From the Guidelines
Bulbar symptoms in dermatomyositis are rare but can include dysphagia and dysphonia 1. These symptoms are attributed to the involvement of proximal striated muscle, particularly the cricopharyngeus muscle.
- Dysphagia is a prominent clinical feature, with 30% to 80% of patients with myositis, including dermatomyositis, experiencing swallowing difficulties 1.
- Dysphonia, or voice difficulties, can also occur due to the involvement of the muscles used for speech. Management of bulbar symptoms typically involves high-dose corticosteroids, such as prednisone 1-2 mg/kg/day, and may require additional immunosuppressive agents like azathioprine 1-2 mg/kg/day or methotrexate 10-20 mg/week 1.
- In severe cases, intravenous immunoglobulin (IVIG) 2 g/kg divided over 2-5 days may be considered. Patients with significant bulbar involvement may require close monitoring and supportive care, including speech and language therapy, and in some cases, temporary feeding tube placement or respiratory support 1.
From the Research
Bulbar Symptoms in Dermatomyositis
Bulbar symptoms in dermatomyositis, an inflammatory muscle disease, include:
- Dysphagia (swallowing disorders) due to inflammation of muscles involved in swallowing 2, 3, 4
- Dysphonia (voice disorders) resulting from weakened esophageal and respiratory muscle 3
- Dyspnea (breathing difficulties) caused by weakened respiratory muscle 3
Clinical Characteristics and Risk Factors
The incidence of dysphagia in dermatomyositis is approximately 18.8%, with predominantly pharyngeal phase impairments 2. Risk factors for dysphagia include:
- Advanced age 2
- Presence of underlying malignancy 2
- Methylprednisolone use 2
- Intravenous immunoglobulin (IVIg) use 2
- Systemic corticosteroid prescription 2
Other Clinical Manifestations
Other clinical manifestations that can occur in patients with dermatomyositis include: