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10 Scleroderma
  1. F CORONA,
  1. Paediatric Rheumatology Centre, I Paediatric Clinic, University of Milan, Italy
    1. A MARTINI,
    2. R MACCARIO,
    3. A RAVELLI,
    4. D MONTAGNA,
    6. F BONETTI,
    7. S VIOLA,
    8. M ZECCA,
    9. P DE STEFANO,
    1. Dipartimento di Scienze Pediatriche, Università di Pavia, IRCCS Policlinico S Matteo, Pavia, Italy

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      10.1 Thermography in juvenile localised scleroderma assessment

      Aim—To evaluate the clinical use of infrared thermography in localised scleroderma (LS) in disease activity assessment and management.

      Methods—We retrospectively reviewed thermal images of children with LS obtained between 1993–2000. Thermographs were included only when a contemporary detailed clinical description of the lesion(s) was available. Lesions were classified as “active” (new or extended) or “quiescent” according to clinical description (colour, skin texture, measurements). Thermographs were considered positive when the affected area temperature was >0.5°C higher than the surrounding skin or the opposite site. Two clinicians (GM and KJM), blinded to the clinical description and thermography report, reviewed the thermal images independently. Full agreement in scoring was achieved in 86%, and discordant results were rescored by mutual examination.

      Results—40 patients were included in the study (26 F, 14 M). The most common diagnosis was a combination of morphoea and linear scleroderma (M+LiS, 14 patients), followed by isolated LiS (11 patients), en coup de sabre (6 patients), and M (5 patients). 68 lesions were examined: 33 affecting the legs, 16 the arms, 10 the face/scalp, and 9 the trunk. We reviewed 130 separate thermal images, 34 lesions having multiple thermographic examinations.

      There was complete agreement between the clinical description and thermography in all new lesions (table 1-1). Of the clinically inactive lesions positive on thermography, most were “old” lesions with the presence of severe atrophy and subcutaneous fat loss.

      View this table:
      Table 1-1

      Conclusions—Infrared thermography is a potentially reliable tool for assessing the activity of LS lesions in conjunction with clinical activity, particularly for clinically suspicious new and extending lesions. Further evaluation is needed to determine whether thermography can predict future progression of lesions, particularly those which are equivocal clinically.

      10.2 An unusual type of scleroderma with neurological disease

      We describe the case of a 14 year old boy who started with a linear scleroderma and presented later a neurological disease.

      At the age of 11 years the boy developed a marked hypotrophy and hypoplasy of the right forearm apparently owing to prolonged immobilisation. Over a period of few months the disease progressed to affect the skin and muscles of the right arm, hand, and fingers with a sclerotic evolution. All laboratory and radiological examinations (x rays and magnetic resonance imaging (MRI) of the arms, x rays of the chest and of the gastrointestinal tract), spirometry, and a nailfold capillaroscopy were normal except for a high antinuclear antibody titre (1/1280).

      Borrelia infection (IgM and IgG low titre) was suspected and the patient began treatment with penicillin 15 000 000 U/d for 10 days, without any improvement.

      Two years later he had uveitis and seizures (abnormal EEG, cranium asymmetry at x ray examination, microvascular ischaemic encephalopathy at MRI, negative cerebral arteriography).

      Recently, facial hemiatrophy with a contralateral hemisyndrome is evident and skin and muscle atrophy have worsened.

      Whether this form is an evolution of linear systemic scleroderma or is linear scleroderma with neurological disease, which has already been described, cannot be stated for sure.

      10.3 Efficacy and safety of autologous peripheral blood stem cell transplantation in three children with systemic sclerosis and progressive pulmonary disease

      Background—Autologous peripheral blood stem cell (PBSC) transplantation has been proposed as a treatment for autoimmune diseases with fatal prognosis.

      Patients—Three children with systemic sclerosis since the age of 4 (patient 1), 7 (patient 2), and 6 (patient 3) years had a severe disease course, with progressive cutaneous, articular, and pulmonary disease despite immunosuppressive treatment. Because pulmonary fibrosis, once established, is progressive, at the age of 11, 10, and 9 years, respectively, they discontinued all drugs and received an autologous PBSC transplantation.

      Transplantation procedure—After obtaining approval from the local ethical committee and parents' informed consent the three children were given CY (4 g/m2) followed by G-CSF (10 mg/kg/d) to allow the collection of PBSC. The conditioning regimen before transplantation comprised CY at a dose of 50 mg/kg from day 5 to day 2 and the monoclonal antibody Campath-1g at a dose of 10 mg/d for 2 days. The post-transplantation period was uneventful in all patients.

      Results—Two patients (patients 1 and 2) had a significant clinical benefit at 40 and 16 months, respectively, after transplantation, with improvement in growth rate and general wellbeing, and general skin softening. In both of them high resolution pulmonary computed tomography (HRCT) scan did not show any progression of fibrotic changes. At variance, in 1 patient (patient 3) the transplantation procedure did not affect significantly the skin disease, and a repeated pulmonary HRCT after 12 months showed a further progression of pulmonary fibrosis.

      Conclusion—Autologous PBSC transplantation associated with Campath-1g treatment was safe and well tolerated in all patients and resulted in marked and sustained clinical improvement in two of the three patients.

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