Laryngitis in Systemic Diseases: Classification, Anatomic Pathology, Clinical Manifestations, Diagnosis, and Treatment

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Laryngitis in systemic diseases is an inflammation of the laryngeal tissues that develops as a secondary complication rather than an independent condition.

Classification

Depending on the underlying systemic disease, the following forms of laryngitis are distinguished:

  • laryngitis in rheumatoid arthritis;
  • laryngitis in systemic lupus erythematosus;
  • laryngitis in amyloidosis;
  • laryngitis in granulomatosis with polyangiitis.

Laryngitis in rheumatoid arthritis

Rheumatoid arthritis is a systemic autoimmune disease that primarily affects the joints. Laryngeal involvement in this condition is observed in 25–30 % of patients and reach up to 75 % according to some sources. This higher rate is likely associated with improved diagnostic capabilities and increased physician awareness. In some cases, laryngeal involvement may be the only sign pointing to rheumatoid arthritis.

Pathogenesis and Anatomic Pathology

Rheumatoid arthritis is characterized by aseptic inflammation of the joints. In the larynx, this leads to arthritis of the cricothyroid joints, with visible edema and hyperemia.

Subsequently, fibrosis and ankylosis of the joints develop, resulting in narrowing of the glottis, sagging of the vocal folds during phonation, and limitation of their mobility (paralysis).

When the true vocal folds (TVFs) are affected, yellowish nodules resembling bamboo stems (“bamboo nodes”) can be observed on their surface.

Laryngitis in Rheumatoid Arthritis
Laryngitis in Rheumatoid Arthritis — 3D Model

Clinical Manifestations

Hoarseness, dysphonia, and aphonia are noted in rheumatoid arthritis. In cases of pronounced vocal fold paresis, respiratory impairment may occur, including stridor and dyspnea, to the point of chronic stenosis and suffocation.

Severe throat pain that intensifies during swallowing (odynophagia) and phonation may radiate to the ear. Patients report a sensation of a foreign body in the throat or globus sensation, as well as dysphagia, or difficulty swallowing.

Diagnosis

Diagnosis is based on medical history, clinical examination, and laboratory findings. Depending on the equipment available in the facility, indirect laryngoscopy or videoendoscopy of the larynx is performed.

Blood tests show elevated ESR, rheumatoid factor, and C‑reactive protein, along with markers of autoimmune inflammation. Computed tomography (CT) of the larynx demonstrates changes in the structure and size of the cricoarytenoid joints.

Treatment Options

Both medical and surgical therapy are used. The primary approach is medical therapy. Patients are prescribed high doses of corticosteroids, cytotoxic agents, and anti‑inflammatory medications. Corticosteroids may also be injected directly into the cricothyroid joint.

“Bamboo nodes” are excised surgically. In cases of cricothyroid joint ankylosis and TVF paralysis, surgical correction is performed. With bilateral TVF paralysis and grade III–IV stenosis, tracheotomy is indicated.

Laryngitis in Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with multisystem involvement. Laryngeal involvement occurs in 30 % of affected individuals.

Anatomical Changes in Larynx

In SLE, immune complexes exert pathological effects, damaging vascular endothelial cells, connective tissue, mucous membranes, and nerves.

Ulcers form in the larynx, and the mucosa becomes hyperemic and edematous. The cricothyroid joints are affected, limiting the TVF mobility. Once ulcers heal, scars start to form, narrowing the laryngeal lumen.

Laryngitis in Systemic Lupus Erythematosus
Laryngitis in Systemic Lupus Erythematosus — 3D Model

Cases of demyelination of the recurrent laryngeal nerve have been described, which also leads to TVF paralysis.

Clinical Manifestations

In the acute stage of systemic lupus erythematosus, patients experience severe throat pain, hoarseness, dysphonia, and dyspnea. As the process progresses, chronic stenosis develops, and asphyxia is possible.

Diagnosis

Diagnosis is based on medical history, clinical examination, and laboratory findings. Depending on the equipment available in the facility, indirect laryngoscopy or videoendoscopy of the larynx is performed. Electromyography of the larynx is also conducted.

