Relationship between change in skin score and disease outcome in diffuse cutaneous systemic sclerosis: Application of a latent linear trajectory model

Abstract

Objective. To explore the relationship between changes in the severity of skin disease and morbidity and mortality in patients with diffuse cutaneous systemic sclerosis (dcSSc).Methods. From a large single-center cohort, we identified 225 patients with dcSSc for whom serial clinical information was available from within 24 months of the onset of the first non-Raynaud's phenomenon manifestation of SSc. The end points analyzed included death and heart, lung, kidney, and gastrointestinal tract involvement. Latent linear trajectory modeling (LTM) was applied to identify patients with a similar trajectory of modified Rodnan skin thickness score (MRSS) changes over the first 3 years of followup. Clinical outcomes were compared between 3 different LTM subgroups.Results. LTM permitted classification of 131 patients (58%) into 1 of 3 subgroups with different skin score trajectories. Survival was lowest in the subgroup of patients who had a high baseline skin score and experienced little improvement during followup (P ‫؍‬ 0.003). However, the frequency of clinical end points was similar in the subgroup with the most favorable trajectory (i.e., a low initial MRSS and subsequent improvement) and the subgroup with a high baseline MRSS and no improvement. Interestingly, the end point frequency was greatest in the subgroup with a high initial MRSS and subsequent improvement, suggesting that sustained severe skin disease does not necessarily predict the number of visceral complications, and that the relationship between the skin score and internal organ involvement in dcSSc is more complex than previously thought.Conclusion. Although mortality was highest among patients with the worst skin-related outcomes, no simple relationship between burden of disease and change in skin score was observed.

Scleroderma Lung Involvement, Autoantibodies, and Outcome Prediction: The Confounding Effect of Time

“…Thus, skin involvement in patients with dcSSc, as measured by the modified Rodnan skin score (mRSS), tends to worsen comparatively rapidly in the initial stages of the disease. The majority of patients with the diffuse subset of SSc reach their peak mRSS within the first 3 years from first non-Raynaud symptom, and skin tends to improve thereafter in about 80% of the subjects 18,19 . Deterioration in skin disease or new development of worsening skin thickening in patients with disease duration longer than 5 years is extremely rare and often a sign of an underlying more sinister pathology.…”

Systemic sclerosis – diagnostic and therapeutic recommendations of the Polish Dermatological Society. Part 2: treatment

“…Severe or rapidly progressive cutaneous lesions at an early disease stage are usually associated with organ involvement and increased mortality rates [21]. Severe skin lesions typically develop in early diffuse cutaneous systemic sclerosis.…”

“…Nasilone lub szybko postępujące zmiany skórne obecne we wczesnym etapie choroby wiążą się często z występowaniem zmian narządowych i zwiększoną śmiertelnością [21]. Zwykle nasilone zmiany skórne stwierdza się we wczesnej twardzinie z uogólnionymi stwardnieniami skóry.…”

Biomarkers in Systemic Sclerosis

“…It has long been recognized that there are at least two distinct clinical subsets of SSc differing in their clinical presentation and evolution, but most importantly, with clearly different outcomes regarding frequency and severity of organ involvement as well as overall mortality [5,[32][33][34]. The extent of cutaneous sclerotic involvement has been found to accurately distinguish the two clinical subsets in the majority of cases.…”