Review article
Ear, nose, and throat manifestations of sarcoidosis

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Epidemiology and etiology

The epidemiology and etiology of sarcoidosis are undoubtedly interwoven because the disease has a clear predilection for certain ethnic and racial groups and certain countries. The etiology probably combines a genetic predisposition with an environmental trigger. Prevalence as high as 50 per 100,000 has been reported in Scandinavian countries and in the African-American population in the United States [2]. Central Europe and the United States have a higher incidence than Central and South

Clinical features

Sarcoidosis is a multiorgan disorder with a variable clinical picture and a tendency to wax and wane. Three distinct patterns exist: asymptomatic sarcoidosis, nonspecific constitutional symptoms, and organ-specific symptoms. The true incidence of asymptomatic sarcoidosis is unknown because these patients are incidentally identified when pulmonary findings are noted on a chest radiograph. It is estimated that 30% to 50% of patients with sarcoidosis are asymptomatic at the time of diagnosis [14],

Diagnosis

Three criteria must be met for a diagnosis of sarcoidosis: (1) compatible clinical and radiographic findings, (2) histologic finding of noncaseating granulomas, and (3) exclusion of other diseases with similar findings or local sarcoid reaction. Patient history, physical examination, and chest radiograph findings typically initiate the work up for sarcoidosis. Tissue for diagnosis is typically obtained by transbronchial lung biopsy through a fiberoptic bronchocope with a diagnostic yield of 80%

Treatment

Systemic corticosteroids remain the mainstay in the treatment of sarcoidosis. Because of the variability in disease progression and high frequency of spontaneous remission, indication for treatment remains controversial. Clearly, life-threatening organ involvement or vision-threatening ocular involvement calls for treatment with systemic corticosteroids. Topical or inhaled corticosteroids can be used in patients with skin disease, nasal involvement, uveitis, or airway disease to reduce

Ear, nose, and throat manifestations of sarcoidosis

Otolaryngologic manifestations of sarcoidosis occur in 10% to 15% of patients and, rarely, may be the presenting disorder [30]. Although no system or organ of the head and neck is entirely spared, cervical adenopathy, parotid swelling, and facial nerve palsy are the most common findings. In patients with otolaryngologic complaints with a previous history of sarcoidosis, however remote, or other signs of the disease such as pulmonary complaints, neuropathies–especially facial nerve

Cervical lymphatics and salivary glands

Cervical adenopathy is the most common head and neck manifestation of sarcoidosis and was present in 48% of patients in one series [31]. Although these lesions may not necessarily require treatment, the otolaryngologist may be called on to obtain a biopsy of the lesion to confirm the diagnosis. Biopsy by fine-needle aspiration has a high yield in making the diagnosis [32] although an open biopsy may be necessary. An alternative is ultrasound-guided fine-needle aspiration of nonpalpable cervical

Nose and sinuses

Sinonasal manifestations in patients with sarcoidosis occur with an incidence of 1% to 4% [36], [37]. Typical symptoms are nonspecific for sinonasal sarcoidosis and include nasal obstruction, postnasal drip, headache, and recurrent sinus infection. Sinonasal disease is not often the presenting symptom of the disease, because patients often have concurrent pulmonary disease and lupus pernio. Physical examination often reveals dry, friable mucosal lesions involving the septum and inferior

Ear and skull base

Sarcoidosis can, rarely, affect numerous regions of the ear including the helix and external auditory canal, middle ear and temporal bone, and the neuro-otologic structures housed within the temporal bone. Because otologic involvement may mimic a number of other diseases of the ear, sarcoidosis will probably not be considered prospectively and may be recognized only on biopsy.

Raised, nodular skin lesions of sarcoidosis have been noted on the auricular helix [41]. If found in isolation,

Larynx and pharynx

Sarcoidosis affects the larynx in 0.5% to 8.3% of patients [52], [53] and may occur in the absence of other manifestations of the disease. The supraglottic structures, especially the epiglottis, are typically involved, probably because of the rich lymphatics in this area. Initially, laryngeal involvement may be asymptomatic and relatively benign. As the disease progresses, dysphagia, dyspnea, globus sensation, and voice changes ensue. In severe cases, airway obstruction may require a

Summary

Sarcoidosis rarely affects the head and neck and represents a diagnostic challenge to the otolaryngologist. The signs and symptoms of sarcoidosis in this area are not specific and can mimic much more common disorders. Biopsy is usually necessary to make the diagnosis. Appropriate evaluations and referrals should be made once there is suspicion of sarcoidosis. Steroids remain the mainstay of the therapeutic options; however, newer agents are being used more frequently. Long-term treatment and

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