Background/Purpose: Idiopathic inflammatory myopathies (IIMs) are rare systemic autoimmune disorders, with a pleiotropic clinical picture, specifically characterized from the inflammatory involvement of striate muscles. Calcinosis cutis is a chronic condition, that could be associated with connective tissue diseases, being a source of pain and functional disability. The aim of the study was to evaluate the prevalence of calcinosis in a monocentric cohort of patients with IIMs, exploring possible correlations with clinical variables and quality of life (QoL).

Methods: We retrospectively analyzed medical records of consecutive patients diagnosed with IIM according the EULAR/ACR 2017 criteria, collecting data about demography, subset and duration of disease, organ involvement, serology and comorbidities. QoL and WA were evaluated with Patients Reported Outcomes (PROs): Short-Form 36 Health Survey (SF-36), Functional Assessment of Chronic Illness Therapy Fatigue Subscale (FACIT-F), Health Assessment Questionnaire (HAQ), Work Productivity and Activity Impairment Questionnaire (WPAI), Hospital Anxiety and Depression Scale (HADS). Intergroups comparisons were assessed by using Chi-square, t-test and ANOVA. P values < 0.05 were considered significant.

Results: A total of 176 patients, 116 (65,9%) female, 79 (44,9%) with DM, 74 (42%) with PM, 11 (6,3%) with CADM, 10 (5,7%) with IBM, 2 (1,1%) with JDM were enrolled; 17 of them (9.7%), 12 (70,6%) women, had calcinosis. A statistically significant difference between respectively calcinosis subgroup and the whole cohort was found for the mean age (53,3±18,3 years vs 67,6±11,4, p< 0.001), the age at disease onset (30,1±16.6 years vs 58.9±12.9, p< 0.001), the age at diagnosis (42.1±17.6 years vs 61.6±12.8, p< 0.001) and the disease duration (14.2±9.0 years vs 8.7±6.7, p=0.003). Taking into account patients’ serological profiles, a significant correlation was observed between anti- PMScl100 positivity and calcinosis development (p=0.007). The majority of patients with calcinosis, as expected, had a cutaneous involvement (p< 0.001) and showed lower values of CPK and aldolase (p=0.03). Analysing patients’ comorbidities, we found no patient with calcinosis had developed cancer; this result remained significant after correcting for age and disease duration. PROs analysis showed patients with calcinosis had significantly lower values of both HADS-A, HADS-D and FACIT (p < 0.03); accordingly, they had significantly higher values of both Role Emotional and Social Function items of SF36 (p=0.03)

Conclusion: From the analysis of our data, calcinosis seems to be more frequent in younger patients, with an earlier disease onset and with a longer disease duration. Interestingly, we found a significant correlation with anti- PMScl100 antibodies and with a less severe muscle involvement. Notably, we found patients with calcinosis seem to have a significantly lower risk of developing cancer, independently from age. Therefore, even if calcinosis is considered a risk factor for disease severity and QoL compromission, our study highlighted a more favourable clinical profile, with a gain for patients in both their emotional and functional spheres.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/phenotyping-calcinosis-a-rare-manifestation-of-rare-diseases/