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Lipodermatosclerosis and Pulmonary Hypertension in Systemic Sclerosis.

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Lipodermatosclerosis (LDS) is rare in systemic sclerosis (SSc) but linked to pulmonary hypertension. Patients with LDS require close monitoring and screening for this serious vascular complication.

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Area of Science:

  • Rheumatology
  • Dermatology
  • Vascular Medicine

Background:

  • Lipodermatosclerosis (LDS) shares mechanistic similarities with systemic sclerosis (SSc) due to vascular dysfunction and dermal inflammation.
  • The clinical association between LDS and SSc is not well-established in existing literature.

Purpose of the Study:

  • To determine the prevalence of LDS in patients with SSc.
  • To investigate the association between LDS and vascular complications, specifically pulmonary hypertension, in SSc patients.

Main Methods:

  • Retrospective cohort study utilizing prospectively collected longitudinal data.
  • Inclusion of adult patients diagnosed with SSc from a tertiary care center (November 2004 - November 2022).
  • Clinical diagnosis of LDS confirmed by expert opinion or histopathology.

Main Results:

  • LDS was present in 4.4% of 567 SSc patients.
  • Patients with LDS showed significantly higher rates of cardiac arrhythmia, heart failure, and pulmonary hypertension.
  • LDS was independently associated with pulmonary hypertension (aOR, 3.10; 95% CI, 1.33-7.25).

Conclusions:

  • Lipodermatosclerosis is an uncommon but significant finding in SSc patients.
  • The presence of LDS in SSc is associated with an increased risk of pulmonary hypertension.
  • Recommend vigilant monitoring and screening for pulmonary hypertension in SSc patients diagnosed with LDS.