Introduction: Pericardial involvement in systemic scleroderma is rare but may be life-threatening when refractory to standard therapy. Among these, refractory pericarditis is a particularly severe complication.

Case Report: The patient presented with diffuse poikilodermal lesions, chest pain, oedema of the lower limbs and dyspnoea for ten days. Physical examination revealed tachycardia, a scleroderma-like facies, dyschromic plaques, skin sclerosis with a modified Rodnan score of 21, and a right heart failure syndrome. The electrocardiogram showed diffuse microvoltage, while echocardiography revealed a large circumferential pericardial effusion (35 mm opposite the left ventricle and 24 mm opposite the right ventricle). Biological tests revealed an inflammatory syndrome. Despite several pericardial aspirations, the effusion rapidly reconstituted. We adopted the diagnosis of a cardiac attack of SSc with refractory pericarditis of large volume. After two weeks, a moderate-sized effusion (17 mm opposite the right ventricle) was noted, treated with corticosteroids, azathioprine and hydroxychloroquine. The patient responded favorably to a combination of corticosteroids, azathioprine, and hydroxychloroquine, with resolution of symptoms and reduction of effusion.

Conclusion: This case illustrates the diagnostic and therapeutic challenges posed by refractory pericarditis in systemic scleroderma.

Pericarditis, refractory, systemic scleroderma, complex autoimmune disease