Systemic sclerosis is a rare and chronic connective tissue disease resulting from an intricate pathogenesis and is expressed in very heterogeneous clinical manifestations. Every year many studies try to unravel and shed new insights into the pathogenesis, organ involvement and treatment of this complex and severe disease. We herein provide an overview of the most relevant studies published in the literature in 2022.
Systemic sclerosis (SSc) is an autoimmune disease characterised by microvasculopathy, immune dysregulation, and skin and visceral organ fibrosis. Every year novel insights into the pathogenesis, organ involvement and treatment of this severe disease are published in the scientific community. In this review we report an overview of some of the most relevant contributions published in 2021.
Objectives
To evaluate the performance of oropharyngoesophageal scintigraphy (OPES) in the assessment of dysphagia in patients with systemic sclerosis (SSc), and to compare OPES results with those of barium esophagogram.
Methods
Adult SSc patients who underwent OPES for the assessment of dysphagia were enrolled. OPES was performed with both liquid and semisolid boluses and provided information regarding oropharyngeal transit time, esophageal transit time (ETT), oropharyngeal retention index (OPRI), esophageal retention index (ERI), and site of bolus retention. Barium esophagogram results were also collected.
Results
Fifty-seven SSc patients (87.7% female, mean age 57.7 years) with dysphagia were enrolled. OPES identified at least one alteration in each patient and findings were generally worse for the semisolid bolus. Esophageal motility was widely impaired with 89.5% of patients with an increased semisolid ERI, and middle-lower esophagus was the most frequent site of bolus retention. However, oropharyngeal impairment was highlighted by widespread increased OPRI, especially in anti-topoisomerase I positivity. Older patients and with longer disease duration presented slower semisolid ETT (p = 0.029 and p = 0.002, respectively). Eleven patients with dysphagia had a negative barium esophagogram: all of them presented some alterations in OPES parameters.
Conclusion
OPES revealed a marked SSc esophageal impairment, in terms of both slowed transit time and increased bolus retention, but also shed light on oropharyngeal swallowing alterations. OPES showed high sensitivity, being able to detect swallowing alterations in dysphagic patients with negative barium esophagogram. Therefore, the use of OPES for the assessment of SSc-related dysphagia in clinical practice should be promoted.
ObjectiveSystemic sclerosis (SSc) is burdened by Raynaud's phenomenon (RP) and digital ulcers (DUs) and sometimes standard vasoactive therapies are ineffective or contraindicated. Selexipag is an oral selective IP prostacyclin receptor agonist approved for the treatment of SSc-related pulmonary arterial hypertension. We aimed to evaluate clinical and instrumental efficacy of selexipag in SSc digital vasculopathy.MethodsSSc patients with severe digital vasculopathy refractory or with contraindication to all other vasoactive therapies were administered selexipag, evaluating at baseline and after 3 months clinical outcomes regarding RP and DUs and digital perfusion assessed by laser speckle contrast analysis (LASCA).ResultsSelexipag was administered to 9 SSc patients (66.6% female, mean age 52.3 ±16.6 years). One of them had to stop the drug for adverse effects. After three months selexipag determined a significant reduction of RP daily episodes (p=0.012) and RP mean duration (p=0.044). DUs decreased from 10 to 4 without reaching the statistical significance. A significant improvement in the mean perfusion of the fingers (p=0.016) was observed at LASCA.ConclusionSelexipag showed good potential for the treatment of SSc-digital vasculopathy. Our results are certainly preliminary but yet quite encouraging. New trials for the evaluation of selexipag efficacy in SSc-digital vasculopathy are needed.
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