Drooling in adults with dermatomyositis may signal advanced disease and a heightened risk for fatal complications, according to a case series and literature review published in Clinical Case Reports.
Researchers described three adults with severe dermatomyositis who developed marked dysphagia followed by drooling. All three patients experienced progressive disease despite intensive immunosuppressive therapy and ultimately died. Aspiration pneumonia occurred in each case, and sepsis was reported in some.
The investigators characterized drooling (sialorrhea) as an infrequently described feature of dermatomyositis that may reflect severe oropharyngeal muscle involvement. In the current series, patients required hospital readmission for worsening weakness, dysphagia, and persistent drooling. Their clinical courses were described as refractory despite treatment with high-dose corticosteroids and combination regimens that included agents such as azathioprine, intravenous immunoglobulin, hydroxychloroquine, and rituximab.
Dermatomyositis is a rare idiopathic inflammatory disease marked by proximal muscle weakness and characteristic skin findings. Dysphagia is a recognized complication when oropharyngeal muscles are affected. The researchers noted that drooling has been rarely documented in this context, identifying only six previously reported cases in the literature, all described as responsive to treatment. In contrast, the patients in the present series had progressive disease despite aggressive therapy.
Muscle biopsy findings in the three cases demonstrated severe pathologic changes, including perifascicular atrophy, marked muscle fiber atrophy, fibrosis, and in one case, end-stage muscle damage with fat replacement. The report also emphasized that when clinical suspicion remains high despite initially normal diagnostic findings, repeat muscle biopsy may be warranted.
In their key clinical message, the researchers stated that drooling in dermatomyositis can signal advanced disease with extensive oropharyngeal involvement and an increased risk of life-threatening complications. They recommended comprehensive myositis antibody panels to help guide prognosis and treatment intensity, along with aggressive combination therapy using a step-down approach and careful infection prevention. They added that a definitive direct link between drooling and mortality risk requires further study.
The researchers reported no conflicts of interest.
Source: Clinical Case Reports
