Background/Purpose: Pulmonary involvement in systemic primary vasculitides is diverse and occurs with variable incidence depending on the type of vasculitis. This study aimed to describe the spectrum and extent of lung manifestations in systemic vasculitides.

Methods: A large, cross-sectional study describing, comparing, and contrasting the lung manifestations of adults with Takayasu arteritis (TAK), giant cell arteritis (GCA), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), polyarteritis nodosa (PAN), and IgA vasculitis (IgAV, Henoch Schönlein) was performed using data from the Diagnostic and Classification Criteria in Vasculitis (DCVAS) study. The DCVAS study is an international, collaborative project that has collected comprehensive clinical data on a large cohort of patients with vasculitis to help in the development of new classification and diagnostic criteria. Data from the baseline and 6-month DCVAS visits were used for analysis.

Results: 1952 patients with primary vasculitides were included: 170 TAK, 657 GCA, 555 GPA, 223 MPA, 146 EGPA, 153 IgAV, and 48 PAN. The presence of respiratory symptoms was frequent in GPA (64.5%), MPA (65.9%), and EGPA (89.0%), but less so in TAK (21.8%), GCA (15.8%), PAN (27.1%) and IgAV (5.9%). Hemoptysis occurred mainly in patients with GPA (26.7%), MPA (23.3%), and EGPA (8.2%) and was rare in TAK (n=3), GCA (n=2), and PAN (n=2); no hemoptysis was reported in IgAV. Intubation for respiratory failure was infrequent and only seen in ANCA-associated vasculitis (AAV; 2.7% in GPA, 2.7% in MPA, 3.4% in EGPA).

Abnormal lung imaging was reported in GCA (19.7%), TAK (12.3%), GPA (59%), MPA (73%), EGPA (70%), PAN (36%), and IgAV (11%). Lung nodules were present in 25%, 11.7%, and 13.3% of patients with GPA, MPA, and EGPA, respectively. Lung imaging in PAN and IgAV revealed non-specific inflammation, consolidation, or pleural effusion.

Pulmonary function tests (done in 32% of patients with AAV) revealed a restrictive pattern in GPA (16.6%), MPA (28.3%), and EGPA (4.6%), and an obstructive pattern in 70% of patients with EGPA. Bronchoscopy was done in 23% of patients with AAV, abnormal in 74% of them (mucosal changes, alveolar hemorrhage, hypersecretion). Lung biopsy, performed in 14% of patients with AAV, showed vasculitis in GPA (60.6%), EGPA (63.2%), but not in MPA (0/6).

At 6 months, lung damage items on the Vasculitis Damage Index (VDI) were present in patients with TAK (4.1%), GCA (3.3%), GPA (15.4%), MPA (28.7%), EGPA (52.7%), PAN (6.2%), and IgAV (1.3%). Only 3 patients with lung damage had died by the 6-month follow-up visit, all with MPA. Death was lung- and vasculitis-related in 1 patient only (alveolar hemorrhage). In GPA, lung damage at month 6 was associated with more frequent baseline gastrointestinal (30.6% vs. 16.7%) and neurological (49.4% vs. 30.3%) symptoms.

Conclusion: Pulmonary manifestations occur in many primary systemic vasculitides, including PAN and IgAV, but are more frequently severe and the source of permanent damage in AAV.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/pulmonary-manifestations-of-primary-systemic-vasculitides/