Research abstracts

New-onset juvenile dermatomyositis (JDM): Increased time to diagnosis and therapy in minority children; documentation by physician report.

L.M. Pachman, E. Mendez, Yi-lin Chiu, A. Chung, J.R.Hayford, R. Ramsey Goldman, R. Lipton; Depts. of Medicine and Pediatrics, Northwestern University Medical School and Dept of Epidemiology, The UIC School of Public Health, Chicago.

Little is known about the association of a spectrum of epidemiological characteristics with the diagnosis and therapy of JDM. A 5-year NIAMS-sponsored National New-Onset JDM registry, (established in 1995) includes 103 children (drawn from 37 states) with the following demographic features: female (n=73), male (n=30); ratio F:M 2.4:1; Caucasian (n=75), Hispanic (n=15), African American (n=7), Native American (n=1), Other (n=4). All had muscle weakness (MW) and/or rash (R) at presentation with 88 children have both MW and R, 2 with (R) only and 13 with MW initially. Only 3 (2.5%) had calcinosis in contrast to 23% in a previous study (Ped Res 104: 1997). Median Interval (Months): First Symptom (1st Sx) to Diagnosis (Dx) or Steroid Therapy (Rx)

1st Sx to Dx to PO Rx to IV Rx NonHispanic White (NHW) 2.7* 2.8 2.6 NonHispanic Black (NHB) 5.6* 5.5 6.4 Hispanic (H) 2.5 3.6 4.1 *p=.05 Total: 2.9 3.6 3.3

In comparison to H or NHW children, NHB children had a significantly longer time to both diagnosis and therapy, confirming our earlier data (Ped Res 104:310A, 1997). This delay may be related to difficulty in detection of the rash in skin of darker pigment, and/or to barriers to obtaining adequate medical care. The initial route of steroid therapy was IV in NHW as contrasted to the H and NHB groups, in which steroids PO were given first, followed by the IV route. These data suggest that ethnic background may affect medical practice.

Newly diagnosed children with juvenile dermatomyositis (JDM): Clinical characteristics on physician chart review; an interim analysis of 101 patients. L.M. Pachman*, E. Mendez*, A. Chung*, R. Lipton^, R.Ramsey-Goldman# and A.Dyer+ From the departments of Pediatrics*, Medicine#, Preventive Medicine+ Northwestern University Medical School, and Epidemiology, Univ of IL, Chicago^.

Referring physicians reviewed their records of the initial visit of 101 children from 37 states enrolled in a NIAMS National New-Onset JDM Registry. Antecedent illness (within three months of the appearance of the JDM rash and/or weakness) was present in 44%. Respiratory symptoms predominated in 24%: (cough, 3%, sore throat 11%, rhinorrhea 4%, headache 4%, earache 4%, cold or flu 7%); only 4% were group A Streptococcal positive on culture. Constitutional symptoms (44%) included fever 18% and fatigue 23%. Gastrointestinal complaints were elicited in 9%. Complaints of pain were frequent: muscle 45%, joint 22% and abdominal 17%. Hepatosplenomegaly was observed in 4% and lymphadenopathy in 6%. Cutaneous signs were focal, 51%, as well as diffuse/generalized changes (43%), displayed on the hands (87%), Gottron's papules (83%), face (73%), elbows (72%), knees (71%) and trunk (26%). Vasculitis was frequent: nail fold capillary changes (67%), eyelid vessel dilation (42%) or thrombosis (14%); involvement of the palate (10%) or other areas (15%). Arthritis was present in 23% of children, lasting all day and localized to the hands/wrists or knees. Weakness was manifest in the neck flexors (70%), proximal muscles both upper and lower (76%) with positive Gower's sign (65%), inability to clear scapula (67%), dysphagia (15%) and nasal quality of speech (23%). There was an 18-23% loss of range of motion in the hands/wrists, elbows, shoulders and ankles; back motion was reduced in 5%. Functional status was limited: 58% had moderate to severe compromise. 3% had calcinosis. We conclude: 1) antecedent symptoms, primarily respiratory, often precede the definite signs of JDM; 2) the classic features of JDM include vascular changes and are often associated with pain as well as moderate to severe functional limitations; and 3) calcinosis at the initial physician visit is less common than reported by parents in previous studies. Supported by NIAMS #NO-AR4-2219.