Background/Purpose: Previously it was understood that all Myositis in childhood was Juvenile Dermatomyositis. More recently Myositis specific antibodies have been discovered and these appear to be linked to specific clinical phenotypes. One of these called anti SRP+ve has been linked to a very severe form of Myositis and this review of 3 patients presents the clinical description and treatment, focusing on the physiotherapy, of these patients.

Methods: It is believed that anti SRP+ve antibodies are only present in 5% of children with Myositis. In the cohort of patients within the paediatric rheumatology service only 3/250 patients were found to be anti SRP+ve. The clinical case notes were reviewed and the results are presented here. The main focus of this review is during the initial presentation and treatment with specific focus upon the physiotherapy, and does not address long term outcome. 

Results: Despite the rare nature of this Myositis and the relatively new recognition of this condition there have been 3 patients diagnosis within the last 1year. All 3 patients have been female aged 11-14 at time of onset. Their initial onset was rapid onset of extremely severe muscle weakness but with minimal skin involvement. CMAS score at onset ranged between 0 – 6 and MMT 8 16 – 23 and they all presented with multiple joint restrictions due to inflamed joints and muscle tightness.  They have all had such severe swallowing difficulties that they were naso-gastrically fed for many weeks. The medical treatment consisted of multiple immunosuppressant agents including high dose steroids, IVIG, Methotrexate, Cyclophosphomide and Rituximab. Intensive physiotherapy was required for all and this ensured the maximum recovery of the muscle strength, muscle patterning and functional improvement. The physiotherapy was started from the initial presentation and was provided twice a day. The initial focus was on regaining range of movement and active-assisted muscle strengthening. The exercises were progressed as they were able to anti-gravity work and then into resisted work.  The recovery for all 3 was very slow initially and required an extended hospital admission of many months. Two of the patients were discharged with an MMT8 of 76/80 and were able to manage full time school, walking independently and managing all activities of daily living, but did require a wheel chair for longer distances. The 3^rd remains in hospital at this point but is improving significantly.

Conclusion: Anti SRP+ve Myositis in children is a very rare and severe disease presenting with profound muscle weakness and requires both intensive physiotherapy and high dose immune-suppressants. Though long-term outcome is not known rehabilitation to restore independent function is possible.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/anti-srpve-myositis-in-childhood-presentation-and-physiotherapy-treatment-of-this-rare-childhood-myositis/