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6 Juvenile dermatomyositis
  2. NJ OWEN,
  3. S BOSE,
  5. CM OWENS,
  1. Department of Rheumatology and Radiology, Great Ormond Street Hospital, London, UK
    1. F CORONA,
    2. V OTELLI,
    4. A GRASSI,
    6. M BARDARE
    1. Paediatric Rheumatology Centre, I Paediatric Clinic, University of Milan, Italy
      1. G CHÉDEVILLE,
      2. P QUARTIER,
      3. M DEBRÉ,
      4. R MOUY,
      5. AM PRIEUR
      1. Rhumatopédiatrie, Hôpital des Enfants Malades, Paris, France
        1. CD ROSE,
        3. B ATHREYA
        1. duPont Hospital for Children, Wilmington Delaware, Thomas Jefferson University, Philadelphia Pennsylvania, USA

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          6.1 A preliminary comparative study of high frequency muscle ultrasound and magnetic resonance imaging in 7 patients with juvenile dermatomyositis

          Objective—To assess the value of ultrasound scans (USS) of the thigh in juvenile dermatomyositis (JDM) in comparison with magnetic resonance imaging (MRI). To correlate these findings with clinicopathological indicators of disease activity.

          Methods—7 white patients with JDM (4 male, 3 female) had a high resolution muscle ultrasound examination. Mean age at onset was 6.8 years (range 1–15), 5/7 were scanned at diagnosis (within 6 months of diagnosis) and at follow up, 2/7 were scanned owing to chronic disease at 6 years from disease onset. Results of these scans were correlated with clinical, biochemical and histological markers. USS were correlated with MRI of the thigh in all patients.

          Results—All 5 USS performed at diagnosis were abnormal. Most showed diffuse symmetrical hyperechogenicity affecting mainly the adductors, vasti and rectus femoris muscles. In several cases there was swelling of the affected muscles. These muscles were shown to be hyperaemic by colour Doppler imaging. After initial steroid treatment all USS showed a remarkable reduction in muscle echogenicity, muscle swelling, and vascularity. Two USS showed marked wasting and fatty infiltration after treatment and a major increase in subcutaneous adipose tissue. In one patient a clear progression from echogenic hyperaemic muscle through reduced echogenicity to fatty atrophy was seen over serial scans. One patient had calcinosis on follow up. The USS findings correlated well with MRI and biochemical markers of disease activity.

          Conclusion—Musculoskeletal ultrasound is a safe, inexpensive, non-invasive means of supporting the clinical diagnosis of JDM and of monitoring disease progression, without needing sedation, which is often needed for an MRI in young children. Ultrasound findings appear to correlate well with other markers of disease activity.

          6.2 FK-506 in the treatment of unresponsive juvenile dermatomyositis

          Objective—To describe the efficacy and safety of FK-506 (tacrolimus) in three children with dermatomyositis.

          Patients and methods—Patient 1: 7 y 8 m old girl, with no resolution of the skin abnormalities, strength 4/5 after 2.33 years of disease, previously treated with high dose steroids, methotrexate and cyclosporin A (CyA).

          Patient 2: 11 y 7 m old girl, with impressive lack of strength (2/5) after 1.16 years of treatment (high dose steroids and CyA); hypertension as CyA side effect.

          Patient 3: 11 y 11 m old boy, longlasting disease (7.83 years), muscle strength 4/5 and persistence of severe skin lesions

          In all three FK-506 was started at 0.1 mg/kg/day and levels (5–10 ng/ml) were monitored every three months. Parent's consent was mandatory to start treatment.

          Results—All three patients responded extremely well to FK-506. Muscle strength was considered normal (5/5) for all three after one year of treatment. Skin lesions disappeared in patients 1 and 2. In patient 3, residual atrophic skin areas are still visible. Patients 1 and 2 have stopped steroid treatment, while patient 3 went from 10 mg/day to 5 mg/48 h. In the only patient with raised lactate dehydrogenase at the beginning of treatment (patient 2), levels normalised after 6 months. Neither major nor minor side effects occurred

          Conclusion—FK-506 might be a good alternative for patients with dermatomyositis who do not respond to or are intolerant of high dose steroids or CyA.

          6.3 Familial occurrence of juvenile dermatomyositis

          A case of familial dermatomyositis (DM) is described, in which all affected family members had onset of symptoms during infancy. Father and daughter have mild dermatological stigmata of DM, with no muscle involvement. The son has juvenile dermatomyositis with biochemical and histological evidence of myositis at diagnosis and florid cutaneous manifestations. No HLA or DQ associations were noted in this family. Familial DM is rare, early onset of disease in infancy in all affected family members has not previously been described.

          6.4 Risk factors at onset in juvenile dermatomyositis: correlation with prognosis

          We describe 33 patients (18 female, 15 male) affected with dermatomyositis, with a mean age at onset 7 y 1 m (range 2 y 3 m–17 y 5 m) and mean period before the diagnosis 4 m (range 7 d–2 y 1 m).

          The evaluation of both signs and symptoms and the muscle enzymes values at the onset allows a subdivision into 2 groups: 16 patients had an acute onset (skin rash, high fever, remarkable muscle weakness, nasal speech, dysphagia, skin vasculitis, respiratory failure, and large increase of skeletal muscle enzymes in serum), and 17 patients had an insidious onset (skin rash, arthralgia, less marked muscle weakness). Six out of 16 patients with acute onset treated with pulses of methylprednisolone, plasmapheresis, IV immunoglobulin, and different immunosuppressive treatment (methotrexate, cyclosporin, cyclophosphamide), also required intensive care treatment, and 1 required surgery for intestinal vasculitis. Three out of six patients who required intensive care treatment died, two for respiratory failure and one for systemic vasculitis.

