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).