Interstitial Lung Disease in Giant Cell Arteritis: Review of 23 Patients

BACKGROUND/OBJECTIVE Giant cell arteritis (GCA) is a large-vessel vasculitis with systemic manifestations. A few case reports have described a possible association of GCA with interstitial lung disease (ILD). The primary aim of the present study was to describe the pattern, severity, and course of ILD in patients with GCA. METHODS This medical records review study evaluated adult patients presenting to Mayo Clinic in Rochester, MN, from January 1, 1997, through December 31, 2018, who had the diagnoses of GCA and ILD. Clinical, laboratory, and radiologic data were analyzed. RESULTS In total, 23 patients were in the study. Median (range) age was 78 (58-93) years, and 14 (61%) were women. Six patients (26%) had a cough at GCA diagnosis. At ILD diagnosis, 15 patients had respiratory symptoms, including dyspnea (n = 12, 52%), dry cough (n = 6, 26%), wheezing (n = 1, 4%), and chest pain (n = 1, 4%). On initial chest computed tomography, the most common pattern of ILD was probable usual interstitial pneumonia (n = 7, 30%), indeterminate for usual interstitial pneumonia (n = 5, 22%), and combined pulmonary fibrosis and emphysema (n = 3, 13%). Airway abnormalities were present in 10 patients: 6 with bronchial wall thickening, 2 with bronchiectasis, and 2 with both. At follow-up computed tomography, 8 patients had ILD progression. Three patients with cough improved after initiation of glucocorticoid therapy. CONCLUSIONS Interstitial lung disease and airway abnormalities may be associated with GCA. Although cough may improve, ILD in some patients with GCA may progress despite immunosuppressive therapy.