Lipodermatosclerosis and Pulmonary Hypertension in Systemic Sclerosis.

Importance: Lipodermatosclerosis (LDS) stems from vascular dysfunction and dermal inflammation and thereby is mechanistically similar to systemic sclerosis (SSc). The association of LDS with SSc in the clinical setting has not been well characterized in the literature.

Objective: To evaluate the prevalence of LDS in SSc and the association of LDS with vascular complications, particularly pulmonary hypertension, in patients with SSc.

Design, Setting, And Participants: This retrospective cohort study used prospectively collected longitudinal data from a cohort of patients from the multidisciplinary rheumatology and dermatology clinic at a single tertiary care center from November 2004 to November 2022. Adult patients (aged ≥18 years at the time of cohort entry) with SSc were included.

Exposure: Clinical diagnosis of LDS based on expert opinion or histopathologic findings.

Main Outcomes And Measures: The main outcomes included prevalence of LDS, the association of LDS with the macrovascular complications, including pulmonary hypertension, digital gangrene and/or scleroderma renal crisis. Disease complications, including cardiac arrhythmias and heart failure, were compared among patients with and without LDS.

Results: Among 567 patients with SSc (494 [87.1%] female; mean [SD] age, 53.4 [14.4] years), 25 (4.4%) had LDS and 542 (95.6%) did not have LDS. Skin ulceration occurred in 8 patients with LDS (32.0%). Patients with LDS had higher frequencies of cardiac arrhythmia (11 of 24 [45.8%] vs 145 of 539 [26.9%]), heart failure (7 [28.0%] vs 55 [10.1%]), and pulmonary hypertension (12 [48.0%] vs 137 of 541 [25.3%]) compared with patients without LDS. Frequency of scleroderma renal crisis and digital gangrene did not differ significantly between patients with and without LDS (0 vs 37 [6.8%] and 4 [16.0%] vs 69 of 538 [12.8%], respectively). Among patients with LDS, 9 (36.0%) were either discharged to hospice or died during follow-up compared with 115 patients without LDS (21.2%). Lipodermatosclerosis was associated with pulmonary hypertension (adjusted prevalence odds ratio, 3.10; 95% CI, 1.33-7.25).

Conclusions And Relevance: In this cohort study, LDS was a rare clinical manifestation in patients with SSc but was associated with pulmonary hypertension. Therefore, patients with LDS should be closely monitored and screened for pulmonary hypertension.