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Rheumatology Advance Access originally published online on March 27, 2006
Rheumatology 2006 45(10):1255-1260; doi:10.1093/rheumatology/kel099
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© The Author 2006. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland)—clinical characteristics of children recruited within the first 5 yr

L. J. McCann1, A. D. Juggins2, S. M. Maillard2, L. R. Wedderburn2, J. E. Davidson3, K. J. Murray4, C. A. Pilkington2, on behalf of the Juvenile Dermatomyositis Research Group

1Royal Liverpool NHS Trust, Alder Hey, Liverpool, 2Rheumatology Unit, Institute of Child Health, University College London, and Great Ormond Street Hospital NHS Trust, London, UK, 3Royal Hospital for Sick Children, Glasgow, UK and 4Princess Margaret Hospital, Perth, Western Australia.

Correspondence to: Dr C. A. Pilkington, Rheumatology Unit, Institute of Child Health, 30 Guilford Street, London, WC1N 1EH, UK. E-mail: c.pilkington{at}ich.ucl.ac.uk


   Abstract
Top
 Abstract
Introduction
 Patients and methods
 Discussion
 Appendix A
 Acknowledgements
 References
 
Objectives. To identify epidemiological, clinical and laboratory characteristics of juvenile dermatomyositis (JDM) in a national multi-centre cohort of patients, and to review recent changes in the understanding of management and prognosis in the light of these data.

Methods. All children with idiopathic inflammatory myositis recruited to the Juvenile Dermatomyositis National Registry and Repository (UK and Ireland) were included. Features at presentation, and later in disease, were assessed and evaluated. A total of 63 out of 175 children with a new diagnosis of myositis were recruited at the time of diagnosis and followed prospectively. Out of the 175 children, 122 diagnosed prior to 2000 were recruited retrospectively, with subsequent data collected prospectively.

Results. One patient died (0.7%), which is equivalent to one death per 465 patient years. Data were available at the time of analysis on 151 registered patients. The most common presenting features were characteristic rash, weakness, tiredness, Gottron's patches and myalgia. Muscle biopsy, magnetic resonance imaging and muscle enzymes were frequently, but not always, abnormal. Muscle enzymes and erythrocyte sedimentation rate were not useful markers of disease activity.

Conclusions. The JDM National Registry and Repository captures data on a significant cohort of children with inflammatory myositis. The current study reports the largest European cohort of children with dermatomyositis to date. This powerful resource will help improve our understanding of this rare disease. Prospective data collection will allow a fuller analysis of poor prognostic features, impact of therapy, and variable outcome of childhood myositis.

KEY WORDS: Juvenile, Dermatomyositis, Myositis, Inflammatory, Weakness.


   Introduction
Top
 Abstract
 Introduction
Patients and methods
 Discussion
 Appendix A
 Acknowledgements
 References
 
Juvenile dermatomyositis (JDM) is a rare inflammatory multi-system disease with a reported incidence of 0.8–4.1 per million children per year [1–4]. Outcomes are variable, making individual prognosis difficult to predict. Clinical characteristics of JDM have been reviewed retrospectively in the USA, Canada and Brazil [5–10]. Over the last 5 yr, there has been increasing interest in multi-centre research into aetiology and optimal management of JDM. In order to facilitate research, a National Registry and Repository for Juvenile Dermatomyositis in the UK and Ireland was established in July 2000.

The aim of this article is to describe epidemiological, clinical and laboratory characteristics of JDM in a national multi-centre cohort of patients. This allows review of recent changes in diagnosis, management and prognosis of patients. We present here, data on the first 151 cases of children recruited to the National Registry and Repository (UK and Ireland).


   Patients and methods
Top
 Abstract
 Introduction
 Patients and methods
Discussion
 Appendix A
 Acknowledgements
 References
 
Patient selection
Between late 2000 and mid-2005, 181 patients were approached from 9 UK centres. Patients were recruited consecutively on visits to paediatric rheumatology departments, with an aim of capturing all JDM patients seen by paediatric rheumatologists in the participating centres in UK and Ireland. Children were included if they had a diagnosis of definite or probable JDM or polymyositis; dermatomyositis or polymyositis overlap with scleroderma, polyarthritis, lupus, mixed connective-tissue disorder (provided myositis was the predominant clinical feature); or a diagnosis of focal, eosinophilic, orbital, inclusion body or sarcoid myositis, or other idiopathic inflammatory myopathy. The onset of diagnosis had to be at 16 yr of age or under. The only exclusion criterion was entry onto another registry.

