Granulomatosis with Polyangiitis: To Know the Nose

Case study highlights importance of collaboration

Granulomatosis with Polyangiitis: To Know the Nose

By Carol A. Langford, MD, MHS

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A 56-year-old woman with a history of granulomatosis with polyangiitis (Wegener’s) (GPA) comes to your clinic with a chief concern of nasal and sinus symptoms. She was diagnosed three years ago with GPA that affected the sinus, lung and kidney. Just prior to her diagnosis, she experienced collapse of the nasal bridge. The GPA was treated with prednisone and rituximab followed by azathioprine for maintenance.

Although she has been in remission, she has had persistent sinonasal symptoms that impact her quality of life. She has questions today about these symptoms and also about surgical repair of her nasal bridge.

16-RHE-XXXX-Langford-Inset-Image-Figs-1-and-2

Figure 1. Computed tomography axial view showing loss of the nasal septum and reactive bony thickening (neo-ossification) of the maxillary sinuses. Figure 2. Computed tomography lateral view showing absence of the nasal septum and evidence of collapse of the external nasal bridge.

Challenges of upper airway disease

The symptoms this patient is experiencing are common in GPA. During the course of their illness, over 95 percent of GPA patients will have upper airway disease, although the severity can vary greatly. Symptoms include congestion, frequent nosebleeds, nasal crusting and sometimes pain across the nasal bridge or face. In some instances, nasal septal perforations or collapse of the cartilaginous portion of the nasal bridge can occur. Cartilage relies on the surrounding mucosa for its blood supply such that severe mucosal inflammation can result in cartilage infarction with resultant perforation or collapse.

Is it active disease or not?

In the GPA patient with ongoing or worsened sinonasal symptoms, the first question to ask is whether these are due to active disease. Trying to determine whether a patient’s symptoms are related to active disease, chronic damage, superimposed infection or a combination of these can be particularly difficult. This is nevertheless important as this distinction influences treatment decisions.

The symptoms of active disease, damage and infection are similar because the sinonasal anatomy is macroscopically and microscopically complex. When mucosal inflammation occurs, permanent damage to the mucociliary apparatus can result and lead to chronic symptoms with crusting and risk of secondary infection from poor sinus drainage.

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Examination and imaging play a valuable but not always definitive role. With active inflammation, the nasal membranes will often have an ulcerated or cobblestoned appearance. Findings on computed tomography can include mucosal thickening, perforations, neo-ossification of the sinuses’ long-standing disease and air-fluid levels that can suggest infection. Mucosal thickening is not in itself indicative of active disease and can persist as a result of scarring.

In patients with a previously established diagnosis of GPA, biopsies of the nasal or sinus mucosa should mainly be considered where an atypical infection or a neoplasm is within the differential. Nasal/sinus biopsies in active GPA most commonly reveal acute and chronic inflammation; granulomas and vasculitis are rarely found. Similar features can be seen in the presence of damage or bacterial infection such that mucosal biopsies generally cannot tell these apart.

For patients who have worsening nasal and sinus symptoms, it is important to perform an assessment for active disease in other locations, which if present would guide treatment decisions.

Treatment of nasal and sinus disease in GPA

Effective treatment of GPA is not all about immunosuppressive medications. Local nasal care is an extremely important part of management in GPA patients with nasal and sinus disease as this reproduces the actions naturally performed by the mucociliary apparatus.

Moisturization techniques include saline spray, nasal emollients and environmental humidification, especially during winter when heating leads to drier air. Bedside room humidifiers can be valuable and should be cleaned frequently to prevent organism growth. Nasal irrigation to remove adherent secretions allows sinus drainage and prevents infection.

There are multiple different nasal irrigation techniques, the choice of which should be individualized to find an approach that the patient will feel comfortable using on a regular basis. Nasal glucocorticoids can be helpful in some patients and are ideally applied following nasal irrigation when the least amount of crusting is present. In patients where there is any suspicion of a superimposed infection, a trial of antibiotics targeting gram-positive organisms can be beneficial in improving symptoms.

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Is surgical repair possible?

Patients who experience collapse of their nasal bridge are often interested in whether this can be surgically repaired. The answer is yes, but with cautionary caveats. The correction of nasal bridge collapse in GPA should be embarked upon carefully as premature intervention can lead to a worsened appearance, which can include cutaneous fistulas. When counseling GPA patients about nasal surgery, there are two essential steps in supporting a good outcome. The first is to ensure that the GPA is locally and systemically in remission. The second is in the choice of the surgeon. In GPA, tissue friability and scarring makes this different from healthy mucosa. It is very important for the physician to have had experience with GPA to understand the challenges of performing surgery on these fragile tissues.

Partnerships for improved outcomes

Working with skilled otolaryngologists is important in optimizing care of people with GPA who have nasal and sinus involvement. At the time of diagnosis, consultation with an otolaryngologist is helpful in providing a thorough baseline examination of the upper airways and in establishing an effective local nasal care program for that patient early on. Continuity of care in otolaryngology can be extremely beneficial for those patients who experience significant mucosal damage in making changes to their local regimen and in providing detailed examinations that may allow better assessment of their disease status.

Here at Cleveland Clinic, physicians within the Head & Neck Institute are invaluable partners in the care of our patients with GPA. They provide expertise for the diversity of upper airway manifestations in GPA, including those in the nose, sinuses, nasolacrimal duct, ears and subglottis. Because of the frequency of upper airway disease and how this can impact quality of life, these collaborations have an extremely meaningful impact not only on patients’ physical health but also their emotional well-being.

Dr. Langford is Director of the Center for Vasculitis Care and Research as well as Vice Chair for Research, Department of Rheumatic and Immunologic Diseases.