Browsing by Author "Chung, Lorinda"
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- ItemA Pilot Study to Evaluate the Safety and Efficacy of Treprostinil in the Treatment of Calcinosis in Patients with Systemic Sclerosis(2020) Chung, Melody P.; Valenzuela Vergara, Antonia María; Li, Shufeng; Catanese, Benjamin; Stevens, Kate; Fiorentin, David; Strand, Vibeke; Chung, Lorinda
- ItemCalcinosis is associated with ischemic manifestations and increased disability in patients with systemic sclerosis(2020) Valenzuela, Antonia; Baron, Murray; Rodriguez-Reyna, Tatiana S.; Proudman, Susanna; Khanna, Dinesh; Young, Amber; Hinchcliff, Monique; Steen, Virginia; Gordon, Jessica; Hsu, Vivien; Castelino, Flavia, V; Schoenfeld, Sara; Li, Shufeng; Wu, Joy Y.; Fiorentino, David; Chung, LorindaObjective: Calcinosis is a debilitating complication of systemic sclerosis (55c) with no effective treatments. We sought to identify clinical correlations and to characterize complications and disability associated with calcinosis in a multi-center, international cohort of 55c patients.
- ItemChange in calcinosis over 1 year using the scleroderma clinical trials consortium radiologic scoring system for calcinosis of the hands in patients with systemic sclerosis(2022) Valenzuela, Antonia; Stevens, Kathryn; Chung, Melody P.; Rodriguez-Reyna, Tatiana S.; Proudman, Susanna; Baron, Murray; Castelino, Flavia, V; Hsu, Vivien; Green, Lorraine; Del Galdo, Francesco; Li, Shufeng; Fiorentino, David; Chung, LorindaIntroduction: Calcinosis cutis is a debilitating complication of systemic sclerosis (SSc). We previously developed a radiographic scoring system to assess severity of calcinosis affecting the hands in patients with SSc. We sought to further validate our radiographic scoring system to assess for change over 1 year and to identify factors associated with improvement or progression.
- ItemGastrointestinal Perforation in a Patient With Antinuclear Matrix Protein 2 Antibody-Positive Dermatomyositis(2022) Valenzuela Vergara, Antonia María; Rieger, Kerri E.; Blish, Catherine A.; Chung, Lorinda; Fiorentino, David
- ItemMoving forward together: collaborative landscapes of research in idiopathic inflammatory myopathies and calcinosis(Oxford Univ. Press, 2023) Saketkoo, Lesley Ann; Valenzuela Vergara, Antonia Maria; Kim, Susan; McCann, Liza J.; Lood, Christian; Wahezi, Dawn M.; Werth, Victoria P.; Yi, Belina; Alexanderson, Helene; Maillard, Susan; Pilkington, Clarissa; Fligelstone, Kim; Limbach, Barbara; Orandi, Amir B.; Regardt, Malin; Russell, Anne-Marie; Davuluri, Srijana; deGroot, Ingrid; Ernste, Floranne; Paik, Julie J.; von Muhlen, Carlos A.; Dimachkie, Mazen M.; Machado, Pedro M.; Naddaf, Elie; Shafranski, Barbara M.; Gupta, Latika; Zulian, Francesco; Chung, Lorinda; International Myositis Assessment and Clinical Studies Group and The Myositis International Research and Health Collaborative Alliance (IMACS/ MIHRA) Calcinosis Scientific Interest Group
- ItemOsteoclastogenesis in Patients With Systemic Sclerosis With and Without Calcinosis Cutis(Wiley, 2025) Valenzuela Vergara, Antonia María; Pérez, Guillermo; Chung, Lorinda; Sánchez, Felipe; Iturriaga Ortiz, Carolina Alejandra; Montalva, Rebeca; Borzutzky Schachter, ArturoObjective. We aimed to assess whether the presence of radiographically confirmed calcinosis of the hands in patients with systemic sclerosis (SSc) is associated with increased osteoclastogenesis. Methods. We recruited 20 patients with SSc (10 with calcinosis and 10 without calcinosis) and 10 age- and gender-matched healthy controls. Hand radiographs were scored using the validated Scleroderma Clinical Trials Consortium (SCTC) radiographic severity score for calcinosis. To evaluate osteoclastogenesis, peripheral blood mononuclear cells (PBMCs) were cultured with RANKL and macrophage colony-stimulating factor; osteoclasts were identified using tartrate-resistant acid phosphatase staining. Measures of bone resorption (RANKL, osteoprotegerin [OPG]) and ischemia or endothelial function (vascular endothelial growth factor, angiopoietin-1, and angiopoietin-2 [Ang-2]) were also evaluated. Results. Patients with SSc were all women and Hispanic, and the majority (n = 12, 60%) had limited SSc skin type. Mean +/- SD age was 55.2 +/- 14.8 years; mean +/- SD disease