Article
Rheumatology
Michael Hughes, Suiyuan Huang, John D. Pauling, Maya Sabbagh, Dinesh Khanna
Summary: This study explores patient priorities and ranking of factors influencing decision-making regarding treatment escalation in Raynaud's phenomenon secondary to systemic sclerosis. The results show that patients prioritize factors such as inability to use fingers properly, new digital ulcers, worsening pain or discomfort, more severe attacks, and potential internal problems when considering treatment escalation. Patients also express concerns about potential treatment side effects and show a preference for nonpharmacologic management of SSc-RP.
ARTHRITIS CARE & RESEARCH
(2021)
Review
Medicine, General & Internal
Tijana Tuhy, Paul M. Hassoun
Summary: Systemic sclerosis is an autoimmune disorder that affects the connective tissue and causes skin thickening and complications in the internal organs. Pulmonary involvement, including pulmonary arterial hypertension and interstitial lung disease, is the main cause of morbidity and mortality in scleroderma patients. Current treatment options are not specific to pulmonary involvement in scleroderma, and patients with pulmonary arterial hypertension in this population tend to have worse outcomes and lower response to therapy compared to other forms of Group I pulmonary hypertension.
FRONTIERS IN MEDICINE
(2023)
Review
Medicine, General & Internal
Magdalena Maciejewska, Mariusz Sikora, Cezary Maciejewski, Rosanna Alda-Malicka, Joanna Czuwara, Lidia Rudnicka
Summary: Raynaud's phenomenon is a vascular condition that can be primary or secondary to other medical conditions. Systemic sclerosis is a common cause of secondary Raynaud's phenomenon.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Rheumatology
Nancy Maltez, Michael Hughes, Edith Brown, Virginia Hickey, Heiyoung Park, Beverley Shea, Ariane L. Herrick, John D. Pauling, Susanna Proudman, Peter A. Merkel
Summary: When studying RP and DUs, it is important to use outcome measures that fully evaluate their complexities. The OMERACT Vascular Disease in SSc Working Group is identifying core disease domains that encompass the full burden of SSc-related RP and DUs.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2021)
Review
Rheumatology
Daniel Ennis, Zareen Ahmad, Melanie A. Anderson, Sindhu R. Johnson
Summary: This article reviews the effectiveness of botulinum toxin injections in treating Raynaud's phenomenon and discusses formulations, dosages, administration sites, and adverse effects. The evidence for botulinum toxin in the treatment of Raynaud's phenomenon is promising, but further research is needed to ensure consistency in treatment outcomes across different patient populations and treatment options.
BEST PRACTICE & RESEARCH IN CLINICAL RHEUMATOLOGY
(2021)
Article
Physiology
Sadaf Iqbal, Jaume Bacardit, Bridget Griffiths, John Allen
Summary: This study demonstrates the potential of using convolutional neural networks for DL-PPG classification to detect SSc. EfficientNetB0 outperforms GoogLeNet, and both CNNs perform better than traditional machine learning methods. This automated AI approach has significant benefits for SSc diagnostics in various clinical settings.
FRONTIERS IN PHYSIOLOGY
(2023)
Review
Medicine, General & Internal
Peter Klein-Weigel, Oliver Sander, Simone Reinhold, Jessica Nielitz, Julia Steindl, Jutta Richter
Summary: Raynaud's phenomenon (RP) is characterized by vasospasm of the fingers or toes triggered by cold or emotional stressors. The most important diagnostic steps include demonstrating a tendency towards vasospasm, excluding occlusions, nail-fold capillaroscopy, and determining autoantibody status. The main treatment options are protection against cold and administration of calcium antagonists.
DEUTSCHES ARZTEBLATT INTERNATIONAL
(2021)
Article
Cardiac & Cardiovascular Systems
An Tran-Duy, Kathleen Morrisroe, Philip Clarke, Wendy Stevens, Susanna Proudman, Joanne Sahhar, Mandana Nikpour
Summary: The cost-effectiveness of combination therapy for systemic sclerosis-related pulmonary arterial hypertension was evaluated. The results showed that combination therapy was more cost-effective compared to monotherapy, especially in terms of quality-adjusted life years. At higher willingness-to-pay thresholds, combination therapy was considered a cost-effective option.
