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During the congress, E-Posters will be accessible to all participants on the congress website 24/7, as well as in the E-poster stations in the congress center.
Preparing your E-Poster
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E-Poster Submission Deadline
Please prepare and upload your E-Poster no later than March 14, 2026 11.59PM CET. After this date, you will no longer be able to prepare and upload your E-poster and it will not be displayed and accessible on the congress website.
Please follow the instructions below to input your abstract title.
Abstract titles should be brief and reflect the content of the abstract.
Scleroderma renal crisis (SRC) is a condition associated with poor prognosis. Compared to seropositive cases, seronegative systemic sclerosis (SSc) involves milder organ involvement. Here, we report a case of seronegative diffuse SSc with multiple organ involvement, including SRC, which necessitated hemodialysis. The patient was later found to have scleroderma-related anti-SSCA1 antibodies.
A 48-year-old man initially presented to our dermatology clinic 5 years ago with skin thickening. Upon examination, he exhibited thickened and hyperpigmented skin on the fingers, dorsal feet, upper arms, and anterior chest, with a modified Rodnan skin score (mRSS) of 44 points. Testing for antinuclear antibodies, anti-Scl-70, anti-centromere, and anti-RNA polymerase Ⅲ antibodies was negative. A skin biopsy confirmed findings characteristic of scleroderma, leading to a diagnosis of seronegative diffuse SSc. Two years prior to his admission, the patient was diagnosed with SRC due to a rapid decline in renal function and severe hypertension. Renal function initially improved with angiotensin-converting enzyme (ACE) inhibitors. However, one year later, proteinuria increased and renal function gradually worsened. He was admitted to our department with joint contractures from skin thickening, difficulty eating, and progressive renal dysfunction. Computed tomography imaging revealed marked intestinal dilatation, and a diagnosis of paralytic ileus secondary to intestinal pseudo-obstruction was made. Testing using the A-cube assay for scleroderma-specific antibodies revealed the presence of anti-SSSCA1 antibodies, which were confirmed with an in-house Western blot assay. The patient’s condition improved after treatment with rituximab and rehabilitation, allowing for discharge two months later. However, he subsequently developed end-stage renal failure requiring emergency dialysis and died one month later. Renal autopsy confirmed the severe and characteristic findings of SRC.
SRC is frequently associated with scleroderma-specific autoantibodies, such as anti-RNA polymerase Ⅲ antibodies. Seronegative diffuse SSc typically manifests as a milder disease; and cases with SRC progressing to end-stage renal failure requiring hemodialysis, as in this report, are rare. Anti-SSSCA1 antibodies have been implicated in systemic scleroderma and Sjögren's syndrome, but there are no previous case reports or series linking these antibodies to SRC. The clinical implications of anti-SSSCA1 antibodies remain poorly understood. The rapid deterioration of the patient's renal function due to SRC may be related to anti-SSSCA1 antibodies.
Diffuse SSc with anti-SSSCA1 antibodies may be associated with severe SRC.
