Association of normal nailfold end row loop numbers with a shorter duration of untreated disease in children with juvenile dermatomyositis.

OBJECTIVE To determine the association of normal numbers of end row loops (ERLs) in nailfold capillaries at the time of diagnosis of juvenile dermatomyositis (DM) with clinical findings in untreated children with the disease and to identify predictors of the development of decreased numbers of ERLs. METHODS Clinical and laboratory data from 80 untreated children with juvenile DM were collected. ERL numbers were recorded at the time of diagnosis and at 24 months and 36 months thereafter. The 12 children who had normal ERLs at diagnosis were compared with the remaining 68 children. Outcomes included the duration of untreated disease, the duration of treatment with immunosuppressive medications, family medical history, Disease Activity Score (DAS) for juvenile DM, creatinine phosphokinase level, aldolase level, absolute number of CD3-CD56+/16+ natural killer cells, and von Willebrand factor antigen level. Cross-sectional and longitudinal analyses were performed. RESULTS At diagnosis, children with normal ERLs had a shorter duration of untreated disease (P = 0.03) and a lower skin DAS (P = 0.045). Over time, an increased likelihood of having decreased numbers of ERLs was associated with a longer duration of untreated disease and with a higher skin DAS. CONCLUSION The presence of a normal number of ERLs in juvenile DM appears to be associated with a shorter duration of symptoms and may be a useful indicator of disease chronicity in the newly diagnosed child. Normal ERLs is also associated with a lower skin DAS. The lack of association between normal ERLs and other variables indicates that normal findings on nailfold capillaroscopy should not be used as justification to delay immunosuppressive therapy in children with typical symptoms of juvenile DM.

[1]  B. Feldman,et al.  Predicting the course of juvenile dermatomyositis: significance of early clinical and laboratory features. , 2008, Arthritis and rheumatism.

[2]  Yi-Wen Chen,et al.  Duration of chronic inflammation alters gene expression in muscle from untreated girls with juvenile dermatomyositis , 2008, BMC Immunology.

[3]  B. Feldman,et al.  Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood , 2008, The Lancet.

[4]  L. Pachman,et al.  Pharmacokinetic study of oral prednisolone compared with intravenous methylprednisolone in patients with juvenile dermatomyositis. , 2008, Arthritis and rheumatism.

[5]  A. Paller,et al.  Persistent association of nailfold capillaroscopy changes and skin involvement over thirty-six months with duration of untreated disease in patients with juvenile dermatomyositis. , 2008, Arthritis and rheumatism.

[6]  B. Feldman,et al.  An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM). , 2006, Rheumatology.

[7]  A. Dyer,et al.  Duration of illness is an important variable for untreated children with juvenile dermatomyositis. , 2006, The Journal of pediatrics.

[8]  A. Dyer,et al.  History of infection before the onset of juvenile dermatomyositis: results from the National Institute of Arthritis and Musculoskeletal and Skin Diseases Research Registry. , 2005, Arthritis and rheumatism.

[9]  A. Dyer,et al.  Skin involvement in juvenile dermatomyositis is associated with loss of end row nailfold capillary loops. , 2004, The Journal of rheumatology.

[10]  P. Bacon,et al.  Nailfold capillary microscopy in healthy children and in childhood rheumatic diseases: a prospective single blind observational study , 2003, Annals of the rheumatic diseases.

[11]  R. Bode,et al.  Disease activity score for children with juvenile dermatomyositis: reliability and validity evidence. , 2003, Arthritis and rheumatism.

[12]  L. Pachman,et al.  Increased plasma thrombospondin-1 (TSP-1) levels are associated with the TNF alpha-308A allele in children with juvenile dermatomyositis. , 2002, Clinical immunology.

[13]  L. Pachman,et al.  TNFα-308A allele in juvenile dermatomyositis: Association with increased production of tumor necrosis factor α, disease duration, and pathologic calcifications , 2000 .

[14]  H. Maricq,et al.  Childhood dermatomyositis: serial microvascular studies. , 1989, Pediatrics.

[15]  H. Maricq,et al.  Serial changes in nailfold capillary morphology in childhood dermatomyositis. , 1983, Arthritis and rheumatism.

[16]  A. Bohan,et al.  Polymyositis and dermatomyositis (second of two parts). , 1975, The New England journal of medicine.

[17]  A. Bohan,et al.  Polymyositis and dermatomyositis (second of two parts). , 1975 .