Systemic Sclerosis Sine Scleroderma in Mexican Patients. Case Reports

Reumatol Clin (Engl Ed). 2018 Jul-Aug;14(4):230-232. doi: 10.1016/j.reuma.2016.11.004. Epub 2017 Jan 3.
[Article in English, Spanish]

Abstract

Systemic sclerosis sine scleroderma (ssSSc) is a form of systemic sclerosis that is characterized by Raynaud's phenomenon (RP), visceral involvement without thickening of skin and anticentromere antibodies (ACA). We studied 10 ssSsc patients with a prevalence of 2%. The clinical signs were: RP 9/10, esophageal manifestations 8/10, pulmonary arterial hypertension 4/10, interstitial lung disease 4/10, cardiac signs 3/10 and ACA 8/10.

Conclusion: In patients with RP, esophageal dysmotility, interstitial lung disease and pulmonary arterial hypertension should be tested for ACA in order to establish a prompt diagnosis and treatment of ssSSc.

Keywords: Clinical manifestations; Esclerosis sistémica sin esclerodermia; Manifestaciones clínicas; Prevalence; Prevalencia; Systemic sclerosis sine scleroderma.

MeSH terms

  • Adult
  • Aged
  • Female
  • Humans
  • Male
  • Mexico
  • Middle Aged
  • Retrospective Studies
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / diagnosis*
  • Scleroderma, Systemic / pathology