Anti-Signal Recognition Particle-Positive Immune-Mediated Necrotizing Myopathy with Acute Inpatient Myocarditis and Pharyngeal Involvement in a Young Adult: A Case Highlighting the Importance of Early Biologic Escalation – A Case Report
Abstract
Abstract Background Immune-mediated necrotizing myopathy (IMNM) is a rare and clinically severe subtype of idiopathic inflammatory myopathy, characterised by predominant myofibre necrosis with a paucity of lymphocytic infiltration. Anti-signal recognition particle (anti-SRP)-positive IMNM represents a particularly aggressive disease phenotype, frequently resistant to corticosteroid monotherapy and complicated by extramuscular manifestations, of which cardiac involvement remains under-recognised. Case Presentation: We report a 26-year-old Indian woman who presented with a two-month history of symmetric, insidious-onset proximal limb weakness, one month of truncal weakness, and ten days of dysphagia. Investigations revealed markedly elevated creatine kinase (peak 7,112 IU/L), anti-SRP antibody positivity on a myositis-specific line-blot panel, concurrent strong positivity for anti-Ro52 and anti-SS-A antibodies without fulfilling criteria for Sjögren's syndrome, a myopathic electromyogram, and muscle biopsy findings of necrotising autoimmune myopathy. Concurrent corticosteroid and methotrexate therapy produced clinical deterioration. On the eighth inpatient day, probable immune-mediated myocarditis supervened, manifest as sinus bradycardia, anterior T-wave inversions, and a positive cardiac troponin T (cTnT) in the absence of wall-motion abnormality on echocardiography. Sequential immunosuppressive escalation encompassing pulse methylprednisolone, intravenous immunoglobulin…
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