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P.161 Severe cardiac involvement in systemic sclerosis: two cases of scleroderma cardiac crisis

jsrd · 2026-06-05 · canonical JSON source

10 visible annotations · policy: published · automated confidence ≥ 75.00%

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Introduction Cardiac involvement in systemic sclerosis (SSc) remains a significant cause of morbidity and mortality, particularly in diffuse cutaneous forms with high disease activity. Scleroderma cardiac crisis, though rare as a phenotype, poses a critical diagnostic and therapeutic challenge due to its rapid progression and high fatality rate.Material and Methods We present two cases of female patients with recent onset of diffuse cutaneous SSc and anti-Scl-70 positivity, high Modified Rodnan Skin Score and systemic inflammation, who developed severe myocardial dysfunction progressing to cardiogenic shock and death.Results The first patient - aged 55 - was admitted with fatigue, hypotension, and conduction abnormalities, evolving to rapidly declining left ventricular ejection fraction (LVEF: 51% to 35% to 20%) as first manifestations of the disease. Cardiac Magnetic resonance (CMR) showed myocardial fibrosis but despite immunosuppression (intravenous methylprednisolone and rituximab) and cardioprotective treatment she developed acute cardiogenic shock, necessitated ICU admission and vasopressor support but she did not respond and died.The second patient – aged 61- had a diagnosis of diffuse SSc for 16 months and was on treatment with low dose prednisolone, mycophenolate, tocilizumab and nifedipine with partial control of her disease. She was on regular follow-up with a normal cardiac echocardiogram 3 months ago. She presented with acute-onset dyspnea due to heart failure. Despite initial response to diuretics, she deteriorated hemodynamically with a rapid reduction of cardiac function, leading to ICU transfer and non-response to standard interventions.These cases highlight the need for heightened clinical suspicion by rheumatologists and cardiologists for cardiac crisis in patients with early, diffuse SSc and persistent inflammatory burden. Timely CMR imaging, multidisciplinary management, and aggressive immunomodulation in parallel with cardioprotective treatment may improve outcomes, although prognosis remains guarded.Conclusions Scleroderma cardiac crisis is a devastating and often fatal complication of SSc. Early recognition, cardiac monitoring, and prompt initiation of immunosuppressive, cardio-protective and supportive therapy are essential, however no specific treatment has been proved to be effective in these patients.