Systemic sclerosis & Raynaud's

Clinical characteristics, predictors and prognosis of systemic sclerosis with scleroderma renal crisis and pulmonary arterial hypertension

Clin Exp RheumatolPublished Retrospective cohort study

Authors' conclusion

PAH may precede, follow, or coincide with SRC. Patients with both SRC and PAH had a significantly higher risk of GIB. Older age and elevated NT-proBNP were independent factors associated with the coexistence of SRC and PAH.

Study context

Population
Patients diagnosed with systemic sclerosis (SSc) at the authors' institute, January 2013 to April 2024
Participants
234 patients
Exposure
SSc with both scleroderma renal crisis (SRC) and pulmonary arterial hypertension (PAH) vs PAH alone, SRC alone, and neither
  • SRC + PAHn = 17
Outcome
Coexistence of PAH and SRC: clinical features, predictors and prognosis
Methodological detailssingle-centre, multivariate logistic regression
  • Single-centre
  • Multivariate logistic regression

Main finding

As reported in the abstract

Prevalence of both PAH and SRC

SRC + PAH

7.3%

17

PAH preceded SRC
5
PAH followed SRC
3
PAH and SRC occurred simultaneously
9

Observational data: associations, not effects.

Further findings

OutcomeOR (95% CI)P value
Predictor of coexistence of PAH and SRCvs SRC alone: GIBGIB, independently associated9.545 (1.679–54.264)P = 0.011
Predictors of coexistence of PAH and SRC vs PAH alone: older age at onset and elevated NT-proBNPSRC + PAHvs PAH aloneOlder age at disease onset1.200 (1.014–1.420)P = 0.033
Predictors of coexistence of PAH and SRC vs PAH alone: older age at onset and elevated NT-proBNPSRC + PAHvs PAH aloneElevated NT-proBNP1.001 (1.000–1.001)P = 0.030

SRC + PAH vs SRC alone: clinical characteristics

SRC + PAH had higher prevalence of ILD, higher rate of gastrointestinal bleeding (GIB) and lower serum albumin than SRC alone

Original publication

Clinical characteristics, predictors and prognosis of systemic sclerosis with scleroderma renal crisis and pulmonary arterial hypertension. Clin Exp Rheumatol. Published 5 January 2026. doi:10.55563/clinexprheumatol/jr0zl5

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