Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disorder that is characterized by a interplay of vascular abnormalities, immune system activation and an uncontrolled fibrotic response associated with interstitial lung disease affecting about 40% of patients. Identification of ILD relies on high-resolution CT that identify features suggestive of the histologic patterns of SSc(1). CT is used to determine pattern and extent of ILD and participates in the prediction of ILD progression(2). All group of pulmonary hypertension (PH) may occur with an overall prevalence reported in up to one fifth of patient. Whereas extensive SSc-ILD can be responsible for PH, PH can also be seen as a consequence of myocardial abnormalities or as primarily affecting small pulmonary arteries and classified as pulmonary arterial hypertension. Dual-energy CT introduction offers perspectives in the evaluation of SSc-related pulmonary manifestations. While these are not strictly perfusion images, they have been reported as adequate surrogate markers of lung perfusion (3). In the field of PH, detection of perfusion defects highly concordant with V/Q scintigraphic findings has been reported in the diagnostic approach of CTEPH but also in the differential diagnosis between PAH and peripheral forms of CTEPH (4). Our objective is to investigate lung perfusion abnormalities in patients with SSc

Methods: The study population included 101 patients who underwent dual-energy CT (DECT) angiography in the follow-up of SSc. CT examinations were obtained on a 3rd-generation dual-source CT system with reconstruction of morphologic and perfusion images. All patients underwent pulmonary function tests within two months of the follow-up CT scan. Fifteen patients had right heart catheterization-proven PH.

Results: Our population included patients without SSc lung involvement (Group 1; n=37), patients with SSc-related ILD (Group 2; n=56) of variable extent (Group 2a: ≤10%: n=17; Group 2b: between 11-50%: n=31; Group 2c: >50%: n=8) and patients with PVOD/PCH (Group 3; n=8). Lung perfusion was abnormal in 8 patients in G 1 (21.6%), 14 patients in G 2 (25%) and 7 patients in G 3 (87.5%). Perfusion changes were mainly composed of bilateral perfusion defects, including patchy, PE-type perfusion defects and areas of hypoperfusion of variable size. In G 1 and G 2a (n=54): (a) patients with abnormal lung perfusion (n=14) had a significantly higher proportion of NYHA III/IV scores of dyspnea (p=0.031), a shorter mean walking distance at the 6MWT (p=0.042) and a trend towards lower mean DLCO% (p=0.055) when compared to patients with normal lung perfusion (n=40); (b) a negative albeit weak correlation was found between the iodine concentration in both lungs and the DLCO% (r=- 0.27; p=0.059) whereas no correlation was found with PAPs (r=0.16; p=0.29) and walking distance during the 6MWT (r=-0.029; p=0.84).

Conclusion: DECT lung perfusion provides complementary information to HRCT scans, depicting perfusion changes in SSc patients with normal or minimally infiltrated lung parenchyma.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/can-dual-energy-ct-lung-perfusion-detect-abnormalities-at-the-level-of-lung-circulation-in-systemic-sclerosis-ssc-preliminary-experience-in-101-patients/