Reliability of LoSCAT score for activity and tissue damage assessment in a large cohort of patients with Juvenile Localized Scleroderma [PDF]
Objectives To assess reliability of the two indexes of Localized Scleroderma Cutaneous Assessment Tool (LoSCAT), the modified Localized Scleroderma Skin Severity Index (mLoSSI) and the Localized Scleroderma Skin Damage Index (LoSDI), when applied by ...
Anna Agazzi +4 more
doaj +2 more sources
Calcinosis Cutis and Delayed-Onset Myositis in a Case of Suspected Localized Scleroderma: A Diagnostic and Therapeutic Challenge. [PDF]
ABSTRACT A 16‐year‐old girl presenting with calcinosis cutis and localized scleroderma subsequently developed delayed‐onset idiopathic inflammatory myopathy five years after initial skin involvement. Despite the absence of typical dermatomyositis features and negative myositis‐specific antibodies, whole‐body MRI revealed extensive subclinical muscle ...
Marrani E +5 more
europepmc +2 more sources
Nailfold capillary phenotypes distinguish juvenile myositis subtypes and associate with disease activity. [PDF]
Nailfold capillaroscopy is a non‐invasive imaging tool that can aid in the diagnosis and monitoring of juvenile myositis. Branched nailfold capillary morphology is more common in juvenile myositis patients, particularly those positive for TIF1γ+ autoantibodies. Juvenile myositis patients with more active disease demonstrate increased microhaemorrhages,
McClellan N +7 more
europepmc +2 more sources
JAK inhibitors for the treatment of juvenile localized scleroderma: a case report and literature review [PDF]
Background Juvenile localized scleroderma (JLS) is a chronic autoimmune and fibrosing disease that can cause significant damage and impairments. Currently, management of JLS is challenging due to the paucity of antifibrotic drugs. In patients with severe
Rongjun Zheng +3 more
doaj +2 more sources
Developing comparative effectiveness studies for a rare, understudied pediatric disease: lessons learned from the CARRA juvenile localized scleroderma consensus treatment plan pilot study [PDF]
Background We designed and initiated a pilot comparative effectiveness study for juvenile localized scleroderma (jLS), for which there is limited evidence on best therapy.
Suzanne C. Li +17 more
doaj +2 more sources
Nailfold Videocapillaroscopy in Pediatric Rheumatology: Established Roles, Emerging Applications, and Future Perspectives [PDF]
Nailfold videocapillaroscopy (NVC) provides direct, non-invasive access to the peripheral microcirculation and has become a central tool in adult rheumatology. Its primary clinical value lies in the evaluation of patients presenting with signs of Raynaud
Kübra Öztürk +4 more
doaj +2 more sources
Long-term methotrexate efficacy in juvenile localized scleroderma [PDF]
Giuliotto S +5 more
doaj +5 more sources
Sarcopenia in juvenile localized scleroderma: new insights on deep involvement
Juvenile localized scleroderma (JLS) is a rare chronic autoimmune disease which can also affect bones and muscles. Nevertheless, muscle loss was not previously investigated in patients with JLS. Thus, the aim of this study was to retrospectively evaluate deep involvement and assess and quantify sarcopenia in JLS patients using magnetic resonance ...
Silvia Karem Janet Flores Quispe +7 more
openaire +3 more sources
Revealing Microvascular Involvement in Pediatric Localized Scleroderma Through Nailfold Capillaroscopy [PDF]
Background/Objectives: Juvenile localized scleroderma (jLoS) is a chronic inflammatory disorder with skin and subcutaneous tissue involvement. Microvascular alterations are thought to contribute to its pathogenesis.
Sema Nur Taşkın +5 more
doaj +2 more sources
Localized scleroderma in a newborn (case report) [PDF]
N.I. Akhmina1, P.S. Utkin2, M.P. Shalatonin3, Zh.L. Chabaidze1, A.A. Dement’ev1, A.L. Zaplatnikov1 1Russian Medical Academy of Continuous Professional Education, Moscow, Russian Federation 2Children’s Infectious Clinical Hospital
N.I. Akhmina +5 more
doaj +1 more source

