Introduction
ENT symptoms may become tricky to identify, as they are common symptoms with many autoimmune diseases. These symptoms sometimes appear first in an undiagnosed condition. Even in people diagnosed with autoimmune disease, some symptoms get easily ignored, like eye-related ones, especially when the immune system mistakenly attacks the muscle tissue.
For example, if the immune system attacks the inner ear, as in autoimmune inner ear disease, it can cause progressive hearing loss in both ears. This form of hearing loss, associated with tinnitus, has been linked with autoimmune disorders like Wegener's granulomatosis, a disorder that affects organs and blood vessels; Cogan's syndrome, which has ocular and otologic effects, and systemic lupus erythematosus, a disease capable of damaging many organs.
Recurrent mouth ulcers could also be caused by Behcet's disease or SLE. Nasal and sinus diseases may point to Wegener's granulomatosis or an inflammatory process referred to as sarcoidosis, characterized by the formation of lumps in organs. Salivary gland problems may suggest Sjogren's syndrome or sarcoidosis.
Dysphagia or trouble swallowing may indicate systemic sclerosis, a syndrome characterized by skin and connective tissue hardening and scarring. It may also be a feature of dermatomyositis, a condition with muscle inflammation associated with a rash. Rheumatoid arthritis and its related diseases can cause joint problems in the larynx, or voice box. These examples illustrate well how ENT manifestations are not only local but sometimes point to some more systemic, autoimmune disorders.
How Do Different Parts of the Ear Become a Target of Various Autoimmune or Autoinflammatory Diseases?
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External Ear: In this rare disorder of unknown etiology, which classically presents with relapsing polychondritis, about 90 percent of the patients develop auricular chondritis, characterized by painful, red, and swollen auricles. Untreated inflammation may lead to cauliflower ear deformity or hardening due to connective tissue changes. Rheumatoid polyarthritis is quite an uncommon autoimmune disease that manifests by the inflammation of cartilage-rich areas, including the nose, ears, and respiratory tract. Auricular chondritis may also be caused by granulomatosis with polyangiitis, a very rare autoimmune granulomatous disease that leads to blood vessel inflammation.
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Middle Ear: Other conditions that can cause ear pain and discharges with fluid accumulation are GPA and immunoglobulin G4-related disease. One rare chronic inflammatory disease is IgG4-RD. The pancreas, salivary glands, and other organs are impacted. It occasionally involves the inner ear; however, the middle ear disorder is more common. The patient can also have facial numbness and recurrent mastoiditis.
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Inner Ear: Many systemic autoimmune or autoinflammatory disorders are associated with sensorineural hearing loss (SNHL), corresponding to hearing loss due to inner ear or nerve damage. Rates of SNHL in rheumatoid arthritis span from 21 percent to 69 percent, in systemic lupus erythematosus from eight percent to 28 percent, and in systemic sclerosis from 21 percent to 77 percent. It may result from vascular involvement or inflammation or be secondary to drug exposure. Vestibulo-auditory symptoms, especially with the ocular participation, may suggest the occurrence of Cogan syndrome, or CS, an autoimmune-related illness characterized by manifestations with ocular and aural involvement, specifically, tinnitus and vertigo that developed abruptly with symptomatic concurrent nausea and ocular motility impairment. The suspicion of diagnosing autoinflammatory disorders in patients with unremitting SNHL, elevated inflammatory markers, and occasional recurrent fever following infectious workup is essential. NLRP3-associated autoinflammatory disease is one of the most common genetic autoinflammatory disorders associated with hearing loss and was previously called cryopyrin-associated periodic syndrome, or CAPS. In one type of CAPS, MWS, SNHL typically progresses quickly, resulting in complete deafness. Hearing loss often results first in high-frequency sounds and can be identified by specific MRI findings indicative of injury to certain inner ear structures.
What Are the Oral and Throat Symptoms Associated With Autoimmune Diseases?
Oral Involvement:
Sicca syndrome, affecting about 95 percent of those with Sjogren's syndrome, or SS, is the most common oral manifestation of autoimmune disorders. It results in xerostomia or dry mouth. This dryness can, in turn, lead to secondary problems such as gum disease, dental cavities, and oral yeast infections called candidiasis. Hoarseness and dry cough may also occur due to the dryness. Parotitis is when there is recurring swelling of the parotid glands, and in about 30 to 50 percent of patients with SS, it occurs. It is usually firm, diffuse, and not painful. This is important to differentiate from frequent recurring juvenile parotitis, which typically does not have the presence of ANA antibodies.
The most common presentation of Behcet's disease is recurrent aphthous ulceration. This is a systemic vasculitis of all sizes of blood vessels. This presentation is seen in 95 percent of patients. These painful spots, measuring two mm (millimeters) to 20 mm in diameter, develop inside the cheeks, tongue, mouth, and throat. An ulcer of a bigger diameter may leave scars after healing. The appearance of six or more painful recurrent ulcers on the soft palate or throat, accompanied by redness, would suggest BD in a patient.
Oral ulcers may also be a complication of systemic lupus erythematosus. These lesions are usually painless, appear on the hard palate, and are characterized by telangiectasia patches with whitish plaques and redness in the middle.
Throat Involvement:
One common manifestation of RP is swelling from inflammation of the laryngeal cartilage or chondritis of the larynx. In severe conditions, this is likely to result in laryngomalacia or softening of the larynx stenosis or narrowing. It can also lead to hoarseness and soreness above the thyroid gland.
Hypoglottic stenosis is inflammation of the lower throat, affecting 20 percent of those with GPA. It can be life-threatening due to significant narrowing of the airway. The common symptoms are changes in voice, cough, and breathlessness. In more severe situations, even high-pitched wheezing or audible stridor can be appreciated.
The main organs affected by the chronic inflammatory disease of rheumatoid arthritis are the joints. In this case, the synovial joint in the larynx, the cricoarytenoid joint, gets damaged.
Dysphagia, sore throats, and even sensations of throat fullness or tightness may thus arise due to the disorder. The condition may cause sore throat, dysphagia, or even fullness or tightness in the throat.
Conclusion
Various ENT symptoms involving the ears, nose, throat, and oral cavity are frequently present in autoimmune and inflammatory rheumatic disorders. Although often disregarded, these symptoms can be early warning signs of underlying systemic disorders. ENT specialists are essential in identifying and referring patients to rheumatologists for comprehensive care since they are critical in promptly diagnosing and treating these symptoms.
