Pulmonary Hypertension Screening in Scleroderma

Scleroderma is a heterogenous connective tissue disorder characterized by fibrosis of the skin, with or without internal organ involvement. The aetiology of scleroderma may involve both environmental and genetic factors. Abnormalities involving the immune system, vascular tissue and extracellular matrix have been demonstrated.

One of the major cause of mortality in patients with scleroderma is pulmonary arterial hypertension (PAH). International recommendations advise annual screening for the early detection of PAH in asymptomatic patients with scleroderma.

scleroderma is group 1 in clinical classification of pulmonary hypertension Pulmonary hypertension (PH) is a haemodynamic condition characterised by elevation of mean pulmonary arterial pressure (mPAP) \>20 mmHg, assessed by right heart catheterisation. Pulmonary arterial wedge pressure (PAWP) and pulmonary vascular resistance (PVR) distinguish pre-capillary PH (PAWP ≤15 mmHg, PVR \>2 Wood Units (WU)), isolated post-capillary PH (PAWP \>15 mmHg, PVR ≤2 WU) and combined post- and pre-capillary PH (PAWP \>15 mmHg, PVR \>2 WU) Scleroderma has an annual prevalence of one to five cases for every 1000 individuals and nearly 15 percent of all cases develop PAH. A diagnosis of PAH in Scleroderma is virtually a death sentence, with studies reporting a mortality rate of 50 per cent in the 3 years of diagnosis. Therefore, developing and implementing screening and diagnosis protocol is important in the fight against this disease. echocardiography as the leading screening tool for scleroderma -PAH. In particular, systolic pulmonary arterial pressure (sPAP) and tricuspid regurgitation velocity (TRV). Echocardiography is non invasive tool for eary screening in pulmonary hypertension according ESC-ERS guideline If the peak tricuspid regurgitation velocity (TRV) is ≤ 2.8 m/s or not measurable, and there are no other echocardiographic signs of pulmonary hypertension, the probability is low.

If the TRV is ≤ 2.8 m/s or not measurable, but other echocardiographic signs of pulmonary hypertension are present, the probability is intermediate low

If the TRV is between 2.9 and 3.4 m/s and no other signs are present, the probability is intermediate high

If the TRV is between 2.9 and 3.4 m/s and other signs are present, the probability is high.

PH probability LOW → other causes . PH probability ntermediate low → follow up Echocardiography PH probability INTERMEDIATE high or HIGH → right heart catheterization Additional echocardiographic signs suggestive of pulmonary hypertension

A: The ventricles

RV/LV basal diameter area ratio ≥1.0

Flattening of the interventricular septum (LVEI \>1.1 in systole and/or diastole)

TAPSE / sPAP ratio \<0.55 mm/mmHg

B: Pulmonary artery

RVOT AT \<105 ms and/or mid-systolic notching

Early diastolic pulmonary regurgitation velocity \>2.2 m/s

PA diameter \>25 mm

RPA diameter \>25 mm

C: Inferior vena cava and RA

IVC diameter \>21 mm with decreased inspiratory collapse (\<50% with a sniff or \<20% with quiet inspiration)

RA area (end-systole) \>18 cm²