In systemic lupus erythematosus, serum levels of antineutrophil antibodies and antibodies to double‑stranded DNA are elevated.

Approaches to therapy

No specific treatment for this condition is available. Patients are prescribed high doses of corticosteroids, cytotoxic agents, and anti‑inflammatory medications. In cases of pronounced TVF paralysis, tracheostomy is performed.

Laryngitis in Amyloidosis

Amyloidosis is a systemic disease in which an insoluble protein — amyloid — is deposited in tissues. Laryngeal amyloidosis is rare and accounts for about 1 % of all benign laryngeal tumors.

Primary (AL) and secondary (AA) amyloidosis are distinguished. The primary form arises spontaneously, whereas the secondary form develops against the background of inflammatory or infectious disorders.

Pathomorphology and Localization of Deposits

Amyloid deposits in the larynx may occur in both primary and secondary amyloidosis.

Primary amyloidosis is characterized by an isolated form of the disease in which light chains of immunoglobulins are produced in the bone marrow. They accumulate as amyloid plaques in various organs and tissues. In the larynx, deposits are localized in the submucosal layer of the vocal folds, have a focal pattern, and may appear as nodules or polyps.

In secondary amyloidosis, amyloid deposition occurs in the setting of an existing disease, such as rheumatoid arthritis or Crohn’s disease. Systemic infections trigger the synthesis and aggregation of an acute‑phase protein (serum amyloid A). In the larynx, diffuse amyloid deposits are observed as polypoid pale‑yellow masses covered by a smooth, waxy, pale mucosal surface.

Symptoms and Clinical Manifestations

In amyloidosis, the clinical manifestations are generally limited. Typical complaints include dysphonia, hoarseness, dyspnea, throat pain, and a globus sensation. With extensive deposits on the TVFs and in the subglottic region, respiratory impairment and stridor may develop.

Diagnostic Methods

Diagnosis is based on anamnesis and clinical examination. Depending on the equipment available in the facility, indirect laryngoscopy or videoendoscopy of the larynx is performed.

If amyloidosis is suspected, CT of the neck is conducted to identify thickening of affected tissues. The final diagnosis is established upon histological examination of the lesions.

Treatment regimens

Treatment depends on the type of amyloidosis.

In primary amyloidosis, corticosteroid therapy is ineffective, so surgical excision of affected areas is performed.

In secondary amyloidosis, treatment of the underlying disease is the priority: patients are prescribed high doses of corticosteroids and anti‑inflammatory medications. Surgical removal of amyloid deposits, on the contrary, leads to even more accumulations.

Distinguishing Features of Primary and Secondary Laryngeal Amyloidosis

Feature Primary laryngeal amyloidosis Secondary laryngeal amyloidosis
Etiology Immune system dysfunction — production of abnormal proteins (immunoglobulin light chains) in the bone marrow. A complication of chronic inflammation or infection in the body (i. e., tuberculosis, rheumatoid arthritis).
Protein type AL‑type proteins (amyloid light chain) SAA proteins (serum amyloid  A — inflammation‑related proteins)
Localization May be limited to the larynx More often part of a systemic process — other organs are also affected
Laryngoscopy More commonly appears as localized nodules or small masses on the vocal folds or other parts of the larynx. Lesions may be yellow‑pink, wax‑like, nodular, and covered by mucosa. May also present as diffuse thickening of the laryngeal mucosa in the form of polypoid pale‑yellow growths covered by smooth, waxy, pale mucosa.
ENT Complaints Hoarseness, changes in voice timbre, throat irritation, a globus sensation, intermittent coughing, throat pain (rare), and difficulty breathing (with extensive deposits) The same complaints occur as in primary amyloidosis, though less pronounced.
General Complaints When the process is localized exclusively in the larynx, there may be no additional complaints. In systemic amyloidosis, fatigue, weakness, dyspnea, numbness in the hands and feet are observed Presence of an underlying disease (tuberculosis, rheumatoid arthritis). Fatigue, weakness, weight loss, low‑grade fever
Clinical Course The disease progresses slowly, the lesion is often benign, but may interfere with breathing or voice. It is frequently associated with a severe underlying disease and requires treatment of the primary cause.
Treatment Chemotherapy, steroid therapy, and bone marrow stem cell transplantation may be used. Surgical removal of deposits is possible Treatment of the underlying disease is essential.
Note that surgical removal of amyloid deposits leads to even greater accumulation.

