Validation of LoSQI in JLS
Research type
Research Study
Full title
Cross-cultural adaptation and validation of the Localised Scleroderma Quality of Life Instrument (LoSQI) in juvenile localised scleroderma (JLS): a multicentre study of the PRES scleroderma working party in collaboration with members of the CARRA scleroderma working group
IRAS ID
288439
Contact name
Clare Pain
Contact email
Sponsor organisation
Alder Hey Childrens NHS Foundation Trust
Duration of Study in the UK
3 years, 0 months, 1 days
Research summary
Juvenile localised scleroderma (JLS) is characterised by chronic inflammation within the skin and tissues leading to fibrosis. It can occur at any age and is rare. It can be associated with significant complications including joint contractures, limb length discrepancy, and facial atrophy, that impact quality of life. Scleroderma means “hard skin.” Children with localised scleroderma often have involvement of the tissues below the skin, including muscle and bone. Besides the skin hardening, there can be changes in skin colour and texture, and the underlying tissues may fail to grow normally.
Patient reported outcomes (PRO) are not well established and are not part of routine clinical care in many centres. The Localised Scleroderma Quality of Life Instrument (LoSQI) has been developed in partnership with patients and families to capture aspects of disease which may not be expressed well within current broad quality of life measures.
This study has two workstreams: the first will test the translation of the LoSQI (translated from English to the child’s first language) and the second workstream will compare the LoSQI with other questionnaires and measures. This part of the study will include other patient-reported measures which capture how scleroderma affects each child. These measures are not specific to scleroderma and have been tested in children with other conditions. If they work well in children with scleroderma, we will be able to compare how scleroderma affects children compared to other conditions.
This study is being led by Dr Clare Pain and the UK sponsor site is Alder Hey Children's NHS Foundation Trust. However, as well as UK sites, there will be multiple non-UK sites and the study will be facilitated outside of the UK by PRINTO (the Pediatric Rheumatology International Trials Organization). This will allow us to translate the LoSQI measure into multiple languages.
REC name
West Midlands - Black Country Research Ethics Committee
REC reference
21/WM/0037
Date of REC Opinion
2 Mar 2021
REC opinion
Further Information Favourable Opinion