024. Anti-KU antibodies in two paediatric cases with juvenile dermatomyositis-scleroderma overlap: why is it important to know?

Khaosut, Parichat; Castagno, Simone; Aluko, Abimbola; Al-Obaidi, Muthana

doi:10.1093/rheumatology/kex356.008

Cited by 2 publications

(1 citation statement)

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“…Adult patients with these antibodies have been described with skin thickening, articular disease, Raynaud phenomenon, interstitial lung disease (ILD), and myositis 1 . However, descriptions of the clinical features and outcome of children with this antibody are limited, with only six previously reported pediatric cases of anti‐Ku antibody‐positive overlap syndrome 2‐6 . Including our patient, all seven children had myositis.…”

Section: Discussionmentioning

confidence: 88%

Anti‐Ku antibody‐positive systemic sclerosis‐polymyositis overlap syndrome in an adolescent

Loo

Nocton

Harmelink

et al. 2020

Pediatric Dermatology

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Systemic sclerosis-polymyositis overlap syndrome is rare in children. Anti-PM/Scl is the most common autoantibody associated with this syndrome. We present a case of systemic sclerosis-polymyositis overlap syndrome in a child with isolated anti-Ku antibodies, an uncommon antibody associated with this rare syndrome. | 961 Pediatric Dermatology BRIEF REPORT the clinical features and outcome of children with this antibody are limited, with only six previously reported pediatric cases of anti-Ku antibody-positive overlap syndrome. 2-6 Including our patient, all seven children had myositis. Five also had SSc, three had articular disease, two had SLE, two had Raynaud phenomenon, one had overlap of SSc, SLE, and myositis, and one developed ILD. Additional skin findings included 2 with generalized morphea, 2 with malar erythema, 1 with pernio-like erythema of the hands and feet, 1 with facial discoid lesions, and 1 with heliotrope rash of the eyelids. These findings suggest that the clinical manifestations in children and adults with anti-Ku antibody are similar. Anti-Ku-associated SSc-myositis overlap syndrome is rare in children. Our report underscores the importance of testing for anti-Ku antibodies specifically in patients with myositis and SSc suggestive of this syndrome.

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Section: Discussionmentioning

confidence: 88%