Clinical and microbiologic features of patients with bronchiectasis and autoimmunity

SESSION TITLE: Respiratory Infections: It's Not Just About AntimicrobialsSESSION TYPE: Original InvestigationsPRESENTED ON: 10/16/2022 10:30 am - 11:30 amPURPOSE: This study describes the demographics, microbiologic features, and exacerbation frequency in bronchiectasis patients with autoimmunity compared to patients with idiopathic bronchiectasis.METHODS: The Bronchiectasis and NTM Research Registry (BRR) is a central database for studying patients with non-cystic fibrosis bronchiectasis from 18 sites throughout the U.S.Bronchiectasis patients with autoimmunity were defined by the presence of bronchiectasis and an established autoimmune disorder. Patients with overlap between autoimmunity and immunodeficiency were excluded. Patients with idiopathic bronchiectasis were those patients in which no causative factor could be identified. Descriptive statistics were computed for the main demographic and clinical characteristics of the sample stratified by group. Values between the groups were compared using Kruskal-Wallis test, and Chi-squared / Fisher's exact tests.RESULTS: A total of 4015 bronchiectasis patients were enrolled in the BRR at the time of analysis. Baseline data at the time of enrollment was used for the study. After 2,441 patients were excluded due to presence of a causative etiology other than autoimmunity, 1574 patients remained for analysis, 192 with autoimmunity and 1382 deemed idiopathic. Autoimmune conditions were rheumatoid arthritis, inflammatory bowel disease, relapsing polychondritis, and systemic lupus erythematosus. Demographics were similar in both autoimmune and idiopathic groups with the majority of patients were female 83.8% vs 77.4%, respectively, p=0.219. Mean age at diagnosis (n=1086) was 61.3± 14.6 (autoimmune) and 59.8± 16.3 (idiopathic), p=0.290. Lung function was also similar between the groups; mean FEV1=79.3%predicted± 20.2 (autoimmune) vs 78.3%predicted± 21.0 (idiopathic)(p=0.585). With regard to chronic infection with advanced gram-negative organisms, 16.7% of autoimmune vs 15.9% of idiopathic patients manifested at least one respiratory culture with either Pseudomonas aeruginosa or Stenotrophomonas maltophilia (p=0.791). NTM pulmonary disease diagnosed by ATS criteria was present in 45.8% of autoimmune vs 42.2% of idiopathic patients (p=0.338). The mean number of exacerbations during the 2 years prior to enrollment was not significantly different between the 2 groups: 1.01± 1.47(autoimmune) vs 1.06± 1.79(idiopathic)(p=0.709).CONCLUSIONS:Autoimmune related bronchiectasis is not different from idiopathic bronchiectasis regarding patient demographics, lung function, exacerbation frequency or prevalence of Pseudomonas, Stenotrophomonas and NTM infection.CLINICAL IMPLICATIONS: Airway microbiology, lung function, and exacerbation frequency does not appear to be influenced by the presence of underlying autoimmune mechanisms in patients with bronchiectasis. This could suggest that response to new bronchiectasis therapies may be similar in patients with autoimmunity to those who have idiopathic bronchiectasis.DISCLOSURES: No relevant relationships by Radmila ChoateNo relevant relationships by Angela DiMangono disclosure submitted for Nicole Lapinel;No relevant relationships by Pamela McShaneNo relevant relationships by Olivia RohretResearch relationship with Insmed Please note: 24 months or more Added 03/30/2022 by Kevin Winthrop, value=Consultation feesResearch relationship with Paratek Please note: 24 months or more Added 03/31/2022 by Kevin Winthrop, value=Consulting feesResearch relationship with Redhill Biopharma Please note: 24 months or more Added 03/31/2022 by Kevin Winthrop, value=Consultation feesResearch relationship with AN2 Please note: 24 months or more Added 03/31/2022 by Kevin Winthrop, value=Grant/Research SupportConsultant relationship with AN2 Please note: 24 months or more Added 03/31/2022 by Kevin Winthrop, value=Consulting feeConsultant relationship with Spero Therapeutics Please note: 24 months or more Added 03/31/2022 by Kevin