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OBJECTIVES We aimed to clarify the definition, distribution, clinical association and outcomes of large calcinosis in patients with systemic sclerosis (SSc). METHODS We conducted a systematic literature review (SLR) focusing on SSc-related large calcified masses. Upon updating the terminology and definition, all cases of "pseudotumoral" calcinosis seen at the Cochin and Padova University Hospitals were reviewed. RESULTS The SLR yielded 30 SSc cases, with large calcified masses mainly defined as "tumoral" or "pseudotumoral". Among the 629