Systemic sclerosis-associated pulmonary arterial hypertension in children

Abstract

Systemic sclerosis (SSc) usually presents with a combination of vasculopathy, inflammation, autoimmunity, and fibrogenesis that can individually vary (Figures 1-3) (1). Major complications can include digital vasculopathy, gastrointestinal complications, cardiac and pulmonary fibrosis, scleroderma renal crisis, digital contractures, calcinosis, and acro-osteolysis (2).The etiology and pathogenesis of SSc is largely unknown. There is evidence of multifactorial genesis with interacting genetic, vascular, autoimmunological and metabolic factors (3,4). Genome-wide analysis showed a significant association of SSc with modifications of the fibrillin-1 gene and other genes involved in collagen metabolism. In skin and lung fibroblasts, collagen production is activated by transcription. Other candidate genes relate to HLA molecules and inflammation mediators, especially transforming growth factor β (TGF-β) and connective tissue growth factor (CTGF). Furthermore, a disturbed expression of metalloproteinases and their inhibitors has been described. An increased expression of cytokines and chemokines (IL-1, IL-4, TNF-α, IL-17, IL-21, MCP-1, MCP-2) can lead to an increased presence of immunological effector cells in the

Hipertensión arterial pulmonar en pacientes con dermatomiositis juvenil y esclerosis sistémica en un centro pediátrico de tercer nivel

“…A pesar de los avances en el tratamiento de la HAP, el pronóstico de HAP en ES es peor que otras formas de HAP con una mortalidad al año de hasta el 30%. En un informe francés reciente, durante el período 2006-2017, la supervivencia mejoró con el tiempo en pacientes de 70 años, pero no en pacientes mayores con ES e HAP 22 . En un metaanálisis de nueve estudios, que incluyeron 2700 pacientes con ES, el 55% murió de causas relacionadas con ES.…”

Calcinosis Cutis in Juvenile Systemic Sclerosis

“…Oral video capillaroscopy can be developed into a reliable technique for detecting oral microcirculation abnormalities [9]. The 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for systemic sclerosis still maintains its effectiveness in diagnosis and monitoring disease prognosis [10][11][12][13]. This patient was started on the tablet nifedipine (60 mg), a CCB, to treat the monophasic Raynaud's phenomenon.…”

Juvenile systemische Sklerodermie

“…Aufgrund dieser Werte wird die Indikation für eine Rechtsherzkatheter-Untersuchung gestellt. Bei pädiatrischen Patienten ist eine anti-Centromer-Antikörper-Positivität nicht mit einer pulmonalen Hypertension zu korrellieren [16]. Der DETECT-Algorithmus ist für pädiatrische Patienten nicht validiert und nicht anwendbar.…”