P052 Case series: a rare form of calcinosis in patients with systemic sclerosis-myositis overlap. (23rd April 2022)

• Record Type: Journal Article
• Authors: Achieng, Sheilla; Samaranayaka, Muditha; Herrick, Ariane L
• Is Part Of: Rheumatology. Volume 61(2022)Supplement 1
• Publication Date: 2022-04-23
• Subjects: Rheumatism -- Periodicals; Rheumatology -- Periodicals; 616.723005
• DOI: 10.1093/rheumatology/keac133.051
• Languages: English
• ISSNs: 1462-0324

Abstract

Abstract: Background/Aims: Calcinosis is a well described entity that occurs in systemic sclerosis (SSc) and dermatomyositis. In patients with SSc, calcinosis typically occurs over pressure points but very rarely, widespread calcinosis may occur, presenting in an extensive pattern. We present a case series of four patients with SSc with this rarer form of diffuse calcinosis with the aim of raising awareness of this poorly recognised pattern of debilitating disease. Methods: Four cases of SSc with extensive calcinosis were identified from a cohort of 461 patients with SSc who had attended a tertiary referral centre over the preceding 3 years, and their case notes reviewed. Results: Demographic and clinical features of the four patients are summarised in Table 1 . All four patients had the diffuse cutaneous subtype of SSc in the context of 102 patients [22%] of the entire SSc cohort of 461 patients, and all had overlapping features of myositis. Two were anti-Scl 70 antibody positive and the other two were anti-PMScl- positive. Widespread calcinosis was evident in all four patients in a pattern akin to 'sheets' of calcified deposits, very different to the lobular pattern of disease that has been well-described in the literature. Development of calcinosis occurred between one and six years of the onset of SSc, and in one patient the calcinosis discharged through skin at multiple sites. In all patients, treatment included mycophenolate mofetil. Treatments tried for the calcinosis included intravenous sodium thiosulfate in two patients (on the basis of anecdotal reports), but without significant clinical improvement. Conclusion: It is well recognised that dystrophic calcification in SSc usually associates with disease duration. In our case series, however, all four patients presented with calcinosis very early on in the course of their disease and another striking point was that all had overlapping features of diffuse cutaneous SSc and myositis. Awareness of this widespread pattern of disease (and its associations) is lacking, highlighting a need for further research into the pathophysiology of this rare phenotype of debilitating calcinosis to identify future targeted treatments. Disclosure: S. Achieng: None. M. Samaranayaka: None. A.L. Herrick: None.