Granulomatous Diseases of the Larynx

Granulomatous Diseases of the Larynx

Module Summary

Granulomatous diseases of the larynx are generally relatively rare systemic diseases with manifestations in the larynx and other sites in the head and neck. When they affect the larynx, this can result in dysphonia and dyspnea, and less likely dysphagia. While common diseases have predilection for laryngeal subsites (with sarcoidosis generally involving the supraglottis, amyloidosis the glottis, and granulomatous with polyangiitis the subglottis), in practice each pathology can affect each subsite. At times the Otolaryngologist may be the first physician to diagnose the disease with serologic testing and/or tissue biopsy confirming clinical suspicion. Treatment is usually in collaboration with a Rheumatologist/Pulmonologist/Oncologist with the Otolaryngologist offering local therapies and laryngoscopic surveillance.

Module Learning Objectives 
  1. Recognize the common etiologies of granulomatous diseases of the larynx and the subsites they generally involve.
  2. Identify the pathognomonic histologic findings for granulomatous diseases of the larynx.
  3. Explain the evaluation and treatment of granulomatous diseases of the larynx.

Anatomy

Learning Objectives 

Understand the anatomy of the supraglottis, glottis, and subglottis and how certain pathologies affect subsites within the larynx.

References 
  1. Pillsbury HC, Sasaki CT. Granulomatous diseases of the larynx. Otolaryngol Clin N Am. 1982;15:539-51.
  2. Schwartzbauer HR, Tami TA. Ear, nose, and throat manifestations of sarcoidosis. Otolaryngol Clin N Am. 2003;36:673-84.
  3. Gubbels SP, et al. Head and neck manifestations of Wegener’s granulomatosis. Otolaryngol Clin N Am. 2003;36:685-705.

Pathogenesis

Learning Objectives 
  1. Granulomatosis with Polyangiitis (formerly known as Wegener’s Disease), Sarcoidosis, and Amyloidosis are the most common pathologies affecting the larynx
  2. Very, very rarely, other granulomatous disease of the larynx is caused by Actinomyces, Blastomycosis, Syphillis, Histoplasmosis, or Tuberculosis
References 
  1. Pillsbury HC, Sasaki CT. Granulomatous diseases of the larynx. Otolaryngol Clin N Am. 1982;15:539-51.

Basic Science

Learning Objectives 

Know the serologic markers for Sarcoidosis, Granulomatosis with Polyangiitis, and Amyloidosis.

References 
  1. Seo P, Stone JH. The antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med. 2004;117:39-50.
  2. Thomas KW, et al. Sarcoidosis. JAMA. 2003;289:3300.

Incidence

Learning Objectives 

Know the incidence of Otolaryngologic and Laryngeal manifestations of Sarcoidosis, Granulomatosis with Polyangiitis (formerly known as Wegener’s Disease), and Amyloidosis.

References 
  1. Pillsbury HC, Sasaki CT. Granulomatous diseases of the larynx. Otolaryngol Clin N Am. 1982;15:539-51.
  2. Schwartzbauer HR, Tami TA. Ear, nose, and throat manifestations of sarcoidosis. Otolaryngol Clin N Am. 2003;36:673-84.
  3. Seo P, Stone JH. The antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med. 2004;117:39-50.
  4. Gubbels SP, et al. Head and neck manifestations of Wegener’s granulomatosis. Otolaryngol Clin N Am. 2003;36:685-705.

Patient Evaluation

Learning Objectives 
  1. Assess the patient for dyspnea, dysphonia, and less likely dysphagia.
  2. Be able to perform laryngoscopy and bronchoscopy to assess for lesions, inflammation, and fibrosis of the larynx, and to assess for narrowing of the supraglottic, glottic, and/or subglottic airway.
  3. Be cognizant of other sites of involvement in the head and neck or elsewhere in the body.
References 
  1. Pillsbury HC, Sasaki CT. Granulomatous diseases of the larynx. Otolaryngol Clin N Am. 1982;15:539-51.

Measurement of Functional Status

Learning Objectives 

Be familiar with appropriate indications for imaging in a patient with suspected granulomatous disease of the larynx.

References 
  1. Pillsbury HC, Sasaki CT. Granulomatous diseases of the larynx.Otolaryngol Clin N Am. 1982;15:539-51.
  2. Schwartzbauer HR, Tami TA. Ear, nose, and throat manifestations of sarcoidosis. Otolaryngol Clin N Am. 2003;36:673-84.
  3. Gubbels SP, et al. Head and neck manifestations of Wegener’s granulomatosis. Otolaryngol Clin N Am. 2003;36:685-705.

Pathology

Learning Objectives 

Be familiar with the histologic appearance and special histologic stains for granulomatous diseases of the larynx.

References 
  1. Pillsbury HC, Sasaki CT. Granulomatous diseases of the larynx. Otolaryngol Clin N Am. 1982;15:539-51.
  2. Schwartzbauer HR, Tami TA. Ear, nose, and throat manifestations of sarcoidosis. Otolaryngol Clin N Am. 2003;36:673-84.
  3. Gubbels SP, et al. Head and neck manifestations of Wegener’s granulomatosis. Otolaryngol Clin N Am. 2003;36:685-705.

