Article
Dermatology
Claudia Delbruck, Thilo Gambichler, Laura Susok, Frank Peinemann
Summary: The study aimed to evaluate the effects of extracorporeal photopheresis as a treatment modality for patients with systemic sclerosis. After conducting a meta-analysis on three randomized clinical trials, it was found that extracorporeal photopheresis did not show a superior effect on skin scores compared to no treatment, sham photopheresis, or D-penicillamine.
DERMATOLOGIC THERAPY
(2022)
Review
Medicine, General & Internal
Mahmoud Nassar, Victoria Ghernautan, Nso Nso, Akwe Nyabera, Francisco Cuevas Castillo, Wan Tu, Luis Medina, Camelia Ciobanu, Mostafa Alfishawy, Vincent Rizzo, Saphwat Eskaros, Mamdouh Mahdi, Mohamed Khalifa, Mohamed El-Kassas
Summary: The gastrointestinal tract is frequently affected in individuals with systemic/localized scleroderma, leading to various GI manifestations and complications. Females are more susceptible to developing scleroderma, and esophageal and intestinal symptoms are common. Abnormal bowel movement and bacterial overgrowth contribute to malabsorption and increased mortality rate. Fecal incontinence is a common symptom that can lead to depression.
Article
Rheumatology
Robyn T. Domsic, Shiyao Gao, Maureen Laffoon, Steven Wisniewski, Yuqing Zhang, Virginia Steen, Robert Lafyatis, Thomas A. Medsger
Summary: Regardless of whether the first SSc manifestation or first non-Raynaud manifestation is used to define disease onset, a disease duration of less than 18 months at enrollment is preferable. A longer disease duration criterion more frequently results in regression to the mean of the mRSS score, which likely contributes to negative trial outcomes.
Review
Immunology
Maheswari Muruganandam, Angie Ariza-Hutchinson, Rosemina A. Patel, Wilmer L. Sibbitt
Summary: Systemic sclerosis is a complex autoimmune disease characterized by vascular damage, inflammation, and fibrosis. Biomarkers play an important role in understanding the disease process and potential therapeutic targets. Anti-nuclear antibodies are classical biomarkers, while other proteins and pathways are also implicated. The use of biomarker panels combined with advanced analysis techniques can help determine disease activity and treatment response.
JOURNAL OF INFLAMMATION RESEARCH
(2023)
Article
Immunology
Jakob Hoeppner, Christoph Tabeling, Vincent Casteleyn, Claudia Kedor, Wolfram Windisch, Gerd Ruediger Burmester, Doerte Huscher, Elise Siegert
Summary: The aim of this study was to comprehensively analyze the serum autoantibody status in patients with systemic sclerosis (SSc) and correlate it with the clinical course of the disease. The study found that specific autoantibodies are associated with specific clinical manifestations, while the association of some rare antibodies is not fully clarified. The results of this study reveal important associations between the serologic status of SSc patients and disease manifestation, comorbidities, and complications.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Biochemistry & Molecular Biology
Bianca Saveria Fioretto, Irene Rosa, Marco Matucci-Cerinic, Eloisa Romano, Mirko Manetti
Summary: Systemic sclerosis (SSc, scleroderma) is a rare connective tissue disease characterized by immune dysregulation, small vessel vasculopathy, impaired angiogenesis, and fibrosis. Microvascular impairment is the initial event of the disease and is linked to disabling and life-threatening clinical manifestations. Despite available treatment options, managing SSc-related vascular disease remains challenging. Vascular biomarkers have shown promise in assessing disease progression, predicting prognosis, and guiding therapy.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Dermatology
Scott McGaugh, Penelope Kallis, Anna De Benedetto, Renee M. Thomas
Summary: Translation: This article reviews preclinical studies and clinical cases of JAK inhibitors for the treatment of morphea and the cutaneous manifestations of SSc, providing evidence to support further research and use of JAK inhibitors in the management of skin fibrosis in morphea and SSc.