Diagnosis of inflammatory myopathy was made at the discretion of the principal investigator at each centre, based on clinical features supported by investigation results. Two patients declined to participate for personal reasons. Four patients were withdrawn during the diagnostic work-up period after the revision of diagnosis, leaving 175 patients.

Data collection
Following written parental consent and patient assent, serial clinical data were collected using standardized proformas (available on request). Biological specimens including cells, serum, DNA, and muscle or skin biopsies were also collected. Children diagnosed prior to 2000 were recruited retrospectively, with an initial data proforma describing signs and symptoms up until recruitment, and subsequent forms collecting data prospectively. Data collected every 3–6 months included clinical history, examination findings and laboratory results. Muscle strength testing by Manual Muscle Test (MMT) [11] and Childhood Myositis Assessment Scale (CMAS) [12] was also carried out, as well as Childhood Health Assessment Questionnaire (CHAQ) [13], and Childhood Health Questionnaire (CHQ) [14]. Evidence of disease activity was noted by the presence of an active rash, reduced CMAS score, muscle weakness demonstrated on MMT, or other symptoms and signs such as dysphagia or skin ulceration. Data were stored, using ‘linked anonymous’ codes in a central database. An independent steering committee controls access to clinical data and biological repository. Multi-centre Research Ethics Committee Approval was obtained.

Results
Of the 175 children registered, sufficient data were available on 151 at the time of analysis. These included 120 children with a diagnosis of definite or probable JDM according to Bohan and Peter criteria [15], and 31 children with other diagnoses as shown in Table 1. Of these, >1 yr follow-up data were available for 117 children ranging from 1 to 15 yr from diagnosis. A total of 81 patients were recruited retrospectively after diagnosis, 36 patients were recruited at the time of diagnosis, or within 6 months of the onset of symptoms, and followed prospectively for up to 5 yr. This group underwent a sub-analysis looking at change in features over time.


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  		TABLE 1. Diagnoses of children included in the analysis

 
Patient characteristics
The childhood population of Great Britain (excluding Ireland) totalled 14.8 million in 2001 [16]. Ethnic classification was 90% White, 2.4% Black, 5.3% Asian, 1.9% mixed race and 0.4% other [16]. Within the cohort described here, 126 children were White (83%), 12 Black (8%), 10 Asian (7%) and three of mixed race (2%). There was a male:female ratio of 1:2.2. Mean age at diagnosis was 7.7 yr (S.D. 3.85) and median of 7 yr (range 1–16 yr). Age distribution for males and females is shown in Fig. 1.


Figure 1
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  		FIG. 1. Age distribution at the onset of myositis symptoms in 151 children recruited to the UK and Ireland National Registry and Repository. Legend {square}: Female; {blacksquare}: Male.

 
Time from the start of illness to diagnosis ranged from 1–118 months (mean of 8.5 months, median of 3 months). There was a suggestion of seasonality of onset with 46 patients (30%) presenting in spring and 45 (30%) in winter, compared with 25 (17%) presenting in summer and 35 (23%) in autumn. This did not reach statistical significance (P = 0.053). Investigations performed at the time of diagnosis in this cohort are shown in Table 2.


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  		TABLE 2. Diagnostic investigations carried out and percentage of abnormal investigations

 
Clinical features at diagnosis and over time
Clinical features at presentation are shown in Table 3. Eighteen patients (12%) did not have a characteristic rash or Gottron's patches. Of these, 11 subsequently developed dermatological features. Seven patients (5%) had no rash over a 1–7 yr follow-up period, with diagnoses of polymyositis (3), focal myositis (3) and undifferentiated connective tissue disease (1).


