Key Words

Sarcoidosis, noncaseating granuloma- sarcoidosis of the larynx,- epiglottis involvement in sarcoidosis Dr Christopher de Souza.

Sarcoidosis is a noncaseating granuloma that is a multisystem inflammatory disease.

Sites most commonly affected by sarcoidosis are bilateral hilar lymphadenopathy and pulmonary opacities. Other extrapulmonary sites involved are the skin, the joints, lymph nodes, and eyes.

Laryngeal involvement is very uncommon, occurring in approximately 0.5% to 6% of reported cases and is often underdiagnosed. Middle-aged women are often the most affected.

Patients with isolated laryngeal sarcoidosis (LS) present with symptoms of upper airway compromise such as dyspnea, change in voice and dysphagia.

The commonest subsite, in order of frequency, is the supraglottis, of which the epiglottis is most commonly involved. The epiglottis has a characteristic thick, edematous, turban-like appearance. This is pathognomonic of LS.

Serum ACE levels are most commonly elevated in 80% of untreated LS patients.

Other treatment modalities include systemic immunosuppression and antineoplastics.

Targeted laryngeal interventions include surgical excision and laryngeal steroid injections. Currently, there is no standard treatment algorithm for isolated LS.

Single or multiple intralesional steroid injections for initial treatment have been described. Intralesional steroid injection has been a reliable alternative to systemic steroids.

Medical literature recommends systemic steroids for persistent or recurrent symptoms.

Some reports have described a high success rate following an initial course of systemic steroids accompanied by simultaneous intralesional steroids and then followed by curative excision.

Other treatment modalities are clofazimine and riminophenazine. This modality is used to treat Hansen's disease (Aka Leprosy).

Antineoplastic modalities such as the TOR inhibitor sirolimus and the Tumour Necrosis Factor (TNF-α) inhibitor infliximab have also been described. Antineoplastic agents like methotrexate and mitomycin have also been described.

At this time, the accepted standard of care for this rare disease is steroids (systemic and intralesional) followed by curative excision.