JOURNAL OF THE AMERICAN HEART ASSOCIATION
(2021)
Article
Rheumatology
Susan L. Murphy, Alain Lescoat, Mary Alore, Michael Hughes, John D. Pauling, Maya Sabbagh, Dinesh Khanna
Summary: There are differences in how people with primary and secondary RP define RP, with primary RP patients more likely to focus on the impact on quality of life and pain, while secondary RP patients tend to emphasize specific body parts and management of attacks. These findings have implications for assessing RP outcomes in different patient populations.
CLINICAL RHEUMATOLOGY
(2021)
Article
Rheumatology
Francesco Zulian, Gloria Lanzoni, Biagio Castaldi, Alessandra Meneghel, Francesca Tirelli, Elisabetta Zanatta, Giorgia Martini
Summary: This study reports the first case series of patients with ssJSSc, a rare condition in childhood characterized by no skin involvement. Compared to classic JSSc, ssJSSc showed a longer diagnostic delay, higher frequency of cardiac involvement, and worse outcomes.
Review
Biochemistry & Molecular Biology
Nehme El-Hachem, Manal M. Fardoun, Hasan Slika, Elias Baydoun, Ali H. Eid
Summary: Raynaud's Phenomenon is caused by exaggerated cold-induced vasoconstriction, leading to compromised digital blood perfusion and potential tissue damage. Various drugs have been used to alleviate symptoms, but there is currently no FDA-approved drug for treating RP. One potential option being explored is the repurposing of cilostazol, which acts as a selective inhibitor of phosphodiesterase-III with antiplatelet and vasodilating properties.
CURRENT MEDICINAL CHEMISTRY
(2021)
Review
Biology
Michele Colaci, Maria Letizia Aprile, Domenico Sambataro, Gianluca Sambataro, Lorenzo Malatino
Summary: The presence of liver involvement in systemic sclerosis (SSc) is considered atypical, besides the possible coexistence of other autoimmune hepatic disorders. However, the occurrence of portal hypertension and, more specifically, of the syndromes called idiopathic portal hypertension (IPH) and regenerative nodular hyperplasia (RNH) have been anecdotally reported in the literature for SSc patients. No specific SSc pattern linked to IPH emerged, even though reports from the literature often described the limited skin subset. Coexistence of prothrombotic states and overlap with other hepatic diseases could facilitate IPH onset. Besides being a rare condition, the onset of IPH in SSc patients is an occurrence that should be taken into account.
Review
Immunology
Manuel Rubio-Rivas, Narcis A. Homs, Daniel Cuartero, Xavier Corbella
Summary: The overall PAH prevalence found was 6.4% (95%CI 5%-8.3%) and the overall PAH incidence 18.2 cases per 1000 person-years (95%CI 12-27.4). Studies on lcSSc and dcSSc showed different prevalence rates, with lcSSc at 7.7% (95% CI 5.3%-11.1%) and dcSSc at 6.3% (95% CI 4.5%-8.9%). Overall, the incidence of PAH in SSc patients was found to be 18.2 cases per 1000 person-years.
AUTOIMMUNITY REVIEWS
(2021)
Article
Medicine, General & Internal
Adem Erturk, Ozgur Erogul, Murat Kasikci
Summary: This cross-sectional study compared the findings of optical coherence tomography angiography (OCT-A) in patients with primary Raynaud's phenomenon (PRP), very early disease of systemic sclerosis (VEDOSS), and systemic sclerosis (SSc). OCT-A shows potential diagnostic value in differentiating patients with PRP from those with SSc and VEDOSS, and there is potential value in assessing prognostic roles.
Review
Rheumatology
Nancy Maltez, Michael Hughes, Edith Brown, Virginia Hickey, Beverley Shea, Ariane L. Herrick, Susanna Proudman, Peter A. Merkel, John Pauling
Summary: Despite the challenges in assessing SSc-RP, various research studies have used different methods and measures to study this condition. The main domains explored in these studies include severity, frequency, and duration of RP attacks. The findings from this study will help establish a core set of disease domains to assess the impact of RP in SSc.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2023)