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  		TABLE 3. Clinical features at presentation in this cohort of patients

 
Twenty-seven patients (18%) had no documented weakness at presentation. Of these, 22 subsequently had weakness recorded. Five patients (3%) had no weakness noted during a follow-up period of 3 months to 7 yr. Four of these patients were recruited retrospectively with a diagnosis of JDM (1), JDM and arthritis overlap (1), and focal myositis (2). One was recruited prospectively with a diagnosis of JDM without muscle weakness, but was followed only for 6 months.

Of the 117 patients with follow-up data for more than 1 yr, calcinosis was observed in a total of 14 patients (12%). Thirty-five developed ulceration (30%). One patient recruited to the Registry and Repository died. The cause of death was respiratory failure secondary to profound muscle weakness and repeated respiratory infections.

Medication use over time
Medication use over time was documented for all 151 children (Fig. 2). A total of 139 (92%) received steroids (oral prednisolone or intravenous methylprednisolone), and 105 (70%) were started on additional immunosuppressive medication. Of those on additional immunosuppressant, methotrexate was the drug of choice, used in 82 out of 105 (78%) patients. Combinations of other immunosuppressants were used in 64 out of 105 (61%) children, either synergistically with methotrexate (41/64 children, 64%) or alone (23/64 children, 36%). The presence of dyspnoea, dysphagia or dysphonia in 37 (25%) patients, or ulceration, seen in 34 (23%) patients, prompted aggressive treatment.


Figure 2
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  		FIG. 2. Use of immunosuppressant medication in children with inflammatory myositis in the UK and Ireland National Registry and Repository. PRED, prednisolone; MTX, methotrexate; CIC, ciclosporin; HCQ, hydroxychloroquine; IVIG, intravenous immunoglobulin; AZA, azathioprine; CYCLO, cyclophosphamide; INFLIX, infliximab; PLAS EX, plasma exchange.

 
Characteristics of the prospective cohort
A small cohort of 36 children had data collected prospectively from diagnosis. All of these children were given steroids. Thirteen out of 36 (36%) children were started on an immunosuppressant at diagnosis. Methotrexate was used in 11 of these children. Subsequently, four more children were given methotrexate, totalling 15 out of 36 (42%) children in the prospective group on methotrexate by 1 yr. At diagnosis, in addition to methotrexate, two children were started on cyclophosphamide, and one on intravenous immunoglobulin (IVIG). One child was started on azathioprine, and one on hydroxychloroquine. Figure 3 shows the 95% confidence interval for time to resolution of documented muscle weakness or characteristic rash in those children started on a disease-modifying drug (methotrexate, hydroxychloroquine, azathioprine or cyclophosphamide) at onset (n = 13) compared with those not started on disease-modifying medication at onset (n = 23).


Figure 3
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  		FIG. 3. 95% confidence interval for time to resolution (in months) of documented muscle weakness or characteristic skin rash in those children started on a disease-modifying drug (methotrexate, hydroxychloroquine, azathioprine or cyclophosphamide) at onset (n = 13) compared with those not started on disease-modifying medication at onset (n = 23).

 
Creatine kinase (CK) and lactic dehydrogenase (LDH) levels decreased rapidly and remained low during the illness, despite clinical evidence of ongoing disease activity. In many cases, erythrocyte sedimentation rate (ESR) and C-reactive protein were not useful markers of disease activity (data not shown).

Rash and Gottron's papules persisted in a significant number of patients 2–3 yr after the onset of symptoms (Table 4). Residual systemic disease activity, including myalgia, weakness and systemic features (Table 4), were apparent in this cohort several years after the onset of disease.


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  		TABLE 4. Number of patients with symptoms over a 5 yr period from a group of 36 patients followed prospectively from diagnosis

 

   Discussion
Top
 Abstract
 Introduction
 Patients and methods
 Discussion
Appendix A
 Acknowledgements
 References
 
This is the largest European cohort of patients collected to date. The female predominance (2.2:1), mean age of diagnosis (7.7 yr) and median time from symptom onset to diagnosis (3 months) were consistent with previous reports from the Western world [4, 5, 7–10, 17–19]. The increased incidence of myositis among patients of Black ethnic origin has been noted previously [1], although this may be influenced by referral centres based in large cities. The slight increased frequency of onset in winter and spring is similar to previous UK data [4]. Other studies report seasonal clustering in spring and summer [5, 7, 17], possibly reflective of viral infection patterns or sun-exposure as disease triggers.

