Background: Systemic sclerosis (SSc), a progressive disease characterized by excessive accumulation of connective tissue components. Although most patients have long survival, some of them progress rapidly to death. Pulmonary system involvement and pulmonary hypertension are the most frequent cause of death. When the patient with SSc is to be operated, the anesthetic procedure could be a serious problem. In this article, we report a combined spinal--epidural technique in a patient with progressive SSc and the anesthetic considerations that could be recommended for these patients.
Case Presentation: A 68-year-old woman who had a history of progressive systemic sclerosis, pulmonary fibrosis, kyphoscoliosis and decreased oral apertura underwent total hip arthroplasty. This operation was performed successfully under combined spinal epidural anesthesia.
Conclusion: Systemic sclerosis is a complex disease that involves multiple organ systems. Every aspects of anesthetic care may be altered or hindered by the pathogenesis of disease. Although the choice of regional or general anesthesia is unclear, to choose combined spinal epidural anesthesia may be useful.
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http://dx.doi.org/10.1186/1471-2253-6-11 | DOI Listing |
BMC Pulm Med
January 2025
Department of Pulmonary Medicine, National Hospital Organization MinamiKyushu Hospital, 1882 Kida, Aira-Shi, Kagoshima, 899-5293, Japan.
Background: Reports of autoimmune diseases coexisting with autoimmune pulmonary alveolar proteinosis (autoimmune PAP; APAP) are extremely rare. APAP coexisting with autoimmune diseases may often be misdiagnosed as connective tissue disease-associated interstitial lung disease (ILD). Here, we describe a rare case of a patient with systemic sclerosis who was diagnosed with APAP after the exacerbation of lung opacities during treatment with immunosuppressive agents.
View Article and Find Full Text PDFRheumatology (Oxford)
January 2025
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Objective: Early personalized identification of systemic sclerosis (SSc) patients at risk for scleroderma renal crisis (SRC) can help provide better treatment and improve outcomes. This study aimed to create and validate a new multi-predictor nomogram to predict SRC risk and compare it to an existing model.
Methods: A retrospective multicentre observational study was conducted using clinical data from SSc patients with SRC registered in the Chinese Rheumatism Data Center (CRDC) database.
Eur Heart J Cardiovasc Imaging
January 2025
Department of Cardiovascular Medicine, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan.
Aims: Left ventricular (LV) diastolic dysfunction and heart failure with preserved ejection fraction (HFpEF) are common cardiac complications of patients with systemic sclerosis (SSc). Exercise stress echocardiography is often used in symptomatic patients with SSc to detect abnormal increases in pulmonary pressures during exercise, but the pathophysiologic and prognostic significance of exercise stress echocardiography to assess the presence of HFpEF in these patients is unclear.
Methods And Results: Patients with SSc (n=140) underwent ergometry exercise stress echocardiography with simultaneous expired gas analysis.
Cureus
December 2024
Department of Oral Medicine, Nihon University School of Dentistry, Tokyo, JPN.
Calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome, a systemic sclerosis subtype, features skin thickening, vascular issues, and organ involvement, causing complications in the gastrointestinal and musculoskeletal systems. Herein, we present a rare case of oral candidiasis, with CREST syndrome. The patient presented with xerostomia, tongue erythema, and burning pain.
View Article and Find Full Text PDFACS Nano
January 2025
Department of Rheumatology and Immunology, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing 210008, China.
Cyclophosphamide has a certain therapeutic effect on treating systemic sclerosis (SSc), while difficulties exist in controlling severe systematic side effects and enhancing targeting capacity. Here, inspired from the natural extracellular matrix composition, we propose a cyclophosphamide-encapsulated nanogel based on dendritic polymers polyamidoamine (PAMAM) for SSc treatment. We combine bovine serum albumin and generation 5 (G5) PAMAM dendrimers with polyphenol modification to obtain nanogels featured with antioxidant and anti-inflammatory effects.
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