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.
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
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
Timothy Kaniecki, Tsion Abdi, Zsuzsanna H. McMahan
Summary: Systemic sclerosis often affects the gastrointestinal tract, leading to impaired function and significant morbidity. This article consolidates current guidelines and proposes a symptom-based framework for diagnosis and management, while also identifying areas in need of further research.
BEST PRACTICE & RESEARCH IN CLINICAL RHEUMATOLOGY
(2021)
Review
Rheumatology
Elizabeth R. Volkmann, Zsuzsanna McMahan
Summary: This article reviews the assessment and treatment strategies for gastrointestinal involvement in patients with systemic sclerosis (SSc) and provides updates on recent research contributions in this area. The findings offer opportunities to enhance patient care and identify future research directions.
CURRENT OPINION IN RHEUMATOLOGY
(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
Rheumatology
Jamie Bering, W. Leroy Griffing, Michael Crowell, Sarah B. Umar
Summary: Research shows that patients with SSc generally demonstrate longitudinal stability or improvement in GI symptoms, but a subset of patients experience worsening of GI symptoms, leading to negative impacts on GI-related quality of life.
RHEUMATOLOGY INTERNATIONAL
(2021)
Article
Rheumatology
Rory H. Maclean, Fiza Ahmed, Voon H. Ong, Charles D. Murray, Christopher P. Denton
Summary: In this phenome-wide association study (PheWAS), we investigated the causes and contributors to gastrointestinal dysfunction in systemic sclerosis (SSc) using clinical records data. We found associations between drugs, diagnoses, and antinuclear antibodies (ANA) with important SSc-GI outcomes such as constipation, diarrhea, and gastroesophageal reflux. Our study also identified potential novel risk factors for SSc-GI dysfunction.
JOURNAL OF RHEUMATOLOGY
(2023)
Article
Immunology
Patrick Coit, Kaila L. Schollaert, Emily M. Mirizio, Kathryn S. Torok, Amr H. Sawalha
Summary: The study assessed DNA methylation differences between juvenile systemic sclerosis (jSSc) and juvenile localized scleroderma (jLS), revealing distinct epigenetic patterns and unique methylated genes in both diseases. Compared to healthy controls, differential methylation sites and genes were identified in jSSc and jLS, indicating potential novel diagnostic biomarkers for localized scleroderma. Pathway analysis showed enrichment of inflammatory pathways in jSSc and the HIPPO signaling pathway in jLS, highlighting different biological processes involved in each condition.
CLINICAL IMMUNOLOGY
(2021)
Review
Gastroenterology & Hepatology
Ayesha Shah, Veenaa Pakeerathan, Michael P. Jones, Purna C. Kashyap, Kate Virgo, Thomas Fairlie, Mark Morrison, Uday C. Ghoshal, Gerald J. Holtmann
Summary: This systematic review and meta-analysis investigated the prevalence of small intestinal bacterial overgrowth (SIBO) in systemic sclerosis (SSc), as well as the risk factors and the effects on gastrointestinal symptoms. The study found that SIBO prevalence in SSc patients is 39.9%, with a 10-fold increased prevalence compared to controls. It also suggested that antimicrobial therapy should be considered for SSc patients with SIBO and diarrhea.
JOURNAL OF NEUROGASTROENTEROLOGY AND MOTILITY
(2023)
Article
Rheumatology
Giuseppina Abignano, Gianna Angela Mennillo, Giovanni Lettieri, Duygu Temiz Karadag, Antonio Carriero, Angela Anna Padula, Francesco Del Galdo, Dinesh Khanna, Salvatore D'Angelo
Summary: The study demonstrated that SSc patients with impaired esophageal transit scintigraphy findings have higher GIT 2.0 reflux scores, while esophageal emptying activity is negatively correlated with reflux scores.
JOURNAL OF RHEUMATOLOGY
(2021)
Article
Medicine, General & Internal
Chingching Foocharoen, Ajanee Mahakkanukrauh, Pewpan Maleewong, Wanchai Maleewong, Amonrat Jumnainsong, Patnarin Pongkulkiat, Nattiya Teawtrakul, Siraphop Suwannaroj, Ratanavadee Nanagara
Summary: Peripheral eosinophilia is observed among systemic sclerosis (SSc) patients, with approximately one in five patients showing elevated eosinophil count. Factors associated with eosinophilia in SSc patients include male gender, longer disease duration, and the presence of Raynaud's phenomenon, while SSc subset, serology, and cytokine levels are not correlated with eosinophilia.
AMERICAN JOURNAL OF THE MEDICAL SCIENCES
(2022)
Article
Rheumatology
Melody P. Chung, Christopher A. Mecoli, Jamie Perin, Carrie Richardson, Zsuzsanna H. McMahan
Summary: The objective of this study was to determine the association between perifollicular hypopigmentation and demographics, clinical features, and autoantibody profiles in systemic sclerosis patients. The results showed that perifollicular hypopigmentation was associated with black race, diffuse cutaneous SSc, higher skin scores, myopathy, pulmonary fibrosis, lower lung function, and anticentromere antibodies exhibited an inverse association. After adjusting for age, race, and disease duration, diffuse cutaneous SSc and increased skin scores were significantly associated with perifollicular hypopigmentation.
JOURNAL OF RHEUMATOLOGY
(2022)