In keeping with previous reports [4–7, 10, 18], the most common presenting features were characteristic skin manifestations (88%) and weakness (82%), although these were not universal. Of the 18 patients without rash at diagnosis, 11 developed rash later, with only 7 (5%) remaining adermatitic. Only 5 (3%) children had absence of muscle weakness over time, endorsing previous suggestions [7] that adermatitic and amyopathic dermatomyositis is rare.

The Bohan and Peter criteria [15] remain the only validated diagnostic standards to date despite change in clinical practice [4, 5, 7, 10, 20]. A recent international survey established that all responders use proximal muscle weakness and characteristic skin rash to diagnose JDM, together with elevated muscle enzymes, or a combination of other laboratory markers. Electromyogram (EMG) was used routinely in 55%, and magnetic resonance imaging (MRI) in 59%. [21] The working group survey agreed the need for revised criteria that were in line with current clinical practice and would include MRI. In our cohort, EMG was used in only 8% of patients, 36% had muscle biopsies performed and MRI was used in 68%. Further research is needed to validate the use of MRI, but it has shown benefit in the assessment of disease activity or damage, and as a quantitative measure of muscle inflammation [22, 23].

This study highlights that no single test is consistently abnormal in all cases, supported by previous data [4, 5, 7, 10, 20]. MRI was abnormal in 78 out of 102 (76%) patients. Normal results may reflect late presentation of disease or subtle changes that were not recognized due to the lack of standardization of methods and reporting. In this cohort, 49 out of 55 (89%) had an abnormal muscle biopsy. Data concerning immunological stains were unavailable, but may be relevant. For example, MHC class-1 up-regulation on muscle biopsies is considered a highly sensitive indicator of muscle damage [24]. Standardization of histological processing of biopsies is being looked at and might improve diagnostic information [32].

Within this cohort, 80% of those with muscle enzyme data available had elevation in muscle enzymes (adolase not routinely available) consistent with previous studies [4–7]. In patients with an auto-antibody profile documented, ANA was positive in 56% and anti-ENA (anti-Ro, anti-La, anti-Sm, anti-RNP, anti-Scl-70, anti-KV or anti-Jo-1) in 13%. Anti-Jo-1 was positive in only two patients, in harmony with other reports acknowledging its infrequency in childhood dermatomyositis [8, 25].

Management of JDM has become more intensive with recognition that early diagnosis and aggressive treatment can prevent mortality and future complications [5, 6, 26]. Previously, immunosuppressant medication was added when patients suffered unacceptable adverse effects, or failed to respond to steroids [8, 10, 18, 20, 27, 28]. A shift to use immunosuppressants earlier in disease course to reduce duration and cumulative dose of steroid treatment, and possibly achieve faster disease control, is suggested by the current series. Methotrexate was used in addition to steroid in 15 out of 36 (42%) children in the prospective group within 1 yr. This contrasts with only 9% of patients on methotrexate within 1 yr of diagnosis in a series described by Pachman et al. [5]. Within our cohort, decisions regarding medication use were made by the consultant in charge at individual centres, based on usual practice and severity of disease. After steroids, methotrexate was the drug of choice (82 out of 105, 78%), with cyclosporin chosen less commonly (32 out of 105, 30%) and often synergistically with methotrexate. Azathioprine and IVIG were used in some cases. Ten out of 105 (9.5%) children on an immunosuppressant were given cyclophosphamide, indicated for severe ulcerative or vasculitic presentations, interstitial lung disease, gastrointestinal pathology, or central nervous system involvement. Cyclophosphamide has demonstrated clinical benefit without evidence of short-term toxicity in severe or refractory JDM within the UK [29]. Hydroxychloroquine was used in 22 patients (21% of those on an immunosuppressant), and may be a beneficial adjunctive treatment for skin manifestations [30]. Its effects on fatigue, myalgia and arthralgia await confirmation in controlled trials.

