Session Information
Date: Wednesday, October 24, 2018
Title: 6W021 ACR Abstract: Misc Rheumatic & Inflam DZ II (2970–2975)
Session Type: ACR Concurrent Abstract Session
Session Time: 11:00AM-12:30PM
Clinical Features and Pulmonary Function Test Findings Associated with Large Airway Disease in Relapsing Polychondritis
Background/Purpose:
Relapsing polychondritis (RP) is a systemic disease that can lead to fatal end-organ damage, including subglottic stenosis (SGS) and tracheobronchomalacia (TBM). The study objective was to evaluate the clinical disease features and pulmonary function test (PFT) findings associated with SGS and TBM.
Methods:
Patients 18 years and older were selected from a prospective observational cohort of RP. All patients met McAdams or Damiani’s diagnostic criteria and underwent dynamic expiratory phase CT imaging of the thorax, direct laryngoscopy, and PFTs. SGS was defined by pathological narrowing of the subglottis visualized by direct laryngoscopy. TBM was defined as antero-posterior and/or lateral flattening of the tracheal wall during expiration on dynamic CT. Percentage of tracheal collapse during mid-expiration was measured by a single radiologist. Air trapping was defined as an area of low attenuation compared to the remainder of the lung parenchyma on expiratory CT images. Lung volumes and diffusion capacity were assessed for all patients. Differences were assessed by Fisher’s exact test or Kruskal-Wallis test. Median values are presented.
Results:
A total of 46 patients were included. 9 patients (20%) had SGS, 19 patients (41%) had TBM, and 4 patients (8%) had both (Table). Patients with SGS were predominantly female (100% vs 83, p=0.07) and younger at symptom onset (33 vs 40 years, p=0.04). There was no gender or age differences in patients with TBM compared to those without (84% vs 89% female, p=0.68; 43 vs 44 years, p=0.94). No clinical symptoms were significantly associated with SGS. Patients with TBM had significantly more wheezing (68% vs 23%, p<0.01), a prior diagnosis of asthma (79% vs 22%, p<0.01), and hearing loss (47% vs 19%, p=0.05). Patients with TBM had more respiratory symptoms such as choking sensation (84% vs 66%, p=0.37) and pleuritic chest pain (32% vs 15%, p=0.27) but these differences were not statistically significant. The median tracheal collapse in patients with TBM as compared with patients without was 71% vs 19% (p<0.001). Patients with air trapping on dynamic CT scan had significantly worse tracheal collapse (58% vs 24%, p<0.01). There were no significant associations between PFT results and SGS status. Total lung capacity was significantly higher in patients with TBM (102% vs 92%, p=0.03), but no other PFT parameters were associated with TBM.
Conclusion:
SGS and TBM are relatively common in RP, and specific clinical features and PFT findings are associated with airway damage. Female gender and younger age at symptom onset are associated with SGS. Wheezing, hearing loss, air trapping, and increased total lung capacity are associated with TBM. These findings may help identify subsets of patients with RP who are likely to have large-airway involvement.
|
|
With SGS (n = 9) |
Without SGS (n = 36) |
P-value |
With TBM (n = 19) |
Without TBM (n = 25) |
P-value |
|
Sex (Female, %) |
9 (100%) |
30 (83%) |
0.07 |
16 (84%) |
24 (89%) |
0.68 |
|
Age at symptom onset (Years ± SD) |
33 (17-36) |
40 (30-46) |
0.04 |
43 (36-57) |
44 (36-50) |
0.94 |
|
Clinical Symptoms |
||||||
|
Constitutional symptoms |
1 (11%) |
7 (19%) |
1.00 |
3 (16%) |
5 (19%) |
1.00 |
|
Inflammatory eye disease |
2 (22%) |
8 (22%) |
1.00 |
4 (21%) |
6 (14%) |
1.00 |
|
Audiovestibular Symptoms |
||||||
|
Vertigo |
6 (66%) |
23 (62%) |
1.00 |
12 (63%) |
17 (63%) |
1.00 |
|
Hearing loss |
2 (22%) |
12 (32%) |
0.70 |
9 (47%) |
5 (19%) |
0.05 |
|
Tinnitus |
5 (55%) |
25 (68%) |
0.69 |
12 (63%) |
18 (67%) |
1.00 |
|
ENT Symptoms |
||||||
|
Auricular chondritis |
9 (100%) |
35 (94%) |
1.00 |
18 (95%) |
26 (93%) |
1.00 |
|
Saddle nose |
1 (11%) |
5 (14%) |
1.00 |
4 (21%) |
6 (13%) |
1.00 |
|
Nasal chondritis |
9 (100%) |
35 (94%) |
1.00 |
17 (89%) |
23 (85%) |
1.00 |
|
Sinonasal symptoms |
5 (56%) |
27 (73%) |
0.40 |
13 (68%) |
19 (70%) |
1.00 |
|
Respiratory Symptoms |
||||||
|
Voice changes |
31 (89%) |
8 (84%) |
1.00 |
17 (89%) |
22 (81%) |
0.68 |
|
Choking sensation |
7 (78%) |
27 (72%) |
1.00 |
16 (84%) |
18 (66%) |
0.37 |
|
Dry cough |
8 (89%) |
33 (89%) |
1.00 |
18 (95%) |
23 (85%) |
0.38 |
|
Shortness of breath |
8 (89%) |
30 (81%) |
1.00 |
16 (84%) |
22 (81%) |
1.00 |
|
Pleuritic chest pain |
2 (22%) |
8 (22%) |
1.00 |
6 (32%) |
4 (15%) |
0.27 |
|
Stridor |
2 (22%) |
6 (16%) |
0.64 |
4 (21%) |
4 (14%) |
0.70 |
|
Wheezing |
3 (33%) |
17 (46%) |
0.71 |
13 (68%) |
7 (26%) |
<0.01 |
|
Musculoskeletal Symptoms |
||||||
|
Tenosynovitis |
4 (44%) |
21 (57%) |
0.71 |
12 (63%) |
13 (48%) |
0.37 |
|
Arthralgia |
8 (89%) |
33 (89%) |
1.00 |
16 (84%) |
24 (89%) |
0.67 |
|
Costochondritis |
8 (89%) |
32 (86%) |
1.00 |
17 (89%) |
23 (85%) |
1.00 |
|
Subglottic stenosis (SGS); Tracheobronchomalacia (TBM); Standard deviation (SD) |
||||||
To cite this abstract in AMA style:
Ferrada MA, Sirajuddin A, Goh EL, Quinn K, Gribbons KB, Hansen-Falschen J, Seam N, Colbert R, Sikora KA, Goodspeed W, Thomas A, Kim HJ, Clint A, Chen M, Katz JD, Grayson PC. Clinical Features and Pulmonary Function Test Findings Associated with Large Airway Disease in Relapsing Polychondritis [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/clinical-features-and-pulmonary-function-test-findings-associated-with-large-airway-disease-in-relapsing-polychondritis/. Accessed .« Back to 2018 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-features-and-pulmonary-function-test-findings-associated-with-large-airway-disease-in-relapsing-polychondritis/