Intravenous immunoglobulin for dysphagia of inclusion body myositis

Sporadic inclusion body myositis (s-IBM) is the most common muscle disease in patients older than age 50, characterized by an insidious onset, progressive muscle weakness, and resistance to traditional treatment.1 The esophagus is the most common organ involved, reported in 25 to 40% of s-IBM cases.2 Esophageal disorders are a major cause of morbidity and mortality in s-IBM, and may be responsible of infectious aspiration pneumonia, septicemia, and cachexia. Only cricopharyngeal myotomy has been validated for the treatment of severe dysphagia during s-IBM.3 We describe four patients with s-IBM and life-threatening esophageal disorders, whose swallowing disorders dramatically improved after administration of IV immunoglobulin (IVIg) alone or in association with corticosteroids (table). Our results suggest IVIg should be considered as a choice treatment in such cases. ### Patient 1 A 59-year-old man was hospitalized in July 1995 with severe dysphagia and infectious aspiration pneumonia. He had experienced progressive muscle weakness for 4 years. Creatine kinase (CK) levels were 320 UI/L. Muscle biopsies revealed characteristic s-IBM on optic and electron microscopy (EM). Esophageal manometry revealed low pressure in the upper …