          In contrast, all the patients with an insidious onset improved with methylprednisolone alone.

          In conclusion, early and aggressive treatment is mandatory in the high risk subtype to obtain a more rapid clinical and enzymatic improvement and a shorter course.

          6.5 Juvenile dermatomyositis (JDM) in 23 patients

          We retrospectively studied 17 girls and 9 boys with JDM followed up at the paediatric rheumatology unit since 1982. Mean (SD) age at disease onset was 5.8 (2.9) years (range 1–11.8), at diagnosis 6.4 (2.7) years (2.4–12). Disease duration at diagnosis was 7 (8) months (0–27). All patients but one had typical skin lesions, 18 had proximal muscle weakness. Other clinical symptoms at diagnosis were fever and weight loss (14), muscle pain (10), arthralgia/arthritis (9), dysphagia (3), calcinosis (2), tendon retraction (1), tachycardia (1), hepatomegaly (1). All patients but one were treated with oral prednisone. Other drugs were methylprednisolone pulses (11), cyclosporin A (6), methotrexate (6), hydroxychloroquine (5), IV immunoglobulin (5), non-steroidal anti-inflammatory drugs (2). Nineteen patients responded well to the initial treatment. A mean of 2.6 relapses (range 1–5) occurred in 15 patients either during treatment (30 relapses) or after treatment was discontinued (9 relapses). Clinical symptoms seen during follow up were persisting skin lesions (26), muscle weakness (21), muscle pain (14), arthralgia/arthritis (12), calcinosis (12), dysphagia (8), fever or weight loss (6), tendon retraction (7), cutaneous staphylococcus infection (7), clinical lipodystrophy (2), and pancreatitis (1). At the last visit 8 patients had stopped treatment without sequelae, 6 had stopped treatment with sequelae, 12 were still receiving treatment after a mean disease course duration of 3.5, 7.9, and 4.3 years respectively.

          6.6 Treatment of juvenile dermatomyositis (JDM) with high dose oral steroids or with steroid pulses and low dose oral steroids

          Objective—Treatment with oral steroids may control inflammation in the large majority of children with JDM, but is followed by Cushingoid syndrome.

          Methods—Prospective randomised open study of oral high dose steroids (prednisone 2 mg/kg for 4 weeks followed by gradual decrease) versus repeated pulses (IV methylprednisolone 20 mg/kg for 3 days with decreasing frequency of pulses) plus low dose oral steroids (prednisone 0.2 mg/kg). Patients were evaluated after 8 weeks for initial response and for a further 40 weeks for relapse.

          Results—24 patients (18 girls) were enrolled, median age 7 years, range 3–15. 13 patients received steroid pulse treatment, 11 high dose oral steroids. All patients were considered responders. 19/24 patients were followed up for ⩾48 weeks, the remaining patients for 8–38 weeks (3 × pulse, 2 × oral). 7/24 patients had a relapse (3 × pulse, 4 × oral). Cushingoid syndrome was found in 9/11 patients receiving oral steroids and in 3/13 receiving pulse steroids.

          Conclusion—Treatment of JDM with pulse steroids plus low dose oral steroids may be as effective as high dose oral steroids, but the frequency of steroid adverse effects may be diminished.

          Participants—Biedermann/Berlin, Eisenberg/Herford, Häfner/Garmisch, Häβler/München, Haller/Schweiz, Heidemann/Augsburg, Horneff/Halle, Keitzer/Berlin, Lehmann/Bad Bramstedt, Leipold/Erlangen, Leupold/Dresden, Möbius/Cottbus, Queisser/Ludwigshafen, Quietzsch/Plauen, Weiβbarth-Riedel/Hamburg.

          6.7 Low dose short term corticosteroid treatment for “amyopathic” childhood dermatomyositis (DM): the role of MRI

          In 1975 Boham and Peter proposed rash and 1 to 3 out of 4 possible myositis-defining criteria (weakness, increased muscle enzyme, positive biopsy, EMG) for classification of dermatomyositis (DM). In the largest series published to date by LM Pachmanet al all children depicted at least one myositis-defining criterion and all presented at least one abnormal muscle enzyme.7-1 Classically, patients who do not fulfil the myositis-defining criteria are called amyopathic.

          In this report we present 4 children who presented with typical DM rash, normal muscle enzymes, and no history of weakness. The presence on magnetic resonance imaging (MRI) of a typical abnormal signal intensity on fat suppressed fat spin echo T2 weighted image circumvented the need for invasive studies (EMG/biopsy) by rendering us confident of the diagnosis. We treated these children with a short course of prednisone (3 months total) at a low dose (0.5–1 mg/kg for 4–6 weeks followed by tapering) because of our conviction that they had mild disease. Follow up MRI showed resolution at 1–2 months of treatment and follow up at 6–24 months from onset disclosed no recurrence and no calcinosis. All patients are receiving hydroxychloroquine and none is receiving corticosteroids. Toxicity was minimum.

          Conclusions—1 MRI should be considered a candidate non-invasive diagnostic criterion for DM; 2 perhaps, milder disease may be treated less aggressively than classic DM.


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