Head and Neck Symptoms Can Indicate GPA, So Clinicians Must Be Watchful

Alterations in the nasal cavity mucosa and throat often are primary symptoms of granulomatosis with polyangiitis (GPA) and it is critical that otolaryngologists are aware of the disease’s head and neck manifestations, researchers say.

In a case report, Mayo Clinic researchers highlight some common endoscopic and radiologic findings seen in GPA patients with nose and throat manifestations, which might help educate clinicians.

The study, “Images in Clinical Otolaryngology: Nasal and Subglottic Manifestations of Granulomatosis with Polyangiitis,” was published in the journal Current Advances in Otorhinolaryngology Diseases and Therapy.

GPA is an autoimmune disease characterized by inflammation of small- and medium-sized blood vessels. Signs and symptoms of GPA vary, depending on the tissues and organs affected. But studies show that head and neck symptoms are the first manifestations of the disease in about 80-90% of patients.

“Therefore, it is crucial for the otolaryngologist  — head and neck surgeon — to be cognizant of the variety of head and neck manifestations [and management] of this disease process,” researchers said.

The study reported the case of a 66-year-old woman who had a 10-year history of GPA. She had pulmonary manifestations without renal involvement, and for several years she experienced bilateral nasal obstruction, with no history of stridor (breathing noise) or voice changes.

In the 18 months prior to her presentation, she had excellent disease control.

In a first evaluation by nasal endoscopy, clinicians saw significant scarring between the nasal septum and the lateral wall of the nose. The scars obstructed the passage and prevented a thorough examination of the upper portion of the pharynx.

A computed tomography (CT) scan confirmed the dense scarring of the nasal cavity seen on physical examination. CT images also revealed tissue thickening of the sinuses — hollow cavities in the skull surrounding the nasal cavity.

Further evaluation showed that the mucosa of the lower portion of the larynx (subglottis) also was thicker, making the passage narrower. The tissue did not show obvious signs of inflammation.

The woman had surgery to remove the excess tissue in the nasal cavity and sinus cavities. An examination 10 weeks after surgery confirmed the surgery had resolved the obstruction, and she could breathe through her nose again.

“Over the course of the disease, the incidence of these nasal, otologic [ear] and subglottic manifestations increase,” the researchers said.

About 16-23% of GPA patients have subglottic narrowing, which is thought to be more frequent among patients with a disease onset at a younger age. Also, 16-19% of patients experience ear alterations, although that was not the case in this report.

Surgical therapy often is the go-to approach for managing head and neck symptoms. Immunomodulatory agents, such as methotrexate and cyclophosphamide, also are used to control the immune response.