy presence of selection bias and enriching of the sample with healthier patients, the lack of a healthy control group and possible interference from medicines prescribed outside of the study [3].

Practical advice

Using echocardiography as a screening tool can pick up on exercise pulmonary haemodynamics, and pulmonary arterial pressure which can indicate possible pulmonary arterial hypertension and the need to perform an RHC insertion [3]. Further research could indicate the ability of using echocardiography as a diagnostic tool [3]. New research should look at considering a broader population of SSc patients, the use of immunosuppressive medications and a healthy control group would benefit research on the use of non-invasive exercise induced PAH testing measures on SSc patients [3].

Further information and resources

For further support and information on scleroderma please look at the following links:

Organisations and helpful information:

• https://sclerodermainfo.org/guide/
• https://www.scleroderma.org/site/SPageNavigator/patients_whatis.html
• https://www.hopkinsscleroderma.org/scleroderma/frequently-asked-questions/
• https://www.sclerodermaaustralia.com.au/

General information on exercising with scleroderma:

• https://www.hospitalhealth.com.au/content/aged-allied-health/article/is-it-safe-to-exercise-with-scleroderma--850099346#axzz6YAhsZ4lD
• https://www.selfmanagescleroderma.com/lessons/exercise-guidelines-for-general-fitness.html
• https://www.scleroderma.org/site/DocServer/Form_16c_low_res.pdf?docID=19809&AddInterest=1281

References

• ↑ a b c d CALLEJAS-RUBIO JL, MORENO-ESCOBAR E, de la FUENTE PM, PÉREZ LL, FERNÁNDEZ RR, SÁNCHEZ-CANO D, et al. Prevalence of Exercise Pulmonary Arterial Hypertension in Scleroderma. The Journal of Rheumatology. 2008;35(9):1812-6.
• ↑ a b c d e Krieg T, Meurer M. Systemic scleroderma: clinical and pathophysiologic aspects. Journal of the American Academy of Dermatology. 1988;18(3):457-81.
• ↑ a b c d e f g h i j k l m n o p q r s t u v w x y Kovacs G, Avian A, Wutte N, Hafner F, Moazedi-Fürst F, Kielhauser S, et al. Changes in pulmonary exercise haemodynamics in scleroderma: a 4-year prospective study. European Respiratory Journal. 2017;50(1):1601708.
• ↑ Tyndall AJ, Bannert B, Vonk M, Airò P, Cozzi F, Carreira PE, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Annals of the Rheumatic Diseases. 2010;69(10):1809-15.
• ↑ Simonds AK, Reeves EL. ERJ Open Research: FAQs. European Respiratory Journal. 2015;46(2):318-20.
• ↑ a b c Hammoudeh S, Gadelhaq W, Janahi I. Prospective cohort studies in medical research. Cohort studies in health sciences Chile: IntechOpen. 2018:11-28.

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