Background/Purpose: To investigate the progression of hand contractures in patients with systemic sclerosis and to identify disease features predictive of contractures. A sub-group analysis was also performed on patients with less than or equal to 2 years since disease onset.

Methods: Patients enrolled in the GENISOS cohort were assessed for disease features predictive of hand contractures. Such variables are included in Table 1. 

Linear mixed model was used to analyze longitudinal measurements of each hand separately given the potential for variation in the extent of contracture development. The time variable is the date of first non-Raynaud’s symptom.

A subgroup analysis was also performed with the same disease variables to assess for predictive features and to assess if the rate of decline was faster earlier in disease.

Results: 1108 sets of hand measurements were evaluated over a median follow-up period of 8.8 years. 219 patients were included in the analysis and 62 patients in the subgroup analysis. In the right hand, ATA and MRSS scores were predictive of a decrease in hand extension. ATA positive patients showed a decrease in right hand extension by 0.24 cm/year while ATA negative patients showed a decrease in hand extension by 0.075 cm/year. A similar decrease in hand extension was observed in the left hand for ATA positive patients.

A unit increase in the MRSS score was predictive of faster decline in right hand extension by 0.006 cm/yr.

In the subgroup analysis, disease type and ACA were predictive of contracture development in the right hand. Patients with diffuse disease were found to have a decrease in right hand extension by 0.64 cm/year. ACA negative patients were found to have a decrease in hand extension by 0.21 cm/yr in the right hand.

In the left hand, ATA, ACA and digital ulcers were predictive of contracture development. ATA positive patients showed a decrease in left hand extension by 1.54 cm/year while ATA negative patients showed a decrease in hand extension by 0.08 cm/year. Further, the absence of digital ulcers was also predictive of decrease in hand extension in the left hand.

Conclusion:  This is the largest and longest reported prospective study assessing disease features predictive of hand contractures in patients with systemic sclerosis.

ATA and MRSS scores were predictive of hand contracture development in the right hand. ATA was also predictive of hand contracture development in the left hand. Discrepancy in skin score as a predictor between the two hands may be related to hand dominance.

Subgroup Analysis

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/scleroderma-hand-contracture-study/