Dyspnea in a Patient with Wegener’s: How Would You Assess It?

By Carol A. Langford, MD, MHS

Case presentation

You are asked to see a 59-year-old woman with granulomatosis with polyangiitis (Wegener’s) (GPA) with recent dyspnea. She was diagnosed four years ago with disease involving the sinuses, lungs, joints and skin for which she was treated with prednisone and methotrexate and achieved remission. She is physically active and over the past four months has had progressive dyspnea such that she can now jog for only a short distance. She has minimal cough and no fevers or voice changes.

On examination she appears well and has a pulse oxygenation of 99 percent that does not decrease on walking in the halls. There is no audible stridor or abnormalities on pulmonary or cardiac examination. She has scarring of the nasal mucosa without inflammation. The remainder of her examination is normal.

What assessment would you perform?

In a patient with GPA who presents with dyspnea, the differential includes active disease, infection, medication toxicity and causes unrelated to the disease or its treatment. The most immediate priority is to assess the severity of symptoms and signs in considering whether inpatient evaluation is required.

In a medically stable patient, the evaluation is guided by the history and examination, but in all instances it should include lab tests and CT imaging of the chest. For patients in whom there is a concern for pulmonary embolism, imaging should be performed with contrast in the absence of contraindications. If these do not suggest a cause, further investigations may include otolaryngology assessment for subglottic stenosis, pulmonary function tests (PFTs) and evaluation for cardiac causes.

Case continued

Figure 1. Bronchoscopic image showing endobronchial inflammation and stenosis.

In this patient, lab results, chest CT, otolaryngology assessment and stress echo were all unremarkable. PFTs revealed an obstructive pattern that did not respond to bronchodilators. This prompted bronchoscopy, which revealed endobronchial stenoses involving the left mainstem and right upper lobe bronchi (Figure 1).

Endobronchial disease in GPA

Endobronchial disease can be a challenging GPA manifestation. Patients will often present with dyspnea and sometimes with cough. Although wheezing can be present, lung auscultation can also be normal. Occlusive endobronchial lesions can result in lung collapse (Figure 2), but in the absence of parenchymal lung disease, chest CT may be unrevealing. PFTs, which often show an obstructive pattern without response to bronchodilators, are an important clue.

Figure 2. Chest CT showing focal collapse of the medial aspect of the left upper lobe resulting from an occlusive endobronchial lesion of the left upper lobe bronchus.

The diagnosis of endobronchial disease is established by bronchoscopy, which provides information on the presence and severity of airway narrowing and the appearance of the mucosa. Bronchoscopy also allows for lavage and biopsies, which can be diagnostically useful.

Treatment of endobronchial disease can be difficult. In patients with active disease, usually defined by the presence of a new stenosis with an inflamed mucosa, management is similar to that used for other GPA manifestations with an induction regimen that combines prednisone with cyclophosphamide, rituximab or methotrexate.

Interventional pulmonologists with expertise in GPA play an important role not only in detecting and monitoring endobronchial disease but also in addressing tight lesions with cautious use of steroid injections. Stents are to be avoided except in extreme circumstances, and metal stents should never be used. Because of the predilection for endobronchial disease to rapidly scar, persistent airway narrowing is typical. This makes distinguishing active endobronchial disease from damage particularly challenging, as worsening of prior stenosis can also occur as a result of progressive scarring.

Back to our patient

The case patient’s endobronchial disease was felt to be indicative of a GPA relapse, for which she was treated with rituximab and prednisone with symptomatic improvement. One year later, her dyspnea worsened, and on bronchoscopy she was found to have increased narrowing in the left mainstem bronchus with no evidence of mucosal inflammation or active disease in other organ sites. She was treated by the interventional pulmonology team with glucocorticoid injections without an increase in immunosuppression, after which her breathing improved.

This patient illustrates many important aspects of the care of patients with GPA, including the broad differential of dyspnea, the challenges faced in diagnosing and managing endobronchial disease, and how a multidisciplinary approach can greatly enhance the effectiveness of GPA management.

Dr. Langford is Director of the Center for Vasculitis Care and Research as well as Vice Chair for Research in Cleveland Clinic’s Department of Rheumatic and Immunologic Diseases.