A Retrospective Review of Tofacitinib in the Treatment of Refractory Dermatomyositis.

Dermatomyositis (DM) is an autoimmune myopathy with characteristic dermatologic features.1 Tofacitinib is an immunomodulator with proven efficacy against numerous immune-mediated disorders, including rheumatoid arthritis (RA) and psoriasis.2 Several reports have demonstrated oral tofacitinib’s ability to treat the cutaneous and extracutaneous manifestations of refractory dermatomyositis (DM).1,4,5 However, evidence for sustained improvement remains limited.2,3 The goal of this study is to investigate the long-term response of recalcitrant DM to oral and topical tofacitinib at varied dosing regimens.

Filling in the gaps on FILS syndrome: A case report and literature review.

FILS syndrome (facial dysmorphism, immunodeficiency, livedo, and short stature) is a rare autosomal recessive disorder caused by pathogenic alterations in the POLE gene leading to multisystemic manifestations, including poorly characterized skin findings. We report a child with a homozygous variant, c.100C > T (p.Arg34Cys), in POLE and features consistent with poikiloderma, expanding the dermatologic signs associated with this rare disorder. Additionally, we review reported cases of FILS syndrome, discuss possible pathomechanisms for our patient's presentation, and consider implications for management.

High-pitch low-voltage CT coronary artery calcium scoring with tin filtration: accuracy and radiation dose reduction.

OBJECTIVES: To investigate diagnostic accuracy and radiation dose of high-pitch CT coronary artery calcium scoring (CACS) with tin filtration (Sn100kVp) versus standard 120kVp high-pitch acquisition. METHODS: 78 patients (58% male, 61.5±9.1 years) were prospectively enrolled. Subjects underwent clinical 120kVp high-pitch CACS using third-generation dual-source CT followed by additional high-pitch Sn100kVp acquisition. Agatston scores, calcium volume scores, Agatston score categories, percentile-based risk categorization and radiation metrics were compared. RESULTS: 61/78 patients showed coronary calcifications. Median Agatston scores were 34.9 [0.7-197.1] and 41.7 [0.7-207.2] and calcium volume scores were 34.1 [0.7-218.0] for Sn100kVp and 35.7 [1.1-221.0] for 120kVp acquisitions, respectively (both p<0.0001). Bland-Altman analysis revealed underestimated Agatston scores and calcium volume scores with Sn100kVp versus 120kVp acquisitions (mean difference: 16.4 and 11.5). However, Agatston score categories and percentile-based risk categories showed excellent agreement (ĸ=0.98 and ĸ=0.99). Image noise was 25.8±4.4HU and 16.6±2.9HU in Sn100kVp and 120kVp scans, respectively (p<0.0001). Dose-length-product was 9.9±4.8mGy*cm and 40.9±14.4mGy*cm with Sn100kVp and 120kVp scans, respectively (p<0.0001). This resulted in significant effective radiation dose reduction (0.13±0.07mSv vs. 0.57±0.2mSv, p<0.0001) for Sn100kVp acquisitions. CONCLUSION: CACS using high-pitch low-voltage tin-filtered acquisitions demonstrates excellent agreement in Agatston score and percentile-based cardiac risk categorization with standard 120kVp high-pitch acquisitions. Furthermore, radiation dose was significantly reduced by 78% while maintaining accurate risk prediction. KEY POINTS: • Coronary artery calcium scoring with tin filtration reduces radiation dose by 78%. • There is excellent correlation between high-pitch Sn100kVp and standard 120kVp acquisitions. • Excellent agreement regarding Agatston score categories and percentile-based risk categorization was achieved. • No cardiac risk reclassifications were observed using Sn100kVp coronary artery calcium scoring.