Background: systemic sclerosis is a rare systemic acquired disease with a complex pathogenesis which compromises multiple organs. The nutritional approach to treat patients with this disease is not clearly stablished due to its low prevalence. Case report: we present the case of a 37-year-old woman diagnosed with systemic sclerosis with gastrointestinal compromise, 40% weight loss since diagnosis and severe anemia. Despite oral supplementation, placement of a gastrostomy feeding tube and medical therapy with octreotide, her nutritional requirements are not achieved, so we suggest home parenteral nutrition to complete the nutritional support. Discussion: the severe intestinal affection in this patient made it necessary to turn to the parenteral access because of the oral intolerance secondary to the intestinal pseudo-obstruction. In these cases, the lack of etiological treatment makes home parenteral nutrition play an important role in the nutritional approach.
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http://dx.doi.org/10.20960/nh.02732 | DOI Listing |
Clin Rheumatol
January 2025
Rheumatology Unit, Scleroderma Unit, University Hospital of Modena, Via del Pozzo, 71-41125, Modena, Italy.
The aims of this study were to investigate the prevalence of cryofibrinogenemia in a cohort of patients with systemic sclerosis (SSc) regardless of clinical manifestations, who were admitted to our hospital and determine the associations among CF positivity, disease features and ongoing therapies. This was a monocentric and retrospective study. The inclusion criteria were a diagnosis of SSc (according to the ACR/EULAR 2013 classification criteria), regular administration of i.
View Article and Find Full Text PDFNeurol Clin Pract
April 2025
Department of Neurology, New York University Langone Health.
Background And Objectives: Neurosarcoidosis poses a diagnostic and management challenge due to its rarity, phenotypic variability, and lack of randomized controlled studies to guide treatment selection. Recommendations for management based on expert opinion are useful in clinical practice and provide a framework for designing prospective studies.
Methods: In this Delphi survey study, specialists with experience in managing patients with neurosarcoidosis were invited to anonymously complete 2 surveys about key elements of evaluation, diagnosis, treatment, monitoring, and long-term management of neurosarcoidosis.
Rheumatol Adv Pract
December 2024
Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK.
Objectives: Patients' outcomes and experiences can be affected by rheumatologist knowledge and awareness of systemic sclerosis (SSc). Our survey, directed at UK-based rheumatologists, aimed to expand our understanding of the above points along with their ability to keep up to date with guidelines defining best practice.
Methods: Rheumatologists were invited to participate in an online survey, with the results analysed and presented descriptively and graphically by SRUK.
Semin Arthritis Rheum
January 2025
Unit of Immunology, Rheumatology, Allergy and Rare diseases, IRCCS San Raffaele Hospital, Milan, Italy; Vita-Salute San Raffaele University, Milan, Italy.
Background: Primary heart involvement (pHI) is frequent in systemic sclerosis (SSc), and is associated with a poor prognosis. Therapeutic strategies to treat SSc-pHI are not yet defined.
Objectives: To evaluate the efficacy of immunosuppressive therapy on cardiac magnetic resonance (CMR) features in patients with CMR-proven SSc-pHI.
J Dermatol Sci
January 2025
Biosciences Department, Durham University, Durham, United Kingdom. Electronic address:
Background: Systemic Sclerosis (SSc) is an idiopathic rheumatic inflammatory disease that is characterised by inflammation and skin fibrosis. Type I interferon is significantly elevated in the disease.
Objective: The objective of this study is to determine the role of the TCA cycle metabolite fumarate in SSc.
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