Background/Purpose:

Pulmonary arterial hypertension (PAH) is associated with high morbidity and mortality in Systemic Sclerosis. Early diagnosis and treatment leads to substantial improvements in quality of life, prognosis and mortality. Right heart catheterization remains the only test that can diagnose PAH and differentiate it from pulmonary venous hypertension or diastolic dysfunction. Controversies exist in unmasking PAH with exercise or fluid challenge during right heart catheterization. However, there are no studies so far to identify them as one of the means of early diagnosis.

Methods:

Our study includes 173 Systemic sclerosis patients enrolled in the Tasmanian Systemic Sclerosis Epidemiology (TASSIE) study , recruited from rheumatologists, physicians, cardiologists, general practitioners and other health professionals across Tasmania since 2007. Echocardiogram and pulmonary function tests were routinely performed on each patient at screening and at annual follow up. Right heart catheter was performed when clinically indicated. A proportion of symptomatic patients underwent right heart catheterization with fluid challenge based on clinical suspicion of PAH or diastolic dysfunction. Treatment for PAH was initiated in all patients who qualified under current regulations. Treatment effects on their six minute walk test (6MWT) and quality of life were studied at the end of two years.

Results: Seventy-eight of the cohort (45.1%) were diagnosed with PAH. Thirty-seven (21.4%) were diagnosed by standard RHC and 41 (23.7%) had latent PAH unmasked by the fluid challenge on RHC. Patients with PAH showed female predominance, and those who had diagnosis on fluid challenge were younger than those who were diagnosed without fluid challenge (57.5–vs-59.8, p < 0.05). More patients with limited SSc than diffuse disease had PAH (51-vs-27). Improvements in 6MWT were observed in both groups at 6 months and 12 months after treatment commencement for PAH (Table).

	PAH on RHC	PAH on fluid challenge	Any form of PAH
Total	37 (21.4%)	41 (23.7%)	78 (45.1%)
Sex (M:F)	8:29	4:37	12:66
Age (Mean yrs)	59.8	57.5	59
Diffuse	16	11	27
Limited	21	30	51
Mean % improvement from baseline in 6MWT  at 6 months	14.34%	11.33%	13.33%
Mean % improvement from baseline in 6MWT  at 12 months	12.96%	15.84%	14.4%

Conclusion: Fluid challenge is a sensitive measure of early diagnosis of pulmonary arterial hypertension secondary to systemic sclerosis. Early diagnosis has led to improved treatment outcome in our small cohort.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/unmasking-latent-pulmonary-arterial-hypertension-by-fluid-challenge-in-patients-with-systemic-sclerosis/