Change in Calcinosis over 1 Year Using the SCTC Radiologic Scoring System for Calcinosis of the Hands in Patients with Systemic Sclerosis

Antonia Valenzuela¹, Melody Chung ², Tatiana S. Rodríguez-Reyna ³, Susanna Proudman ⁴, Murray Baron ⁵, Flavia Castelino ⁶, Vivien Hsu ⁷, Shufeng Li ⁸, David Fiorentino ⁹, Kathryn Stevens ¹⁰ and Lorinda Chung ², ¹Pontificia Universidad Catolica de Chile, Santiago de Chile, Chile, ²Stanford University, Palo Alto, CA, ³Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, ⁴Royal Adelaide Hospital North Terrace, Adelaide, Australia, ⁵Jewish General Hospital, McGill University, Montreal, Canada, ⁶Harvard University, Boston, MA, ⁷Rutgers- RWJ Medical School, SOUTH PLAINFIELD, NJ, ⁸Division of Immunology and Rheumatology, Department of Medicine, Stanford, Palo Alto, CA, ⁹Department of Dermatology, Stanford University School of Medicine, Redwood City, CA, ¹⁰Stanford University, Stanford

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: calcinosis, hand, radiography and multicenter study, Scleroderma

Session Information

Date: Monday, November 11, 2019

Title: Systemic Sclerosis & Related Disorders – Clinical Poster II

Session Type: Poster Session (Monday)

Session Time: 9:00AM-11:00AM

Background/Purpose: Calcinosis cutis is a debilitating complication of systemic sclerosis (SSc) affecting one quarter of patients, most frequently involving the hands. We previously developed and published a radiographic scoring system to assess severity of calcinosis affecting the hands in patients with SSc that is feasible with excellent inter- and intra-rater reliability. We sought to further validate our radiographic scoring system to assess for change over 1 year and to identify factors associated with improvement or progression.

Methods: Baseline and 1-year antero-posterior hand radiographs were obtained in 39 SSc patients with calcinosis who were prospectively enrolled at 6 centers within the US, Canada, Mexico and Australia. Two blinded readers scored all radiographs using the calcinosis scoring system. We defined progressive calcinosis as >10% increase in score from baseline at 1 year, stable calcinosis as change in score between -10% to 10%, and improvement of calcinosis as decrease in score by >10% (Figure 1).

Results: Our cohort was 85% female, 69% Caucasian, 28% Hispanic, and 3% Asian. 72% had limited cutaneous disease. Mean disease duration from first non-Raynaud phenomenon (RP) symptom was 16.9±11.4 years. Inter-rater reliability was high with intra-class correlation coefficient of 0.93 (0.89-0.95). The median percentage of change from baseline to 1 year was 12.8% (range -89.3-290.2%). Twenty-one patients (54%) experienced progression of calcinosis over 1 year with a range of 12.7-290.2% worsening from baseline; 10 (26%) remained stable; and 8 (20%) had improvement (range -89.3-7.4%)(Figure 1). Patients with progressive calcinosis had lower mean modified Rodnan skin score (mRSS) (3.81 vs. 6.5, p=0.0446) and lower prevalence of pulmonary artery hypertension (PAH) by right heart catheterization than patients who did not progress (0 % vs 23%, p= 0.022)(Table 1). They also exhibited a trend toward having more digital pitting scars (76% vs. 50%, p= 0.0892), and arthritis (52% vs. 34%, p= 0.0694). Patients whose calcinosis improved had higher mean mRSS (7.13 vs. 4.52, p=0.061), less arthritis (0 vs. 50%, p=0.0154) and greater prevalence of antibodies against PM-Scl (43 vs. 3%, p=0.018) than patients whose calcinosis did not improve. They also exhibited a trend toward having less gastrointestinal disease (50 vs. 84%, p=0.0651). In multivariable analysis, a trend for anti-PM-Scl antibodies to be a predictor of calcinosis improvement persisted (OR 13.5 (0.99 – 183.14), p=0.051)(Table 2).

Conclusion: We confirmed the excellent inter-rater reliability of our radiographic calcinosis scoring system and quantified changes in calcinosis severity over 1 year in a longitudinal cohort of SSc patients. More than half of patients experienced >10% progression of calcinosis over one year; however, 20% of patients improved and these patients were more likely to be positive for the PM-Scl antibody.

Figure 1. Distribution of change in calcinosis score over 1 year

ACR 2019_Table 1

Table 1. Characteristics of patients whose calcinosis progressed and did not progress and whose calcinosis improved and did not improve.

ACR 2019_Table 2

Table 2. Predictors for progression and improvement of calcinosis of the hands in multivariable analysis

Disclosure: A. Valenzuela, None; M. Chung, None; T. Rodríguez-Reyna, None; S. Proudman, None; M. Baron, None; F. Castelino, Boehringer-Ingelheim, 5, Corbus, 9, Cumberland, 9, Galapagos, 9, Scleroderma Research Foundation, 2; V. Hsu, None; S. Li, None; D. Fiorentino, Janssen, 5, Pfizer, 2, 5, UCB Pharmaceuticals, 5; K. Stevens, None; L. Chung, BMS, 6, 9, Boehrenger-Ingelheim, 5, Boehringer Ingelheim, 5, Boehringer-Ingelheim, 5, Bristol-Myers Squib, 5, Eicos, 5, 6, 9, Eicos Steering Committee, 5, Mitsubishi Tanabe, 5, Reata, 5, 6, Reata DSMB, 5, Reatta, 5.

To cite this abstract in AMA style:

Valenzuela A, Chung M, Rodríguez-Reyna T, Proudman S, Baron M, Castelino F, Hsu V, Li S, Fiorentino D, Stevens K, Chung L. Change in Calcinosis over 1 Year Using the SCTC Radiologic Scoring System for Calcinosis of the Hands in Patients with Systemic Sclerosis [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/change-in-calcinosis-over-1-year-using-the-sctc-radiologic-scoring-system-for-calcinosis-of-the-hands-in-patients-with-systemic-sclerosis/. Accessed .

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