Winthrop, value=Consulting fee SESSION TITLE: Respiratory Infections: It's Not Just About Antimicrobials SESSION TYPE: Original Investigations PRESENTED ON: 10/16/2022 10:30 am - 11:30 am PURPOSE: This study describes the demographics, microbiologic features, and exacerbation frequency in bronchiectasis patients with autoimmunity compared to patients with idiopathic bronchiectasis. METHODS: The Bronchiectasis and NTM Research Registry (BRR) is a central database for studying patients with non-cystic fibrosis bronchiectasis from 18 sites throughout the U.S. Bronchiectasis patients with autoimmunity were defined by the presence of bronchiectasis and an established autoimmune disorder. Patients with overlap between autoimmunity and immunodeficiency were excluded. Patients with idiopathic bronchiectasis were those patients in which no causative factor could be identified. Descriptive statistics were computed for the main demographic and clinical characteristics of the sample stratified by group. Values between the groups were compared using Kruskal-Wallis test, and Chi-squared / Fisher's exact tests. RESULTS: A total of 4015 bronchiectasis patients were enrolled in the BRR at the time of analysis. Baseline data at the time of enrollment was used for the study. After 2,441 patients were excluded due to presence of a causative etiology other than autoimmunity, 1574 patients remained for analysis, 192 with autoimmunity and 1382 deemed idiopathic. Autoimmune conditions were rheumatoid arthritis, inflammatory bowel disease, relapsing polychondritis, and systemic lupus erythematosus. Demographics were similar in both autoimmune and idiopathic groups with the majority of patients were female 83.8% vs 77.4%, respectively, p=0.219. Mean age at diagnosis (n=1086) was 61.3± 14.6 (autoimmune) and 59.8± 16.3 (idiopathic), p=0.290. Lung function was also similar between the groups; mean FEV1=79.3%predicted± 20.2 (autoimmune) vs 78.3%predicted± 21.0 (idiopathic)(p=0.585). With regard to chronic infection with advanced gram-negative organisms, 16.7% of autoimmune vs 15.9% of idiopathic patients manifested at least one respiratory culture with either Pseudomonas aeruginosa or Stenotrophomonas maltophilia (p=0.791). NTM pulmonary disease diagnosed by ATS criteria was present in 45.8% of autoimmune vs 42.2% of idiopathic patients (p=0.338). The mean number of exacerbations during the 2 years prior to enrollment was not significantly different between the 2 groups: 1.01± 1.47(autoimmune) vs 1.06± 1.79(idiopathic)(p=0.709). CONCLUSIONS: Autoimmune related bronchiectasis is not different from idiopathic bronchiectasis regarding patient demographics, lung function, exacerbation frequency or prevalence of Pseudomonas, Stenotrophomonas and NTM infection. CLINICAL IMPLICATIONS: Airway microbiology, lung function, and exacerbation frequency does not appear to be influenced by the presence of underlying autoimmune mechanisms in patients with bronchiectasis. This could suggest that response to new bronchiectasis therapies may be similar in patients with autoimmunity to those who have idiopathic bronchiectasis. DISCLOSURES: No relevant relationships by Radmila Choate No relevant relationships by Angela DiMango no disclosure submitted for Nicole Lapinel; No relevant relationships by Pamela McShane No relevant relationships by Olivia Rohret Research relationship with Insmed Please note: 24 months or more Added 03/30/2022 by Kevin Winthrop, value=Consultation fees Research relationship with Paratek Please note: 24 months or more Added 03/31/2022 by Kevin Winthrop, value=Consulting fees Research relationship with Redhill Biopharma Please note: 24 months or more Added 03/31/2022 by Kevin Winthrop, value=Consultation fees Research relationship with AN2 Please note: 24 months or more Added 03/31/2022 by Kevin Winthrop, value=Grant/Research Support Consultant relationship with AN2 Please note: 24 months or more Added 03/31/2022 by Kevin Winthrop, value=Consulting fee Consultant relationship with Spero Therapeutics Please note: 24 months or more Added 03/31/2022 by Kevin Winthrop, value=Consulting fee