Systemic sclerosis & Raynaud's

Treatment patterns and outcomes of scleroderma renal crisis in the international scleroderma renal crisis survey II – An inception cohort study

Semin Arthritis RheumPublished prospective, international inception cohort

Authors' conclusion

Our data show that 1-year mortality and dialysis rates remain high in SRC and suggest that blood pressure should be controlled within 5-10 days after SRC presentation, as opposed to the expert-recommended 72 h, in order to maximize survival.

Study context

Population
Subjects with new-onset scleroderma renal crisis (SRC) in systemic sclerosis, identified by physicians with expertise in systemic sclerosis (SSc); outcomes collected from January 2020 to January 2022subjects with new-onset SRC identified, 83 with 1-year follow-up · with 1-year follow-up 83
Participants
97 subjects
Exposure
Survival of subjects by the number of days to control blood pressure
Outcome
All-cause mortality
Follow-up
Within 12 months of SRC
Methodological details2-year web-based survey completed by international SSc experts, Cox proportional hazards models
  • 2-year web-based survey completed by international SSc experts
  • Cox proportional hazards models

Main finding

As reported in the abstract

One-year incidence mortality rate

39 per 100 person-years

Observational data: associations, not effects.

Further findings

Survival by time to control blood pressure

Time to control blood pressure associated with best survival
between 5-10 days after SRC presentation

Characteristics of the 83 with 1-year follow-up

Median disease duration, of the 83 with 1-year follow-up
9 (interquartile range (IQR) 5-24) months
Diffuse cutaneous disease, of the 83 with 1-year follow-up
63 (76%)
RNA polymerase III autoantibodies, of the 83 with 1-year follow-up
38 (46%)

Anti-hypertensive treatment

ACE inhibitors used (most frequently used anti-hypertensive)
98%
Required ≥ three antihypertensives to control blood pressure
54%

Dialysis · one year

Required dialysis
49%
Remained on dialysis, of the 55 survivors at one year
25%

Time required to control blood pressure

Median time
7 (IQR 3-10) days

Original publication

Treatment patterns and outcomes of scleroderma renal crisis in the international scleroderma renal crisis survey II – An inception cohort study. Semin Arthritis Rheum. Published 25 October 2026. doi:10.1016/j.semarthrit.2026.153093

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