Laryngitis in Granulomatosis with Polyangiitis (GPA, Wegener’s Granulomatosis)

Granulomatosis with polyangiitis is an autoimmune disease characterized by necrotizing inflammation of blood vessel walls. According to various sources, laryngeal involvement is observed in 15–55 % of patients. In addition, the pathological process affects the kidneys and other parts of the upper respiratory tract.

Anatomy

In the larynx, the subglottic region is most commonly affected. Edema and ulceration of the mucous membrane with hemorrhage are observed in the subglottic area. Granulomas form and subsequently undergo scarring, leading to stenosis.

Clinical Manifestations

Granulomatosis with polyangiitis manifests clinically with sore throat, hemoptysis, and hoarseness. In later stages, stridor and difficulty breathing develop.

Diagnostic Criteria

Diagnosis is based on anamnesis and clinical examination. Depending on available equipment, indirect laryngoscopy or videoendoscopy of the larynx followed by biopsy is performed.

Granulomatosis with polyangiitis is confirmed by detecting antineutrophil cytoplasmic autoantibodies (ANCA) in the blood.

Treatment

No specific treatment for this condition is available. Patients are prescribed high doses of corticosteroids, cytotoxic agents, and anti‑inflammatory medications. When stenosis develops, tracheostomy is performed.

FAQ

1. What is secondary laryngitis in systemic diseases?

This is a specific lesion of the larynx that does not arise from infection (viruses or bacteria) but develops as a complication of severe autoimmune or metabolic disorders — rheumatoid arthritis, systemic lupus erythematosus, amyloidosis, or granulomatosis with polyangiitis. In this case, the body’s own immune complexes or abnormal proteins damage the cartilages, joints, and mucous membranes of the respiratory tract.

2. What are “bamboo nodes” in the larynx?

“Bamboo nodes” are specific yellowish thickenings that appear on the vocal folds. Visually, they resemble bamboo stems. This is a pathognomonic (characteristic) sign of laryngeal involvement in rheumatoid arthritis. The nodes cause persistent dysphonia and are removed surgically.

3. Is laryngitis caused by autoimmune disorders contagious?

Unlike classic viral or bacterial inflammation of the upper respiratory tract, the systemic form of the disease is absolutely not contagious. This pathology is not transmitted by airborne droplets or through household contact, as it is caused not by an infectious agent but an internal malfunction of the patient’s immune system.

4. Which examinations and tests help identify systemic laryngitis?

Since symptoms may resemble ordinary inflammation, specific studies are required for an accurate diagnosis. An ENT specialist performs videoendoscopy of the larynx and computed tomography (CT) to assess the condition of the joints and cartilages. Additional blood tests are ordered to detect markers of autoimmune inflammation: rheumatoid factor, C‑reactive protein, ANCA (in granulomatosis), and anti‑DNA antibodies (in lupus).

5. How is laryngitis caused by autoimmune diseases treated?

Unlike typical laryngitis, local treatment becomes secondary. The foundation of therapy is strict control of the underlying disease. Patients are prescribed systemic medications: high doses of corticosteroids, cytotoxic agents, and anti‑inflammatory drugs. In primary amyloidosis, corticosteroids are ineffective, so surgical excision of affected tissues is used.

6. Why is laryngeal involvement dangerous in systemic lupus erythematosus and Wegener’s granulomatosis?

The main life‑threatening consequence of these conditions is irreversible narrowing of the laryngeal lumen. The ulcers and granulomas that form eventually scar, and involvement of the cricoarytenoid joints leads to paralysis of the vocal folds. This causes chronic cicatricial laryngeal stenosis, severe respiratory insufficiency, and risk of asphyxia, requiring emergency tracheotomy.

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