Cricopharyngeal obstruction in inflammatory myopathy (polymyositis/dermatomyositis). Report of three cases and review of the literature

Abstract

Three adults, 2 of whom had polymyositis and 1 with dermatomyositis, developed dysphagia during the course of their illness. Results of esophageal manometry supplemented with esophageal radiography indicated the presence of cricopharyngeal achalasia. Because of the severity of this disorder, which is associated with aspiration of esophageal contents into the airways, surgery to divide the cricopharyngeal musculature was performed in 2 patients, giving complete relief of their symptoms. Prednisone dosage was not increased to treat this condition since it arose not from weakness but from obstruction. A biopsy specimen taken from 1 patient demonstrated inflammatory changes in the obstructing muscle. A review of these 3 patients and 3 previously reported cases indicates that cricopharyngeal obstruction can be a dominant cause of dysphagia in patients with myositis. The recognition of this entity is important in the management of patients with myositis because: 1 it has serious and potentially life‐threatening implications; and 2 in certain cases, it can be effectively treated with surgery.

Manifestações otorrinolaringológicas nas doenças reumáticas auto-imunes

“…O músculo cricoaritenóide posterior é o único músculo capaz de manter as cordas vocais em adução e quando ele é comprometido as cordas vocais se juntam e pode ocorrer obstrução das vias aéreas. Disfagia orofaríngea pode favorecer complicação por aspiração do conteúdo esofágico para dentro das vias aéreas (39) . Estreitamento da abertura oral, rigidez do palato mole, laringe e mucosa oral, são responsáveis por disfagia orofaríngea na esclerose eistêmica (40) .…”

A dysphagia study in patients with sporadic inclusion body myositis (s-IBM)

“…However, the nature of the swallowing impairment in s-IBM and other inflammatory myopathies has not been well characterized. Previous studies suggest that improper contraction of the pharyngeal muscles or cricopharyngeal muscle dysfunction may result in functional obstruction due to dysphagia [3][4][5][6].…”

Cricopharyngeal Muscle Hypertrophy Associated with Florid Myositis

“…The literature on the histopathology of cricopharyngeal muscle biopsies suggests that cricopharyngeal muscle hypertrophy is either localized [6±10] or part of a systemic disease [3,5,11±18] (Tables 1 and 2). The systemic diseases reported so far include dermatomyositis [12], polymyositis [13,14], inclusion body myositis [15±18], generalized granulomatous disease [11], and neuromuscular degeneration [3]. Concomitant scleroderma was observed in one case, but there were no histological signs of scleroderma in the cricopharyngeal muscle biopsy [9].…”

“…Dietz (1980) 1 Diuse ®brosis; minimal evidence of round cell in®ltration. Kagen (1985) 1 Areas of necrosis; in¯ammatory cell in®ltration; increase in connective tisue. Wintzen (1988) 1 Fibrous tissue and fat with a few muscle ®bers, which were completely normal.…”