Article
Immunology
D. A. Haverkort, B. E. Kersten, A. van Rhenen, W. J. F. M. van der Velden, M. C. Vonk
Summary: This article presents a case of a patient with progressive systemic sclerosis (SSc) who underwent a second hematopoietic cell transplantation (HCT) five years after the first treatment. The case observed improvements in skin and pulmonary involvement, and evaluated the changes using sequential nailfold microcapillaroscopy (NCM). This case provides limited evidence on the efficacy and safety of a second HCT in refractory SSc cases, as well as highlighting the potential of HCT in improving microvasculopathy in SSc.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Immunology
Patrick-Pascal Strunz, Matthias Froehlich, Michael Gernert, Eva Christina Schwaneck, Anna Fleischer, Ann-Christin Pecher, Hans-Peter Tony, Joerg Christoph Henes, Marc Schmalzing
Summary: Autologous hematopoietic stem cell transplantation is effective for systemic sclerosis treatment, but may lead to immunological adverse events. This study found that iAEs are common, with engraftment syndrome and secondary autoimmune disorder being major complications.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Rheumatology
Satoshi Ebata, Koji Oba, Kosuke Kashiwabara, Keiko Ueda, Yukari Uemura, Takeyuki Watadani, Takemichi Fukasawa, Shunsuke Miura, Asako Yoshizaki-Ogawa, Asano Yoshihide, Ayumi Yoshizaki, Shinichi Sato
Summary: A post-hoc analysis of data from a clinical trial investigating the effectiveness of rituximab in patients with systemic sclerosis identified CD19-positive cell counts, modified Rodnan skin score (mRSS), and serum surfactant protein D (SP-D) levels as factors influencing the response to treatment. Patients with high CD19-positive cell counts and high mRSS showed the greatest improvement in mRSS with rituximab.
Review
Rheumatology
Monika Dzwigala, Piotr Sobolewski, Maria Maslinska, Ida Yurtsever, Elzbieta Szymanska, Irena Walecka
Summary: Ultrasound is used in skin assessment of patients with systemic sclerosis to help with clinical evaluation of skin involvement, and it should be compared to skin biopsy. Currently, there is a lack of guidance for conducting skin measurements using ultrasound in patients, and the clinical use of skin imaging in systemic sclerosis is limited due to the lack of standard operating procedures.
RHEUMATOLOGY INTERNATIONAL
(2021)
Article
Rheumatology
Ivan Ferraz-Amaro, Esmeralda Delgado-Frias, Vanesa Hernandez-Hernandez, Hiurma Sanchez-Perez, Laura de Armas-Rillo, Estefania Armas-Gonzalez, Jose David Machado, Federico Diaz-Gonzalez
Summary: It is well established that patients with systemic sclerosis (SSc) have disrupted lipid profile and increased cardiovascular risk. Cholesterol efflux capacity (CEC) and lipid profile were impaired in SSc patients compared to controls, with skin thickness being independently and inversely associated with CEC in SSc patients.
ARTHRITIS RESEARCH & THERAPY
(2021)
Article
Immunology
Michael Gernert, Hans-Peter Tony, Matthias Froehlich, Eva Christina Schwaneck, Marc Schmalzing
Summary: This study investigated the use of immunosuppressive medication (IS) after autologous hematopoietic stem cell transplantation (aHSCT) in systemic sclerosis (SSc) patients. The results showed that approximately 60% of SSc patients required IS after aHSCT for disease control. Rituximab was found to be an effective treatment option. Long-term data suggested that aHSCT is safe.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Health Care Sciences & Services
Paul Gressenberger, Philipp Jud, Gabor Kovacs, Sonja Kreuzer, Hans-Peter Brezinsek, Katharina Guetl, Viktoria Muster, Ewald Kolesnik, Albrecht Schmidt, Balazs Odler, Gabriel Adelsmayr, Peter Neumeister, Luka Brcic, Sabine Zenz, Kurt Weber, Thomas Gary, Marianne Brodmann, Winfried B. Graninger, Florentine C. Moazedi-Fuerst
Summary: Systemic sclerosis (SSc) is a difficult-to-treat autoimmune disease characterized by vascular damage and organ fibrosis. Autologous hematopoietic stem cell transplantation (AHSCT) may be considered for high-risk SSc patients, but the need for additional immunosuppressive therapies such as rituximab (RTX) post-AHSCT remains unclear. This report highlights the potential efficacy of RTX in SSc patients who do not respond well to AHSCT, with improved clinical symptoms and organ functions observed after RTX treatment.
