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Autoimmune swallowing disorders.

Although rare, autoimmune disorders should be considered in the differential diagnosis for patients presenting with complaints of swallowing dysfunction. Autoimmune diseases may cause swallowing problems by causing painful oral and oropharyngeal lesions, jaw pain, inadequate lubrication of the food bolus, esophageal dysmotility, or esophageal compression. Recognition of an autoimmune etiology will direct further management that is often coordinated with rheumatologists, employing a combination of systemic corticosteroids and/or immunosuppressive agents. (1)

Painful oropharyngeal lesions occur in granulomatosis with polyangiitis or Wegener granulomatosis, and autoimmune bullous diseases such as cicatricial pemphigoid and pemphigus vulgaris. Patients with Wegener disease may present with hyperplastic gingivitis and ulcerations on the hard and soft palate. Diagnosis of Wegener disease is established with biopsy, revealing necrotizing vasculitis in a setting of granulomatous inflammation and the presence of serum antineutrophil cytoplasmic antibodies.

Painful oral and oropharyngeal lesions often precede any cutaneous involvement in pemphigus vulgaris. Tense, fluid-filled blisters most commonly appear on the buccal mucosa; these rupture, leaving painful, slow-healing, irregular ulcerations. Biopsy of margins of the blisters reveals keratinocytes floating in blister fluid because of splitting in the suprabasilar layer, and direct immunofluorescence shows intercellular immunoglobulin G (IgG) deposits in the epidermis.

Less painful oral lesions appear in cicatricial pemphigold. Smooth-bordered erosions of gingival and buccal mucosa are also common in pemphigoid. Esophageal erosions lead to stricture formation. Direct immunofluorescence of perilesional mucosa reveals linear deposits of IgG and complement in the subepidermal basement membrane zone. (2)

Autoimmune disorders causing jaw pain lead to larger food boluses and increased dysphagia. Giant cell arteritis (temporal arteritis) causes jaw claudication due to involvement of branches of the external carotid artery, such as the ascending pharyngeal artery and internal maxillary artery. Jaw claudication results in improper mastication and larger food boluses, with increased dysphagia. Likewise, rheumatoid arthritis (RA) causes dysphagia by producing jaw pain by affecting the temporomandibular joint (TMJ), one of the last joints affected by RA. Clinically, ankylosis, crepitus, and TMJ pain can be elicited on jaw motion. Malocclusion can develop at late stages. (2)

Swallowing problems may also arise because of inadequate lubrication of food bolus. Sjogren syndrome is characterized by xerostomia and keratoconjunctivitis caused by autoimmune injury of glandular epithelial cells. Decreased tearing may be measured by a Schirmer test I. Autoantibodies, such as anti-SSA and anti-SSB, are not specific to Sjogren. Confirmation of the diagnosis is made by histopathologic evaluation of a labial minor salivary gland biopsy that shows lymphocytic infiltration. Affected patients complain of food becoming stuck in their oropharynx. Decreased salivary flow has been correlated with a subjective sensation of dysphagia, and some studies have shown prolonged pharyngeal transit times. Secondary Sjogren syndrome occurs with other autoimmune diseases such as RA and causes dysphagia in a similar manner. (2)

Dysphagia resulting from esophageal hypomotility is prominent in several autoimmune disorders, such as systemic sclerosis, RA, and sarcoidosis. The prototypic disorder in this group is systemic sclerosis or scleroderma that is characterized by infammation, fibrosis, and progressive degeneration of skin, vasculature, and internal organs such as heart, lung, kidneys, and the gastrointestinal tract.

The CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) is one clinical subtype of limited cutaneous systemic sclerosis. The presence of taut, thin skin causing contractures; Raynaud phenomenon; esophageal dysmotility; restrictive lung disease; and the autoantibodies anticentromere and anti-Scl-70 is used to further confirm and distinguish different subtypes of systemic sclerosis.

Esophageal hypomotility is caused by the involvement of the lower two-thirds of the esophagus in 20 to 95% of patients with scleroderma. Esophageal manometry demonstrates peristaltic waves with reduced amplitude occurring in the inferior two-thirds of the esophagus while sparing the striated upper one-third. Additionally, the lower esophageal sphincter has de creased contractility and reduced resting pressure that results in gastroesophageal reflux and esophagitis, causing further dysphagia. (2)

Autoimmune disorders causing dysphagia due to esophageal strictures or extrinsic esophageal compression include Wegener disease, cicatricial pemphigoid, and sarcoidosis, although this occurrence is rare in Wegener disease and cicatricial pemphigoid.

Sarcoidosis is a systemic, chronic, granulomatous disease affecting most commonly the lungs and lymphatic system. Its diagnosis most often is established by flexible bronchoscopy employing transbronchial sampling, or sometimes by cervical lymph node biopsy. Histopathology demonstrates noncaseating granulomas that, in the absence of other granulomatous clinical entities, help to establish the diagnosis. Serum angiotensin-converting enzyme levels are not extremely sensitive or specific but may confirm the diagnosis. Cutaneous involvement of the face, such as lupus pernio or parotid enlargement, occurs in some patients.

Mediastinal adenopathy in sarcoidosis may give rise to extrinsic esophageal compression, causing dysphagia. Additionally, granulomatous infiltration of the esophageal wall may causelong esophageal strictures. Manometrymay also demonstrate devated cricopharyngeal and lower esophageal sphincter pressures. (2)

Various autoimmune disorders may cause dysphagia and odynophagia. Therefore, otolaryngologists seeing patients with these complaints need to maintain a broad differential that includes autoimmune disorders. Understanding the mechanism of dysphagia and odynophagia in autoimmune diseases helps the treating physician to recognize characteristic clinical entities and initiate the appropriate diagnostic workup. Rheumatology consultation will benefit in coordinating effective treatment.

[Editor's note: This Guest Editorial has been adapted with permission from its publication in the Fall 2012 issue of Soundings, the Pennsylvania Academy of Otolaryngology-Head and Neck Surgery's newsletter.]

References

(1.) Davidson A, Diamond B. General features of autoimmune disease. In: Mackay IR, Rose NR (eds). The Autoimmune Diseases. 4th ed. St. Louis:: Elsevier Academic Press; 2006:25-36.

(2.) Soliman AMS, Buchinsky FJ. Pathophysiology of swallowing disorders: Autoimmune disorders. In: Murry T, Carrau RL (eds). Comprehensive Management of Swallowing Disorders. San Diego: Plura Publishing, Inc.; 1999:199-210.

Mursalin M. Anis, MD, PhD

Ahmed M.S. Soliman, MD

Department of Otolaryngology-Head and Neck Surgery Temple University School of Medicine Philadelphia
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Title Annotation:GUEST EDITORIAL
Author:Anis, Mursalin M.; Soliman, Ahmed M.S.
Publication:Ear, Nose and Throat Journal
Article Type:Editorial
Date:Dec 1, 2013
Words:946
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