(Kiosk 6) Comprehensive Rehabilitation in Refractory Juvenile Polymyositis: A Case Report

Case Diagnosis: Juvenile polymyositis

Case Description or Program Description: We present the case of a 16 year-old-male with biopsy confirmed polymyositis. After a mild antecedent COVID19 diagnosis, he presented with bilateral hip and knee weakness that progressed to being bedridden with severe diffuse muscle weakness. His initial treatment was refractory to outpatient physical therapy and multiple rounds of intravenous steroids, intravenous immune globulin (IVIG), methotrexate, and rituximab over 6 months. After transition to a more specialized level of care with appropriate rheumatological evaluation, his treatment was individualized with multiple rounds of pulse steroids, IVIG, methotrexate, and the addition of cyclophosphamide and infliximab while participating in intensive therapies before he began to have significant functional improvements over the course of 5 months.

Setting: Pediatric inpatient rehabilitation unit

Assessment/Results: During the course of his 5 month stay in inpatient rehabilitation, he continued to undergo reassessment of disease burden and re-evaluation of functional improvement leading to adjustments in his regimen by rheumatology. The patient went from being bedridden and requiring total assistance to modified independence with mobility and activities of daily living.

Discussion (relevance): Juvenile polymyositis (JPM) is a rare entity, accounting for 3-6% of childhood idiopathic inflammatory myopathies. The progressive weakness that occurs with JPM frequently leads to inpatient hospital admission for work up and initial treatment. Additionally, prolonged weakness with JPM is known to decrease endurance, functional capacity, and the development of contractures – for which intensive rehabilitation with physical and occupational therapy is most beneficial.

Conclusions: This case reflects the importance of not only understanding the pathophysiology of JPM as it can lead to devastating consequences in pediatric patients when inadequately treated, but also providing comprehensive interdisciplinary care during inpatient rehabilitation in cases of severe refractory polymyositis to improve function and quality of life.