The prognosis of JDM has improved considerably. Prior to corticosteroid use, death was expected in one-third of cases, and severe permanent physical disability in another third [17]. Subsequently, there has been a progressive decrease in mortality, and improved functional outcomes [6, 10, 27, 28]. Prognosis has been linked to early and intensive treatment, the presence of pharyngeal involvement and initial steroid responsiveness [6, 27]. Within the current study, only one patient (0.7%) died (recruited to the retrospective cohort when alive); equivalent to one death in 465 patient years. This may under-represent the true mortality figure as children may have died prior to recruitment to the Registry.

Over time, calcinosis was observed in a total of 14 patients (12%), in contrast to past series, where it was seen in 14–53% [5–8, 19, 26, 28]. The lower incidence of calcinosis perhaps suggests that early diagnosis and aggressive treatment can change the outcome of disease. This needs to be studied in larger prospective series.

Risk of late progression or long-term complications of JDM may be increased by delayed recognition or sub-optimal initial medical or physical therapy [5, 31]. However, even with rapid treatment, prospective follow-up suggests that inflammatory myositis may remain active for 2–3 yr before symptoms settle (Table 4). Early aggressive use of immunosuppressants may shorten the initial period of rash and weakness (Fig. 3), but this needs to be confirmed in larger prospective studies. Muscle enzymes and inflammatory markers alone are not a reliable tool to monitor disease activity. Preliminary results suggest that objective muscle strength scores (MMT and CMAS) may be useful in recording the presence of muscle weakness or myositis [22]. This current study is limited by CHAQ, CHQ, CMAS and MMT being unavailable on retrospective data prior to the Registry being established. Over time, with further prospective data collection, a formal analysis of reliability of these tools will be possible.

In summary, the UK and Ireland JDM National Registry and Repository provides a valuable cohort of children for long-term follow-up. Despite greater understanding of JDM over recent years, multi-centre studies with large numbers are needed to determine optimal management and treatment. Analysis of disease outcome correlated to features at presentation is not yet possible due to the limited amount of prospective data. With greater numbers of children followed over time, the registry should be able to address important questions such as long-term outcome, frequency of complications and relationship to treatment.

Formula


   Appendix A
Top
 Abstract
 Introduction
 Patients and methods
 Discussion
 Appendix A
Acknowledgements
 References
 
Local Research Coordinators and Principle Investigators who participated in recruitment of patients and data collection include: Mr Ian Roberts, The Royal Liverpool Children's Hospital, Alder Hey, Liverpool, and Booth Hall Children's Hospital, Manchester; Dr Eileen Baildam, Booth Hall Children's Hospital, Manchester; Mrs Janis Scott and Dr Clive Ryder, Birmingham Children's Hospital, Birmingham; Mrs Gillian Jackson and Dr Sue Wyatt, Leeds General Infirmary, Leeds; Ms Elizabeth Camp and Dr Janet-Gardner-Medwin, The Royal Hospital for Sick Children, Yorkhill, Glasgow; Mrs Alison Swift and Dr Mark Friswell, The Royal Victoria Infirmary, Newcastle; Mrs Elizabeth Hutchinson and Dr Helen Venning, Queens Medical Centre, Nottingham; Miss Virginia Brown, Juvenile Dermatomyositis Research Centre, London.


   Acknowledgements
Top
 Abstract
 Introduction
 Patients and methods
 Discussion
 Appendix A
 Acknowledgements
References
 
The Juvenile Dermatomyositis Research Group and Dr Liza McCann are funded by the Cathal Hayes Research Foundation.

The authors would like to thank all Local Research Coordinators and Principal Investigators, acknowledged in Appendix A. In addition, we are grateful to all medical and allied health-professional staff involved in the recruitment of patients and the collection of data.

The authors have declared no conflict of interests.


   References
Top
 Abstract
 Introduction
 Patients and methods
 Discussion
 Appendix A
 Acknowledgements
 References
 

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Submitted 19 October 2005; revised version accepted 21 February 2006.
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