En coup de sabre (ECDS) is a form of linear-scleroderma, primarily affecting the face. Despite effectiveness of pharmacological interventions in the management of lesions, sequelae of cutaneous deform...
Review the existing surgical tools for the correction of "en coup de sabre" ECDS lesions and facial deformities....
The authors conducted a literature search for reports on surgical interventions for ECDS deformations, in the electronic databases of PubMed, Scopus, and Cochrane Library Databases....
Twenty-six publications reporting 39 patients were found in the literature. The average age of patients was 25 ± 4.7 years. Thirty-one of the patients were females. Six methods were found in the liter...
Different surgical and minimally invasive options exist in the armamentarium of clinicians correcting ECDS deformities. Tailoring the method of correction to patient's needs and expectation is crucial...
To describe systemic sclerosis (SSc) heart involvement in the ICU....
We retrospectively studied patients with previous diagnosis of SSc admitted to the ICU for acute cardiac dysfunction between 2012 and mid-2021....
9 female patients were included, mainly with diffuse SSc (n = 7, 78%). Six (67%) had digital ulcers. All but one patient complained about physical cardiac symptoms (n = 8, 89%), 5 (56%) had electrocar...
We report an uncommon and specific severe acute life-threatening cardiac dysfunction in SSc patients, which can be reversible but remains associated with a poor long-term prognosis, which can be rever...
Recent evidence has indicated that alterations in energy metabolism play a critical role in the pathogenesis of fibrotic diseases. Studies have suggested that 'metabolic reprogramming' involving the g...
Dermal fibroblasts were isolated from five SSc patients and five healthy donors. Cells were cultured in medium with/without TGF-β1 and with/without ALK5, pan-PIM or ATM kinase inhibitors. Extracellula...
Enhanced mitochondrial respiration coupled to ATP production was observed in SSc fibroblasts at the expense of spare respiratory capacity. Although no difference was found in glycolysis when comparing...
Our data provide evidence for a novel mechanism(s) by which SSc fibroblasts exhibit altered metabolic programs and highlight changes in respiration and dysregulated mitochondrial morphology and functi...
Localized scleroderma (LS), also called circumscribed scleroderma or morphea, comprises a heterogeneous group of diseases that can be classified into four subtypes: limited, linear, generalized, and m...
Patients with diffuse cutaneous systemic sclerosis (dcSSc) display a complex clinical phenotype. Transcriptional profiling of whole blood or tissue from patients are affected by changes in cellular co...
SSc patients were recruited from the Prospective Registry for Early Systemic Sclerosis. Clinical data were collected, as well as peripheral blood for isolation of CMs and NCMs. Age-, sex-, and race-ma...
We used an unbiased approach to cluster patients into 3 groups (groups A-C) based on the transcriptional signatures of CMs relative to controls. Each group maintained their characteristic transcriptio...
We are the first to show that transcriptional signatures of CMs and NCMs can be used to unbiasedly stratify SSc patients and correlate with disease activity outcome measures....
Scleroderma renal crisis (SRC) is a rare and severe manifestation of systemic sclerosis (SSc). Although it is well documented that Black patients with SSc have worse morbidity and mortality than non-B...
Using the electronic health record of the US Military Health System, we conducted a comprehensive chart review of each patient with SSc from 2005 to 2016. The final study cohort was comprised of 31 SR...
Of 353 patients, 294 had identifiable race (79 Black, 215 non-Black). Thirteen of 79 Black patients (16.5%) versus 16 of 215 (7.4%) non-Black patients developed SRC (P = 0.02). On adjusted analysis, B...
Black race was independently associated with a higher risk of future SRC. Further studies are needed to elucidate the mechanisms that underlie this important association....
Nailfold videocapillaroscopy is the gold standard for the early differentiation of primary and secondary Raynaud's phenomenon. Advances in high-frequency ultrasound with superb microvascular imaging s...
Pulmonary arterial hypertension (PAH) is a severe, high mortality and progressive disease. Early diagnosis and treatment improves the prognosis. Patients with scleroderma disease presents high risk of...
Prospective study of 52 consecutive patients diagnosed of scleroderma in our health area, during 2 years (2018 and 2019). All of them undergo CPET, in addition to the annual systematic screening. Sens...
Fifty-two CPET were performed, of which 16 suggested PAH. Resting RHC confirmed PAH in 5 patients and exercise RHC in 7 (diagnostic sensitivity of CPET together with rest and exercise catheterization ...
CPET and exercise RHC could detect PAH earlier than established screening in patients with scleroderma disease, allowing early diagnosis....
Sicca complaints are a frequent reason for rheumatologic consultation. Testing for specific antibodies against Ro/SSA and La/SSB antigens and minor salivary gland (MSG) biopsy are among the main tools...
Demographic, clinico-pathological, and laboratory data were recorded in 216 consecutive patients with sicca complaints. All study participants were tested for SSc-specific autoantibodies (against CENP...
SSc-specific autoantibodies were detected in 41.7% (90/216) patients evaluated (19% at strong, 22.7% at medium titers) without significant differences between anti-Ro/SSA positive and negative groups....
SSc-specific autoantibodies are frequently detected among patients presenting with sicca complaints and at strong but not medium titers are independently associated with MSG biopsy positivity. Taken t...
To evaluate the levels of periostin in patients with systemic sclerosis (SSc) and their association with features of systemic sclerosis....
The levels of periostin were assessed in the serum of 106 SSc patients and 22 healthy controls and by immunofluorescence staining in cardiac tissue from 4 SSc patients and 4 controls. Serum periostin ...
Periostin levels were higher in patients with SSc compared to controls and directly correlated to modified Rodnan skin score and echocardiography parameters of left ventricular measurements. Immunoflu...
Periostin is elevated in SSc cardiac tissue in vivo and circulating levels of periostin are increased in SSc, correlating with the extent of disease duration, degree of skin fibrosis, and left ventric...
