Head and Neck Manifestations of Systemic Disease

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What’s the most common granulomatous disease affecting the head and neck? What are its main otolaryngologic manifestations?

Granulomatosis with polyangiitis (GPA) is a systemic disease characterized by necrotizing granulomas of the upper and lower respiratory tract, vasculitis, and glomerulonephritis. It is the most common granulomatous disease of the head and neck. Rhinologic symptoms include rhinitis, congestion, sinusitis, and inflammation that can be severe and progress to nasal septal perforation or nasal stenosis. GPA affects the larynx and trachea and can cause hoarseness, cough, hemoptysis, wheezing, and stridor and can progress to subglottic stenosis. Oral cavity manifestations include gingival hyperplasia and tooth mobility.

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How do you diagnose GPA, and what is the differential diagnosis?

The diagnosis of GPA is based on clinical symptoms, biopsy, and a positive antineutrophil cytoplasmic antibody (C-ANCA) test. C-ANCA is highly specific and sensitive for GPA, but a negative result does not completely rule out GPA. A liberal biopsy of involved nasal mucosa is recommended to look for the characteristic necrotizing granulomas and vasculitis. Isolated oral or laryngeal/tracheal involvement can occur but is less common, and these areas should be biopsied if diagnosis in unclear. Tissue culture should also be done to rule out bacterial or fungal disease.

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What is the treatment for GPA?

Since GPA is a systemic disease, it is best treated in a multidisciplinary fashion with specialists from otolaryngology, rheumatology, nephrology, and other fields. Medical treatment includes immunosuppression with cyclophosphamide, methotrexate, or glucocorticoids. In severe cases, the biologic agent rituximab may be indicated. After control of systemic disease, nasal or airway reconstruction may be needed, but care must be taken to control the disease medically to prevent relapse and further damage to airway structures.

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What autoimmune disease affects the cartilages of the head and neck in a recurrent fashion?

Relapsing polychondritis (RP) is a rheumatologic disorder that involves the cartilages of the nose, ears, and airway as well as systemic involvement of the lymph nodes, lungs, and joints. It typically occurs in the fourth decade of life and affects men and women equally. The incidence is three cases per million.

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What are some of the symptoms of RP?

Most cases of RP present with recurrent auricular chondritis and arthropathy. Patients can also have audiovestibular system damage, nasal and laryngotracheal chondritis, cardiovascular vasculitis, and ocular inflammation. Nasal symptoms include crusting, drainage, and epistaxis and are usually brought on by mucosal disruption and cartilage exposure. Chronic inflammation can cause septal perforation and saddle nose deformity. Airway involvement can be severe and lead to stenosis and collapse of the laryngeal cartilages and trachea, causing obstruction, and patients may need tracheostomy.

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How do you diagnose RP?

There is no specific laboratory test for RP, but markers of inflammation are often increased, including erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and anti nuclear antibodies (ANAs). There are clinical criteria for the diagnosis or RP and include at least three of the following: (1) bilateral auricular chondritis, (2) non erosive seronegative inflammatory polyarthritis, (3) nasal chondritis, (4) ocular inflammation, (5) laryngotracheal chondritis, and (6) audiovestibular damage. Stricter criteria require at least two separate areas of biopsy-proven chondritis with response to steroid. Biopsy specimens show chondritis, chondrolysis, and perichondritis.

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How do you treat RP?

As an autoimmune disease, RP is treated by immunosuppression including corticosteroids and cytotoxic medications. Additional workup may be needed such as imaging, pulmonary function testing, and echocardiography to determine extent of disease. Surgery is directed at the organ system involved and may involve airway and nasal reconstruction.

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