JOURNAL OF PERSONALIZED MEDICINE
(2021)
Article
Rheumatology
Amra Adrovic, Mehmet Yildiz, Fatih Haslak, Oya Koker, Ayten Aliyeva, Sezgin Sahin, Kenan Barut, Ozgur Kasapcopur
Summary: The study evaluated the efficacy of TCZ treatment in JSS patients, showing significant improvements in HRCT, PGA, and DLCO, as well as reductions in mRSS and J4S scores. TCZ may be a potential treatment option for patients who do not respond to conventional therapy.
RHEUMATOLOGY INTERNATIONAL
(2021)
Review
Cell Biology
Daniel Levin, Mohammed S. Osman, Caylib Durand, Hyein Kim, Iman Hemmati, Kareem Jamani, Jonathan G. Howlett, Kerri A. Johannson, Jason Weatherald, Matthew Woo, Jason Lee, Jan Storek
Summary: Systemic sclerosis is a complex connective tissue disease with limited efficacy from conventional therapies. Autologous hematopoietic stem cell transplantation shows promise but comes with associated risks. Patient selection, efficacy, and toxicity are vital considerations for treatment.
Article
Rheumatology
Kimberly Showalter, Robert Spiera, Cynthia Magro, Phaedra Agius, Viktor Martyanov, Jennifer M. Franks, Roshan Sharma, Heather Geiger, Tammara A. Wood, Yaxia Zhang, Caryn R. Hale, Jackie Finik, Michael L. Whitfield, Dana E. Orange, Jessica K. Gordon
Summary: Histologic and gene expression features were investigated in early diffuse cutaneous systemic sclerosis (dcSSc) to determine clinical improvement. The study found that CD34 and aSMA staining patterns can predict gene expression subsets and are associated with clinical assessments. Furthermore, a 47-gene fibroblast polarisation signature was identified in clinical improvers with CD34 and aSMA binarised scores.
ANNALS OF THE RHEUMATIC DISEASES
(2021)
Article
Medicine, General & Internal
Marin Petric, Dijana Perkovic, Ivona Bozic, Daniela Marasovic Krstulovic, Dusanka Martinovic Kaliterna
Summary: This study suggests a possible association between serum serotonin levels and skin fibrosis and pulmonary dysfunction in patients with systemic sclerosis (SSc). However, further studies are needed to confirm these relationships.
MEDICINA-LITHUANIA
(2022)
Article
Rheumatology
Matthew M. K. Woo, Daniel Levin, Dorothy Y. Li, Joel David, Michelle Buresi, Milli Gupta, Yasmin Nasser, Christopher N. Andrews, Caylib Durand, Mohammed S. Osman, Kareem Jamani, Jason Weatherald, Kerri A. Johannson, Jonathan G. Howlett, Iman Hemmati, Hyein Kim, Michael Curley, Jan Storek
Summary: This study shows that autologous hematopoietic cell transplantation (HCT) has no measurable beneficial effect on esophageal motility in patients with unmeasurable peristalsis. However, in patients with measurable peristalsis, HCT appears to stabilize and partially normalize motility, unless relapse occurs.
CLINICAL RHEUMATOLOGY
(2023)
Review
Medicine, General & Internal
Satoshi Ebata, Asako Yoshizaki-Ogawa, Shinichi Sato, Ayumi Yoshizaki
Summary: This paper outlines the latest advances in the treatment of systemic sclerosis (SSc), with a focus on the efficacy and safety of Nintedanib, Tocilizumab, and Rituximab.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Pharmacology & Pharmacy
Chen Chen, Lingbiao Wang, Jinfeng Wu, Meijuan Lu, Sen Yang, Wenjing Ye, Ming Guan, Minrui Liang, Hejian Zou
Summary: This study found that serum PIIINP, TIMP-1, and ELF score were significantly elevated in patients with SSc and correlated with the extent of skin fibrosis. Serum PIIINP can be a sensitive marker for longitudinal changes in skin fibrosis, while the ELF score can serve as a surrogate marker for skin fibrosis.
FRONTIERS IN PHARMACOLOGY
(2022)
Article
Dermatology
I Marti-Marti, D. Morgado-Carrasco, S. Podlipnik, D. Rizo-Potau, X. Bosch-Amate, G. M. Lledo, M. Suarez-Lledo, G. Espinosa, C. Martinez, J. M. Mascaro Jr, P. Giavedoni
Summary: High-frequency ultrasonography (HFUS) is an effective tool for monitoring disease activity in sclerosing dermatoses (SD) and can impact clinical management by identifying characteristics of active lesions, leading to treatment adjustments for some patients. Further studies are needed to assess the outcomes of management changes supported by HFUS in SD.
CLINICAL AND EXPERIMENTAL DERMATOLOGY
(2022)