Prevalence and clinical association with acro-osteolysis in early systemic sclerosis

Abstract

ObjectiveAcro-osteolysis is often associated with systemic sclerosis (SSc). However, the severity of acro-osteolysis and its clinical association among SSc patients is limited. Our aims were to assess the prevalence of acro-osteolysis and the clinical association with acro-osteolysis among SSc patients at early onset of the disease. MethodsA cross-sectional study of 120 newly diagnosed SSc patients with the onset of less than 4 years were evaluated on clinical characteristics and hand radiographs. Acro-osteolysis was graded on a 0-4-point scale based on the severity and the patients were subdivided into mild, moderate and severe. ResultsAmong all SSc patients enrolled, 62.5% were females, 56.1% dcSSc and the vast majority of them (84.1%) were positive for anti-topoisomerase I antibody (anti-topo I). The mean disease duration was 2.0±1.3 years. Acro-osteolysis was noted in 77 patients with a prevalence of 64.1% (95%CI 54.9-72.7), of which 16.7% were defined as severe acro-osteolysis. Logistic regression analysis revealed that acro-osteolysis was positively associated with anti-topo I (OR 13.96), hand deformity (OR 3.81) and dysphagia (OR 6.66), but negatively associated with oedematous skin (OR 0.05). Analysis stratified by severity of acro-osteolysis showed significant differences between subgroup in terms of the presence of digital gangrene (p=0.02), ischaemic ulcer (p=0.001), oedematous skin (p=0.001), and hand deformities (p=0.01). ConclusionAcro-osteolysis was common in SSc at the early onset of disease. While the presence of anti-topo I, hand deformity and esophageal involvement were strongly associated with acro-osteolysis, oedematous skin was the protective factor for acro-osteolysis.

Acro-osteólisis, hallazgos de imagen y posibles diagnósticos etiológicos

“…Entre las causas de acro-osteólisis, se pueden diferenciar dos grandes grupos: las primarias, como los síndromes de Hajdu-Cheney, de Saroyoshi, de Papillon-Lefèvre, de Kid y de Thenevard, entre otros, donde se combinan alteraciones cutáneas y compromiso vascular 3 , y las secundarias o adquiridas, las más frecuentes, entre las que se encuentran las idiopáticas no familiares, por exposición a tóxicos (cloruro de polivinilo), el estrés mecánico repetido o enfermedades sistémicas como la artritis psoriásica o la esclerodermia 3,[4][5][6] .…”