DERMATOLOGIC THERAPY
(2022)
Review
Biochemistry & Molecular Biology
Devis Benfaremo, Silvia Agarbati, Matteo Mozzicafreddo, Chiara Paolini, Silvia Svegliati, Gianluca Moroncini
Summary: Systemic sclerosis, also known as scleroderma or SSc, is a highly varied disease in terms of clinical presentation, disease progression, and treatment response. Recent advancements in skin molecular profiling technologies have enabled the identification of patient subgroups based on gene expression analysis. This narrative review provides an overview of the current status of skin gene expression analysis and emphasizes the benefits of stratifying patients based on their gene signatures. This stratification has the potential to lead to a precision medicine approach in the management of SSc.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Medicine, General & Internal
Pawel Zebryk, Piotr Przymuszala, Jan Krzysztof Nowak, Tomasz Piorunek, Tatiana Mularek-Kubzdela, Mariusz Puszczewicz
Summary: This study evaluated the prevalence and clinical significance of SSc-related autoantibodies and compared the sensitivity of two line immunoblot assays. The findings indicate that different autoantibodies are associated with specific characteristics of SSc.
JOURNAL OF CLINICAL MEDICINE
(2023)
Review
Rheumatology
Leonardo Martin Calderon, Mitali Chaudhury, Janet E. Pope
Summary: This study provides a systematic review of the economic impact and healthcare resource utilization associated with systemic sclerosis (SSc). The findings indicate that SSc poses a significant economic burden on patients and healthcare resources, with the burden increasing with SSc-associated complications and varying depending on geographical location and access to healthcare resources.
Article
Medicine, General & Internal
Anna Bohdziewicz, Katarzyna Karina Pawlik, Magdalena Maciejewska, Mariusz Sikora, Rosanna Alda-Malicka, Joanna Czuwara, Lidia Rudnicka
Summary: Systemic sclerosis is a complex autoimmune disease characterized by vasculopathy and fibrosis. Current therapeutic options are insufficient, but research on novel targets shows potential for new treatment options in the future.
JOURNAL OF CLINICAL MEDICINE
(2022)
Review
Allergy
Ming Zhao, Jiali Wu, Haijing Wu, Amr H. Sawalha, Qianjin Lu
Summary: Scleroderma consists of two major clinical subsets: systemic sclerosis and localized scleroderma, each with distinct characteristics and clinical manifestations. Systemic sclerosis can lead to organ complications, while localized scleroderma is primarily confined to the skin and subcutaneous tissues.
CLINICAL REVIEWS IN ALLERGY & IMMUNOLOGY
(2022)
Review
Medicine, General & Internal
Kholoud Almaabdi, Zareen Ahmad, Sindhu R. Johnson
Summary: Systemic sclerosis is an autoimmune disease characterized by immune abnormalities, vasculopathy, and fibrosis. Autoantibody testing, including ANA, anti-Scl-70, and anticentromere antibodies, has become increasingly important for diagnosis and prognostication. This review examines the epidemiology, clinical associations, and prognostic value of advanced autoantibody testing in systemic sclerosis.
Article
Biochemistry & Molecular Biology
Caterina Oriana Aragona, Antonio Giovanni Versace, Carmelo Ioppolo, Daniela La Rosa, Rita Lauro, Maria Concetta Tringali, Simona Tomeo, Guido Ferlazzo, William Neal Roberts, Alessandra Bitto, Natasha Irrera, Gianluca Bagnato
Summary: This review discusses recent advancements in the treatment of systemic sclerosis-associated interstitial lung disease, including new molecular targets and approved drugs to slow disease progression. The focus is on personalized treatment strategies based on emerging pathogenic features of the disease.
Article
Rheumatology
Bader A. AlMehmadi, Fergus Z. To, Melanie A. Anderson, Sindhu R. Johnson
Summary: Peripheral neuropathy in systemic sclerosis is not uncommon and can be treated with decompression surgery and medication. Limited and conflicting data exist regarding the use of immunosuppressives and anticonvulsants for treating this condition, highlighting the need for randomized controlled trials to evaluate their efficacy.
JOURNAL OF RHEUMATOLOGY
(2021)
