(Kiosk 1) Early Rehabilitation of Myositis: A Case Report of Necrotizing Myopathy

Case Diagnosis: Necrotizing Myopathy

Case Description: 68-year-old-female with no medical history presented with generalized weakness of three weeks with associated voice change, dysphagia, early satiety, unintentional weight loss, and shortness of breath. She endorsed difficulty feeding, combing hair, and ascending stairs. Physical exam demonstrated decreased inspiratory effort and severe proximal weakness. Admission labs were significant for CPK 917 U/L and ANA 1:1280. Neurology and rheumatology conducted broad work-ups for autoimmune vs paraneoplastic disease; most tests resulted when the patient was in rehabilitation.

Discussion: Necrotizing myopathy is a myositis characterized by necrosis and degeneration of muscle fibers. It often presents as symmetric proximal weakness, muscle atrophy, and bulbar symptoms. The etiology is unknown but may be immune-mediated, paraneoplastic, viral-related (including COVID-19), or drug-mediated. Although the exact diagnosis type of myositis did not result until the end of this patient’s rehabilitation course, the addition of multiorgan involvement (esophageal and lung manifestations) made this patient an appropriate candidate for acute rehabilitation.

Setting: Inpatient Rehabilitation Facility

Assessment/Results: Steroids and IVIG led to some improvement in patient’s weakness. The myositis panel was unremarkable including JO1, PL7, PL12, MI2, KU, EJ, OJ, and SRP antibodies. CT chest was significant for ground glass opacities. EGD was performed showing delayed gastric emptying, and gastric biopsies were taken. Muscle biopsy was performed; results showed myonecrosis and the patient was started on rituximab. During the nine days of rehabilitation, respiratory status was monitored with negative inspiratory force measurements. Dysphagia improved with speech/swallow therapy, allowing for safe consumption of soft, bite-sized diet. By discharge, she was independent with all activities of daily living and ambulating 150 feet with straight cane.

Conclusion: Necrotizing myopathy is a rare and aggressive type of myopathy that presents with progressive muscle weakness and possibly multi-organ involvement. This case illustrates the importance of early multidisciplinary rehabilitation of myositis.