Review
Immunology
Marc Scheen, Arnaud Dominati, Valerie Olivier, Samih Nasr, Sophie De Seigneux, Arsene Mekinian, Naim Issa, Fadi Haidar
Summary: Systemic sclerosis is a rare autoimmune vasculopathy that causes generalized systemic fibrosis due to dysregulated innate and adaptive immunity. Renal involvement is common in systemic sclerosis and is associated with a poorer prognosis. Scleroderma renal crisis, the most studied renal complication in systemic sclerosis, has seen a decrease in mortality rate with the use of ACE inhibitors and improved management.
AUTOIMMUNITY REVIEWS
(2023)
Review
Health Care Sciences & Services
Francesco Reggiani, Gabriella Moroni, Claudio Ponticelli
Summary: Systemic sclerosis is a chronic autoimmune disease that often involves the kidneys. Scleroderma renal crisis is the most serious renal event associated with this condition, and early and aggressive treatment is necessary to prevent irreversible organ damage and death.
JOURNAL OF PERSONALIZED MEDICINE
(2022)
Review
Rheumatology
George Iliopoulos, Dimitrios Daoussis
Summary: Research has shown limited data about renal impairment in SSc beyond scleroderma renal crisis (SRC). However, studies indicate that renal dysfunction in SSc is not uncommon, with a significant percentage of patients having decreased glomerular filtration rate (GFR). The Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) formula is suggested as a suitable tool for assessing renal function in SSc patients.
RHEUMATOLOGY INTERNATIONAL
(2021)
Article
Allergy
Alice Cole, Voon H. Ong, Christopher P. Denton
Summary: Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) with a high mortality rate. Although angiotensin-converting enzyme inhibitor therapy has improved renal outcomes, there are currently no preventative measures and patients may rapidly decline. Studies have identified specific phenotypes at higher risk of developing SRC and novel urinary biomarkers may aid in early identification and treatment. Clear guidelines for management have been established to standardize care and promote collaboration between rheumatology and renal physicians. While outcomes following renal transplant have improved, further research is needed to understand the mechanisms and develop new therapies for SRC.
CLINICAL REVIEWS IN ALLERGY & IMMUNOLOGY
(2023)
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
Hideaki Tsuji, Nobuo Kuramoto, Tsuneo Sasai, Mirei Shirakashi, Hideo Onizawa, Koji Kitagori, Shuji Akizuki, Ran Nakashima, Ryu Watanabe, Akira Onishi, Kosaku Murakami, Hajime Yoshifuji, Masao Tanaka, Motomu Hashimoto, Koichiro Ohmura, Akio Morinobu
Summary: Specific SSc-related autoantibodies are associated with the morbidity and mortality of SRC. Anti-topoisomerase I, anti-RNAPIII, and anti-U1-RNP are associated with the development of SRC, while anti-topoisomerase I is associated with 1-year mortality of SRC.
Article
Immunology
Giacomo De Luca, Giulio Cavalli, Corrado Campochiaro, Cosimo Bruni, Alessandro Tomelleri, Lorenzo Dagna, Marco Matucci-Cerinic
Summary: Systemic sclerosis (SSc) is a rare and severe connective tissue disease characterized by endothelial and vascular damage, immune activation, leading to inflammation and fibrosis of skin and internal organs. Cardiac involvement is common in SSc, often asymptomatic initially, with myocardial fibrosis considered the immunopathologic hallmark. Interleukin (IL)-1 and inflammasome play key roles in SSc-heart inflammation, and targeted inhibition of IL-1 is proposed as a potential treatment strategy.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Medicine, General & Internal
Antonietta Gigante, Giorgia Leodori, Chiara Pellicano, Annalisa Villa, Edoardo Rosato
Summary: Kidney involvement in systemic sclerosis (SSc) can range from scleroderma renal crisis, which is the most severe complication, to subclinical renal vasculopathy. These complications can lead to reduced renal function and vascular damage.
AMERICAN JOURNAL OF THE MEDICAL SCIENCES
(2022)
Article
Emergency Medicine
Mohammed Uddin, Tanveer Mir, Sarvani Surapaneni, Anupamdeep Mehar, Tawseef Dar, Khalid Changal, Waqas Ullah, Prateek Lohia, Zeenat Bhat, Mujeeb Sheikh, Mark Burket
Summary: This study analyzed the mortality rate of scleroderma renal crisis (SRC) among systemic sclerosis (SSc) patients in US emergency departments. The study found that SRC is a relatively rare medical emergency, and although the incidence rate has increased significantly, the mortality rate remains steady. Compared to non-SRC patients, those with SRC have more complications, particularly congestive heart failure. Therefore, SSc patients with congestive heart failure should be considered for early admission to inpatient services.
AMERICAN JOURNAL OF EMERGENCY MEDICINE
(2022)
Review
Immunology
Ayumi Yoshizaki, Takemichi Fukasawa, Satoshi Ebata, Asako Yoshizaki-Ogawa, Shinichi Sato
Summary: This article outlines the role of B cells in the development of systemic sclerosis (SSc), including their involvement in autoimmune abnormalities, pro-inflammatory actions, and inhibitory functions. Studies have found B-cell depletion therapy to be effective for SSc.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Rheumatology
D. Xu, L. Zhu, R. Cai, Z. Yi, H. Zhang, G. Guo, S. Liu, J. Xu, Q. Wang, Y. Su, X. Li, J. Zhao, R. Mu
Summary: A multi-predictor nomogram for evaluating the risk of SRC in SSc patients was successfully developed, containing four easily available predictors. The nomogram showed good discrimination and calibration in both internal and external validation.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2021)
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)
Review
Chemistry, Medicinal
Barbara Ruaro, Marco Confalonieri, Marco Matucci-Cerinic, Francesco Salton, Paola Confalonieri, Mario Santagiuliana, Gloria Maria Citton, Elisa Baratella, Cosimo Bruni
Summary: Systemic sclerosis patients often suffer from interstitial lung disease, with early diagnosis playing a pivotal role in improving prognosis. Treatment strategies typically involve a combination of immunosuppressants and targeted biological therapies based on disease severity and progression risk. Hematopoietic autologous stem cell transplantation has shown benefits for progressive SSc patients, while lung transplantation is considered for refractory cases of SSc-ILD.
Article
Medicine, General & Internal
Yassmin Khadra, Jennifer G. Walker, Paul Hakendorf, Susanna Proudman, Maureen Rischmueller, Catherine L. Hill, Samuel Whittle, Eliza Pontifex, Peter J. Roberts-Thomson
Summary: SRC is a rare but severe complication with a stable incidence of 0.58/million/year in South Australia. Patients have poor survival outcomes, often requiring renal replacement therapy and transplantation. Nailfold capillaroscopy reveals extensive microvascular damage. No improvement in survival was observed over the 34-year study period.
INTERNAL MEDICINE JOURNAL
(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)
