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Juvenile localised scleroderma management -International consensus recommendations

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speciality medical dialogues

Juvenile Localized Scleroderma (JLS) is a rare Autoimmune disorder that may cause severe aesthetic sequelae and functional disability. JLS, often termed ‘morphea’ in the dermatology literature, refers to a number of different conditions characterized by skin thickening with increased collagen deposition. JLS includes several subtypes, including plaque morphea (PM), linear scleroderma (LS) and the en coup de sabre (ECDS) type, which affects the face and head.

An international Expert group has released consensus-based recommendations on the management of juvenile localised scleroderma (JLS).In 2012, a European project called Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases.

This came after an international committee of 15 experts in paediatric rheumatology was established to develop consensus-based recommendations for JLS. European League Against Rheumatism (EULAR) standard operating procedures for developing best practice were used. Ten experts were part of the SHARE consortium; five other experts were asked to take part in the project due to their consolidate clinical experience in the management of JLS.

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Following are the major recommendations:

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Recommendations regarding diagnosis and assessment

All children with suspected localised scleroderma should be referred to a specialised paediatric rheumatology center.

  • LoSSI, which is part of LoSCAT, is a good clinical instrument to assess activity and severity in JLS lesions and is highly recommended in clinical practice.
  • LoSDI, which is part of LoSCAT, is a good clinical instrument to assess damage in JLS and is highly recommended in clinical practice.
  • Infrared thermography can be used to assess the activity of the lesions in JLS, but skin atrophy can give false-positive results.
  • A specialised US imaging, using standardized assessment and colour Doppler, may be a useful tool for assessing disease activity, the extent of JLS and response to treatment.
  • All patients with JLS at diagnosis and during follow-up should be carefully evaluated with a complete joint examination, including the temporomandibular joint.
  • MRI can be considered a useful tool to assess musculoskeletal involvement in JLS, especially when the lesion crosses the joint.
  • It is highly recommended that all patients with JLS involving face and head, with or without signs of neurological involvement, have an MRI of the head at the time of the diagnosis.
  • All patients with JLS involving face and head should undergo an orthodontic and maxillofacial evaluation at diagnosis and during follow-up.
  • Ophthalmological assessment, including screening for uveitis, is recommended at diagnosis for every patient with JLS, especially in those with skin lesions on the face and scalp.
  • Ophthalmological follow-up, including screening for uveitis, should be considered for every patient with JLS, especially in those with skin lesions on the face and scalp.

Recommendations regarding treatment

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  • Systemic corticosteroids may be useful in the active inflammatory phase of JLS. At the same time as starting systemic corticosteroids, MTX or an alternative DMARD should be started.
  • All patients with active, potentially disfiguring or disabling forms of JLS should be treated with oral or subcutaneous methotrexate at 15 mg/m²/week.
  • If acceptable clinical improvement is achieved, methotrexate should be maintained for at least 12 months before tapering.
  • Mycophenolate mofetil may be used to treat severe JLS or MTX-refractory or MTX-intolerant patients.
  • Medium-dose UVA1 phototherapy may be used to improve skin softness in isolated (circumscribed) morphoea lesions.
  • Topical imiquimod may be used to decrease skin thickening of circumscribed morphoea.

For more details click on the link: http://dx.doi.org/10.1136/annrheumdis-2018-214697




Source: self

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