Validation of a score tool for measurement of histological severity in juvenile dermatomyositis and association with clinical severity of disease

Hemlata Varsani, Susan C. Charman, Charles K. Li, Suely K.N. Marie, Anthony A. Amato, Brenda Banwell, Kevin E. Bove, Andrea M. Corse, Alison M. Emslie-Smith, Thomas S. Jacques, Ingrid E. Lundberg, Carlo Minetti, Inger Nennesmo, Elisabeth J. Rushing, Adriana M.E. Sallum, Caroline Sewry, Clarissa A. Pilkington, Janice L. Holton, Lucy R. Wedderburn, Liza McCannIan Roberts, Eileen Baildam, Louise Hanna, Olivia Lloyd, Phil Riley, Ann McGovern, Clive Ryder, Janis Scott, Sue Wyatt, Gillian Jackson, Tania Amin, Mark Wood, Vanessa Van Rooyen, Joyce Davidson, Janet Gardner-Medwin, Neil Martin, Sue Ferguson, Liz Waxman, Mark Friswell, Helen Foster, Alison Swift, Sharmila Jandial, Vicky Stevenson, Debbie Wade, Ethan Sen, Eve Smith, Lisa Qiao, Helen Venning, Rangaraj Satyapal, Elizabeth Stretton, Mary Jordan, Kate Armon, Joe Ellis-Gage, Holly Roper, Clarissa Pilkington, N. Hasson, Sue Maillard, Elizabeth Halkon, Virginia Brown, Audrey Juggins, Sally Smith, Sian Lunt, Elli Enayat, Miss Laura Beard, Miss Laura Kassoumeri, Miss Katie Arnold, Kevin Murray, John Ioannou, Linda Suffield

Research output: Contribution to journalArticlepeer-review

31 Scopus citations


Objectives: To study muscle biopsy tissue from patients with juvenile dermatomyositis (JDM) in order to test the reliability of a score tool designed to quantify the severity of histological abnormalities when applied to biceps humeri in addition to quadriceps femoris. Additionally, to evaluate whether elements of the tool correlate with clinical measures of disease severity.

Methods: 55 patients with JDM with muscle biopsy tissue and clinical data available were included. Biopsy samples (33 quadriceps, 22 biceps) were prepared and stained using standardised protocols. A Latin square design was used by the International Juvenile Dermatomyositis Biopsy Consensus Group to score cases using our previously published score tool. Reliability was assessed by intraclass correlation coefficient (ICC) and scorer agreement (α) by assessing variation in scorers' ratings. Scores from the most reliable tool items correlated with clinical measures of disease activity at the time of biopsy. Results Inter- and intraobserver agreement was good or high for many tool items, including overall assessment of severity using a Visual Analogue Scale. The tool functioned equally well on biceps and quadriceps samples. A modified tool using the most reliable score items showed good correlation with measures of disease activity.

Conclusions: The JDM biopsy score tool has high interand intraobserver agreement and can be used on both biceps and quadriceps muscle tissue. Importantly, the modified tool correlates well with clinical measures of disease activity. We propose that standardised assessment of muscle biopsy tissue should be considered in diagnostic investigation and clinical trials in JDM.

Original languageEnglish (US)
Pages (from-to)204-210
Number of pages7
JournalAnnals of the rheumatic diseases
Issue number1
StatePublished - Jan 1 2015

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Immunology
  • Biochemistry, Genetics and Molecular Biology(all)


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