Pulmonary fibrosis and myositis in a child with anti-Jo-1 antibody.
This is the first report of a child with anti-Jo-1 antibody. At 10, she had a chronic cough, wheezing, dyspnea on exertion, a combined obstructive and restrictive ventilatory defect, and a decreased diffusion capacity compatible with alveolar fibrosis (lung biopsy). By age 12, she developed arthralgias, malar rash, frontal alopecia, Raynaud's phenomenon, and was seropositive for antinuclear antibodies, (SSA)Ro, Jo-1 and rheumatoid factor. At 16, symptomatic inflammatory myositis (elevated creatinine kinase, muscle biopsy) was documented with persistent anti-Jo-1. Her pulmonary symptoms have progressed despite appropriate therapy. Although her digits have become sausage shaped with swan neck deformities, joint pain is not frequent.
Chmiel
J F
JF
Department of Pediatrics, Northwestern University Medical School, Chicago, IL, USA.
Wessel
H U
HU
Targoff
I N
IN
Pachman
L M
LM
D000293:Adolescent; D000328:Adult; D000906:Antibodies / Q000032:analysis*; D002648:Child; D005260:Female; D006643:Histidine-tRNA Ligase / Q000276:immunology*; D006801:Humans; D009220:Myositis / Q000150:complications / Q000276:immunology*; D011658:Pulmonary Fibrosis / Q000150:complications / Q000276:immunology*; D012120:Respiration Disorders / Q000209:etiology
D000906:Antibodies; D006643:Histidine-tRNA Ligase