Laryngeal Sarcoidosis
Laryngeal sarcoidosis is particularly rare, with a large cohort of patients with systemic sarcoidosis demonstrating an incidence of only 0.6% (14). As with other manifestations of sarcoidosis, age at presentation is typically from 20 – 40 years old (13).
The presenting complaint in the majority of patients relates to difficulty breathing and less commonly, dysphonia and cough. Pain is not usually a feature. Only a small proportion of patients complain of dysphagia and if so, it is rarely the sole symptom. Those with dysphagia inevitably modify their diet and some silently aspirate. Given the natural history and slow progression of this granulomatous disease, emergency department presentations are rare. Case reports of tracheostomy exist (20, 21), however most patients present with a gradual decline. Stridor is common but mild. Although symptom onset is gradual, the impact on quality of life is significant with a reduction in exercise capacity, compounding respiratory disease in affected young patients.
Endoscopic examination typically reveals supraglottic swelling and deformity, with the epiglottis, arytenoids and aryepiglottic folds involved, as demonstrated in Figure 1. A ‘turban’-shaped epiglottis is typically described (21), due to the resultant morphological change after inflammatory and granulomatous infiltrate. True vocal fold paresis is rare but there can often be an appearance of restricted glottic mobility contributing to dysphonia due to the inflammatory process and resultant thickening.
Prior to a diagnosis of laryngeal sarcoidosis, a broad differential must be considered given that presenting symptoms related to airway and voice are also seen in many more common laryngeal pathologies including vocal fold paralysis, supraglottitis and laryngotracheal stenosis (15, 21). The laryngeal appearance may resemble acute infection or other granulomatous diseases such as tuberculosis and vasculitis. Malignancy may rarely present in this way and must always be considered. It is crucial that the entire respiratory tract has been assessed, as more foci of disease suggest a systemic process such as sarcoidosis, where solitary lesions are more likely to be due to localised pathology. Investigations may be performed but only a biopsy demonstrating non-caseating granulomata is diagnostic (5).
Conservative options typically relate to speech and language therapy for patients with symptomatic dysphonia or dysphagia (15). Medical therapy for those with pre-existing sarcoidosis has often been initiated as described above. The majority of patients that require surgical intervention have problematic airway symptoms. Microlaryngoscopy and biopsy is performed for a definitive diagnosis, whilst also providing an opportunity to dilate the airway if required. There have been descriptions of intralesional steroid injection, as well as the use of mitomycin-c to prevent recurrent scar (21). In general, surgical techniques employ the CO2 laser for tissue reduction or excision - the addition of the previously described ‘pepper pot’ technique has been demonstrated to reduce patients’ Medical Research Council dyspnoea grade (21).