duration was 9.5 +/- 6.5 years from first non-Raynaud phenomenon symptom. Patients with SSc with calcinosis had more digital ischemia than patients without calcinosis. Median SCTC score in patients with SSc with calcinosis was 11.1 (range 0.7-286). After 9 days in culture, PBMCs from patients with calcinosis originated a significantly higher number of osteoclasts (33.0 +/- 20.3 cells/well) than patients without calcinosis (15.3 +/- 6.9 cells/well) and healthy individuals (11.2 +/- 2.6 cells/well) (P = 0.001). The severity of calcinosis was not correlated with the number of osteoclasts per well (r = 0.27, P = 0.5); however, it was correlated with RANKL (r = 0.82, P = 0.004), RANKL/OPG ratio (r = 0.86, P = 0.002), and Ang-2 levels (r = 0.86, P = 0.002). Conclusion. Calcinosis in patients with SSc is associated with an increased propensity of peripheral blood cells to form osteoclasts. Targeted inhibition of osteoclastogenesis may provide a specific therapeutic option for patients with SSc-associated calcinosis.
- ItemPatient Experience of Systemic Sclerosis-Related Calcinosis An International Study Informing Clinical Trials, Practice, and the Development of the Mawdsley Calcinosis Questionnaire(2023) Saketkoo, Lesley Ann; Gordon, Jessica K.; Fligelstone, Kim; Mawdsley, Anne; Chaudhry, Humza A.; Valenzuela, Antonia; Christensen, Angela; Khalique, Samara M.; Jensen, Kelly; Weinmann, Sophia C.; Busman, Evan; Chung, Lorinda; Hsu, Vivien M.; Russell, Anne-Marie; Steen, Virginia D.The patient experience of SSc-calcinosis was explored iteratively in a diverse and repre-sentative international cohort, reaching concept saturation. Pervasive disability, frustra-tion, symptom distress, and uncertainty created largely by complex, severe pain sensations are related to daily living with SSc-calcinosis. Patient observations and self-management behavior provide opportunities to educate clinicians and patients. Pa-tients are eager for self-management guidance. This investigation informed the develop-ment of a novel PROM, the MCQ, developed according to FDA guidance20,21 to assess the severity and impact of SSc-calcinosis for use in clinical studies and clinical practice. Further validation and translation studies of the MCQ are underway.
- ItemSubcutaneous calcinosis: Is it different between systemic sclerosis and dermatomyositis?(2021) Valenzuela Vergara, Antonia María; Chung, LorindaCalcinosis cutis is the deposition of insoluble calcium in the skin and subcutaneous tissues. It is a manifestation of several autoimmune connective tissue diseases, most frequently with systemic sclerosis and juvenile dermatomyositis, followed by adult dermatomyositis. Autoimmune connective tissue disease-associated calcinosis is of the dystrophic subtype, which occurs at sites of damaged tissue in the setting of normal serum calcium and phosphate levels. In juvenile dermatomyositis, calcinosis is considered a marker of ongoing disease activity and possibly inadequate treatment, while in adult dermatomyositis, it is a hallmark of skin damage due to chronic rather than active disease. Calcinosis is associated with long disease duration in systemic sclerosis and dermatomyositis, anti-polymyositis/sclerosis autoantibodies in systemic sclerosis and NXP-2 and melanoma differentiation-associated gene 5 in dermatomyositis. Calcinosis in systemic sclerosis occurs most frequently in the hands, particularly the fingers, whereas in dermatomyositis, it affects mainly the trunk and extremities. The primary mineral component of calcinosis is hydroxyapatite in systemic sclerosis and carbonate apatite in dermatomyositis. Calcinosis in dermatomyositis and systemic sclerosis share some pathogenic mechanisms, but vascular hypoxia seems to play a more important role in systemic sclerosis, whereas the release of calcium from mitochondria in muscle cells damaged by myopathy may be a primary mechanism contributing to dermatomyositis-related calcinosis. Multiple treatment strategies for dermatomyositis and systemic sclerosis-related calcinosis have been used with variable results. Early aggressive treatment of underlying myositis in patients with dermatomyositis may improve long-term outcomes of calcinosis. A better understanding of the pathogenesis of calcinosis is needed to improve treatment options.