Clinical Implications of Scleroderma in the Decision for Radiotherapy-Based Larynx Preservation
This case report describes 2 patients who presented with laryngeal complications after undergoing radiation therapy.
Publication date: Available online 25 January 2020Source: Reumatología Clínica (English Edition)Author(s): Lucía Suárez Pérez, Luis Caminal Montero, Luis Trapiella Martínez, Jessica Rugeles Niño
CONCLUSIONS: In our cohort, the prevalence of serodiscordant SSc patients was low. They differed from their counterparts in some clinical manifestations. The management of patients with SSc should be guided by both serology and cutaneous subtype. PMID: 31969223 [PubMed - as supplied by publisher]
CONCLUSIONS: Dilatation score performs best of all semi-quantitative NVC parameters in diagnosing SSc. In addition, our study confirms earlier reports that worse capillaroscopy pattern at baseline correlates with higher likelihood for adverse prognosis. PMID: 31969216 [PubMed - as supplied by publisher]
Neurocognitive impairment and musculoskeletal complications are among the most common complications of long-term allogeneic hematopoietic cell transplant (HCT) survivors, for which recommendations regarding routine screening and therapeutic interventions remain limited. Changes in cognitive functioning have been described in 40% of long-term allogeneic hematopoietic cell transplant survivors, and although the incidence of musculoskeletal complications has not been clearly defined, is very common- ranging from steroid-induced myopathy, myositis related to chronic graft-versus-host disease (GVHD), and fasciitis/scleroderma manifestations.
Resetting the immune system through autologous hematopoietic stem cell transplant (autoHSCT) is a highly effective treatment in patients with autoimmune diseases (AID). AutoHSCT achieved long-term remission in patients with relapsed refractory and secondary progressive multiple sclerosis (Muraro 2017), superior to their previous standard of care (Burt 2019). AutoHSCT in scleroderma patients achieved superior outcomes in two randomized studies (Tyndall 2014, Sullivan 2018). These impressive results are achieved by both eradication of autoreactive immune effector cells and re-establishment of a self-tolerant immune system, i...
AbstractIn systemic sclerosis (SSc) therapeutic efforts are often directed to prevent progressive respiratory impairment, but it is unclear to what extent changes in pulmonary function tests (PFTs) are associated with health-related quality of life (HRQoL). The aim of our study is to evaluate how modifications in PFTs contribute to longitudinal variations in HRQoL, assessed through the multidimensional questionnaire EQ-5D, in patients with SSc. We included SSc patients with forced vital capacity (FVC%), diffusing capacity of the lungs for carbon monoxide (DLCO%) and EQ-5D assessed in at least two visits. The EQ-5D consists...
PMID: 31974397 [PubMed - as supplied by publisher]
We report the case of a 72-year-old woman with limited systemic sclerosis, who was initially diagnosed with systemic sclerosis-related pulmonary arterial hypertension. However, after introduction of sildenafil and bosentan, the patient developed acute pulmonary oedema, and findings from complementary exams were suggestive of PVOD.
Publication date: Available online 21 January 2020Source: Best Practice &Research Clinical RheumatologyAuthor(s): A. Quinlivan, L. Ross, S. ProudmanAbstractEpidemiological studies reporting demographic, clinical and serological factors predictive of various outcomes in systemic sclerosis (SSc) range from the prediction of mortality to the development and progression of disease manifestations. However, predicting the disease trajectory in the individual patient is a challenging but important step towards a stratified approach to disease management. Recent technological advances provide the opportunity for new subgroupin...
Understanding the functions of disease-associated noncoding variants is essential for understanding the molecular mechanisms driving diseases with a genetic cause and for identifying therapeutic targets. Combined computational and experimental analyses have demonstrated that IRF5 is hyperactivated by a pathogenic allele of TNPO3 through long-distance chromatin looping. This finding identifies a molecular mechanism contributing to the polygenic autoimmune diseases of systemic lupus erythematosus and systemic sclerosis.