What are sarcoids in Ear, Nose, and Throat (ENT)?

From the Guidelines

Sarcoidosis in ENT is not explicitly mentioned in the provided evidence, however, Granulomatosis with Polyangiitis (GPA) is discussed, which is a distinct condition that can affect the ENT region. The evidence provided focuses on GPA, a condition that can present with ENT-related symptoms, including rhinological, otologic, and laryngopharyngeal manifestations 1.

Key Points

• Two thirds of patients with GPA initially present with an ENT-related symptom, with the majority being rhinological (~40%) 1.
• Nasal symptoms are common, including crusting, discharge, nasal stuffiness, bleeding, reduced sense of smell, and facial pain 1.
• A characteristic supratip collapse can occur in 18-25% of patients, which may or may not be associated with septal perforation in up to 30% 1.
• The entire internal structure of the nose may disappear, creating a large featureless cavity, and patients may experience hearing loss, vertigo, tinnitus, and facial nerve palsy 1.
• Subglottic stenosis and vocal fold paralysis can occur, and symptoms may be wrongly attributed to the lower respiratory tract 1.

Diagnosis and Treatment

• Diagnosis can be delayed by 6 months or longer, rendering the condition potentially life-threatening 1.
• Disease activity can be measured using the Birmingham Vasculitis Activity Score (BVAS), a Vasculitis Damage Index (VDI), and an ELK-classification of organ involvement (ENT, lung, kidney) 1.
• Treatment is not explicitly mentioned in the provided evidence for sarcoidosis, but for GPA, treatment typically involves immunosuppressive therapy, such as corticosteroids and other agents, to control disease activity and prevent organ damage. In the absence of explicit evidence on sarcoidosis in ENT, it is essential to consider GPA as a potential diagnosis in patients presenting with ENT-related symptoms, and to refer them to a specialist for further evaluation and management.

From the Research

Definition and Prevalence of Sarcoids in ENT

• Sarcoidosis is a multisystemic inflammatory disease that can affect various organs, including the ear, nose, and throat (ENT) [ 2 ].
• ENT manifestations are present in 5%-15% of patients with sarcoidosis, often as a presenting feature [ 2 ].
• Sarcoidosis can affect the larynx, nasal cavity, and otological systems, leading to symptoms such as difficulty breathing, dysphonia, cough, crusting, rhinitis, nasal obstruction, and anosmia [ 2 ].

Clinical Features and Treatment of Sarcoids in ENT

• Laryngeal sarcoidosis can be treated with speech and language therapy (SLT), intralesional injection, dilatation, or tissue reduction [ 2 ].
• Nasal disease can be treated with topical nasal or intralesional treatments, and may also require endoscopic sinus surgery, laser treatment, or nasal reconstruction [ 2 ].
• Otological disease is uncommon, but can include audiovestibular symptoms, sensorineural and conductive hearing loss, and skin lesions [ 2 ].
• Effective management strategies require good diagnostic skills and the use of specific therapies combined with established treatments such as corticosteroids [ 2 ].

Case Reports and Treatment Outcomes

• Two case reports of systemic sarcoidosis with ENT onset highlighted the importance of considering sarcoidosis in the differential diagnosis of ENT granulomatosis [ 3 ].
• Treatment with glucocorticoids led to clinical improvement in both cases [ 3 ].
• Comparisons of treatment outcomes are needed to establish best practice in the management of sarcoidosis [ 2 ].

Treatment Options for Sarcoidosis

• Steroids remain the first-choice therapeutic for sarcoidosis, but long-term use is associated with toxicity [ 4 ].
• Methotrexate and azathioprine are commonly used as second-line therapies for sarcoidosis, with significant steroid-sparing potency and similar positive effects on lung function [ 4 ].
• Other treatment options, including hydroxychloroquine, cyclosporine, and cytotoxic agents such as cyclophosphamide, may be considered in certain cases [ 5 ].
• The choice of treatment depends on the individual patient's disease manifestation, clinical course, and potential side effects of immunosuppressive therapy [ 6 ].