Treatment

Learning Objectives 

Be familiar with medical and surgical therapy for granulomatous diseases of the larynx.

References 
  1. Hseu AF, Benninger MS. Haffey TM, Lorenz R. Subglottic Stenosis: a ten-year review of treatment outcomes. Laryngoscope. 2014;124:736-41.
  2. Butler CR, Nouraei SA, Mace AD, Khalil S, Sandhu SK, Sandhu GS. Endoscopic airway management of laryngeal sarcoidosis. Arch Otolaryngol Head Neck Surg. 2010;136:251-5.
  3. Calich AL, Puechal X, Pugnet G, London J, Terreir B, Charles P, Mouthon L, Guillevin L, French Vasculitis Study Group. Rituximab for induction and maintenance therapy in granulomatosis with polyangiitis (Wegener’s). Results of a single-center cohort study on 66 patients J Autoimmun. 2014;50:135-41.

Medical Therapies

Learning Objectives 

Be familiar with steroids, cytotoxic therapy, and biologic therapy for systemic rheumatologic disease.

References 
  1. Calich AL, Puechal X, Pugnet G, London J, Terreir B, Charles P, Mouthon L, Guillevin L, French Vasculitis Study Group. Rituximab for induction and maintenance therapy in granulomatosis with polyangiitis (Wegener’s). Results of a single-center cohort study on 66 patients J Autoimmun. 2014;50:135-41.

Surgical Therapies

Learning Objectives 
  1. Know about awake or endoscopic steroid injection into granulomatous inflammatory disease of the larynx.
  2. Understand the use of endoscopic laser excision of laryngeal amyloidosis or sarcoidosis and about dilation of GPA-associated subglottic stenosis.
  3. Open airway procedures are cautioned against in active rheumatologic disease.
References 
  1. Hseu AF, Benninger MS. Haffey TM, Lorenz R. Subglottic Stenosis: a ten-year review of treatment outcomes. Laryngoscope. 2014;124:736-41.
  2. Butler CR, Nouraei SA, Mace AD, Khalil S, Sandhu SK, Sandhu GS. Endoscopic airway management of laryngeal sarcoidosis. Arch Otolaryngol Head Neck Surg. 2010;136:251-5.

Case Studies

  1. A 58-year-old female presents with severe hoarseness for 2 years. She was noted to have left true vocal fold edema and a left false vocal cord mass. Initial biopsy 1 year ago of the left false vocal cord in was consistent with vocal cord polyp and no evidence of malignancy. Her voice has severe roughness and strain.

    Laryngoscopy demonstrates fullness of the left false vocal fold consistent with a mass or infiltrative lesion with effacement of ventricle and associated edema and epithelial irregularity of left true vocal cord mucosa. Left vocal cord mobility is sluggish compared to the right. Right false cord also has fullness though less than right, while the right true cord with edematous but regular mucosa. There is anterior webbing at level of false cords. There was aperiodic mucosal wave with reduced pliability bilaterally. There is glottic closure.

    The patient is taken to the operating room where biopsy of the left false vocal fold demonstrates a plasma cell neoplasm with positive congo red stain. What further testing is necessary? How would you elect to treat this patient?

  2. A 53 y/o male presents with hoarseness and dyspnea on exertion. No stridor on exam. Stroboscopy demonstrates slightly edematous bilateral vocal folds, though they had good abduction and adduction and did not have a fibrotic appearance. On adduction, there was a near periodic mucosal wave with intact pliability bilaterally. There is glottic closure. Flexible bronchoscopy reveals an inflamed subglottic stenosis that is approximately 60% narrowed. What are your next steps in treatment? What serology would you order? What surgical therapy would you consider?

Complications

Learning Objectives 
  1. Know the complications related to laryngeal involvement of granulomatous disease.
  2. Be cognizant of the complications of open airway surgery in active rheumatologic disease.
References 
  1. Hseu AF, Benninger MS. Haffey TM, Lorenz R. Subglottic Stenosis: a ten-year review of treatment outcomes. Laryngoscope. 2014;124:736-41.
  2. Butler CR, Nouraei SA, Mace AD, Khalil S, Sandhu SK, Sandhu GS. Endoscopic airway management of laryngeal sarcoidosis. Arch Otolaryngol Head Neck Surg. 2010;136:251-5.
  3. Pillsbury HC, Sasaki CT. Granulomatous diseases of the larynx. Otolaryngol Clin N Am. 1982;15:539-51.

Review

Review Questions 
  1. What are the common granulomatous diseases affecting the larynx?
  2. What laryngeal subsites do these common granulomatous diseases generally involve?
  3. What serologic tests may be performed to diagnose common granulomatous diseases of the larynx?
  4. What are the histopathologic findings in Amyloidosis, Granulomatosis with Polyangiitis, and Sarcoidosis?
References 
  1. Pillsbury HC, Sasaki CT. Granulomatous diseases of the larynx. Otolaryngol Clin N Am. 1982;15:539-51.
  2. Schwartzbauer HR, Tami TA. Ear, nose, and throat manifestations of sarcoidosis. Otolaryngol Clin N Am. 2003;36:673-84.