[Validation of the total morbidity score and investigation of the efficacy of methotrexate in localized scleroderma].

BACKGROUND

Localized scleroderma is an autoimmune disease belonging to the group of collagenoses, which can manifest cutaneously and extracutaneously. The extracutaneous manifestations may have significant morbidity but are not considered in previous scoring systems. For this reason, another scoring system, the total morbidity score (TMS) was developed, which also takes into account the extracutaneous symptoms.

METHOD

In the retrospective monocentric study at the Hamburg Center for Pediatric and Adolescent Rheumatology, the TMS was applied to patients from 2004-2019 suffering from localized scleroderma who had at least one control presentation. In addition, data were analyzed according to the previously established localized scleroderma cutaneous assessment tool (LoSCAT) scoring systems to ensure better comparability to the TMS. Furthermore, the score values were considered and compared during the course of treatment with methotrexate (MTX).

RESULTS

Due to a lack of control presentations, data from 51 of the 95 patients with a confirmed diagnosis could be included in the retrospective evaluation. The treatment of these patients was considered over a period of 2 years, from the initial presentation over at least 3 further control presentations. The TMS total score remained largely constant. There was a weak correlation between the TMS total score and the localized scleroderma skin damage index (mLoSDI), which indicates the degree of damage. In addition, insignificant changes in the TMS total score were shown over time with MTX treatment (T1/T4: -0.007).

DISCUSSION

The evaluation showed that the TMS total score is mainly fed by the extracutaneous manifestations, demonstrating the inaccuracy of previous scores. Another advantage of the TMS is that different scores are assigned depending on whether the feature is new, persistent, improving, or even worsening. The TMS is more time consuming to collect but enables a more accurate assessment of disease activity.