Systemic sclerosis (also known as scleroderma) is a chronic fibrosing autoimmune disease with both skin and multisystem organ involvement. Scleroderma has the highest mortality among all rheumatic diseases. The pathophysiology mechanism of systemic sclerosis is a progressive self-amplifying process, which involves widespread microvascular damage, followed by a dysregulation of innate and adaptive immunity and inflammation and diffuse fibrosis of the skin and visceral organs. Fibrosis of internal organs is a hint for systemic sclerosis, moreover associated with interstitial lung disease (SSc-ILD) is a complex process. In order to correlate scientific data from the literature with clinical experience, we present the case of a 56-year-old woman who was diagnosed with systemic sclerosis 16 years ago. The association of numerous comorbidities characterized by a considerable level of seriousness characterizes this case: the highly extensive systemic damage, the cardiovascular impact of the illness, and the existence of severe pulmonary arterial hypertension. The systemic and clinical manifestations, respiratory functional tests, radiological features, and specific therapy are discussed.

Background/Objectives: Quantitative patent ductus arteriosus (PDA) geometry in congenital heart disease (CHD), particularly in relation to aortic arch abnormalities, is incompletely characterized. We evaluated CT-derived PDA geometry. Methods: This retrospective exploratory single-center study included 168 infants with CHD and a PDA on cardiovascular CT. Aortic-end, pulmonary-end, and mid-duct diameters and ductal length were measured. The Ductal Length-to-Diameter Index (LDI), selected during exploratory analysis, was defined as ductal length divided by the mean end diameter. Seventy-three patients had CT-documented aortic arch obstruction or hypoplasia. Results: LDI was lower in patients with arch obstruction/hypoplasia than in those without these findings (2.04 [1.39–2.84] vs. 3.51 [2.23–4.89]; p < 0.001; rank-biserial r = −0.473) and remained associated after adjustment for age, weight, and sex (OR per +1 LDI, 0.534; 95% CI, 0.412–0.693; p < 0.001). The association remained robust with a conservative definition restricted to coarctation or interrupted aortic arch. LDI had an interobserver ICC of 0.843 (95% CI, 0.685–0.954), with wide limits of agreement (−1.98 to +2.45). Conclusions: CT-documented aortic arch obstruction or hypoplasia is associated with lower LDI. This exploratory geometric association warrants prospective external validation before clinical use.

Systemic sclerosis (also known as scleroderma) is a chronic fibrosing autoimmune disease with both skin and multisystem organ involvement. Scleroderma has the highest mortality among all rheumatic diseases. The pathophysiology mechanism of systemic sclerosis is a progressive self-amplifying process, which implicates the widespread microvascular damage, followed by a dysregulation of innate and adaptive immunity and inflammation, and diffuse fibrosis of the skin and visceral organs. Fibrosis of internal organs is a hint for systemic sclerosis, moreover associated with interstitial lung disease (SSc-ILD) is a complex process. We report a case of a 56 years old female, diagnosed with Systemic Sclerosis 16 years ago. The systemic and clinical manifestations, respiratory functional tests, radiological aspects and specific therapy were discussed.

Background/Objectives: Ventricular measurements from cardiac computed tomography (CCT) and cardiovascular magnetic resonance (CMR) may differ due to modality-specific, physiological, or observer-dependent reasons. We compared cross-modality disagreement with intra-modality interobserver variability. Methods: Fifty-four patients with CCT and CMR examinations within 36 months were retrospectively analyzed. Left ventricular (LV) end-diastolic volume (EDV), right ventricular (RV) EDV, and LV mass were measured on the same platform; 18 underwent independent second-observer measurements. The primary metric was the standard deviation of paired differences [SD(D)], with bootstrap 95% confidence intervals (CIs). Results: In the repeat-measurement subset, cross-modality SDs were 29.78 mL, 29.19 mL, and 16.72 g for LV EDV, RV EDV, and LV mass, versus 4.83, 7.33, and 6.71 within CCT and 3.57, 3.58, and 4.11 within CMR. Cross-modality variability was 6.16, 3.98, and 2.49 times the CCT and 8.34, 8.15, and 4.07 times the CMR interobserver variability; all 95% CIs exceeded 1. Examination-interval restriction did not materially reduce dispersion, and body-surface-area indexation changed scale but not relative disagreement. Conclusions: Under routine clinical conditions, cross-modality variability in EDV and LV mass exceeded interobserver variability by 2.5- to 8.3-fold. CCT-CMR differences are unlikely to reflect observer-related post-processing variability alone, and consistent modality selection appears preferable to interchanging quantitative thresholds.