Pulmonary involvement in primary systemic vasculitides

Rheumatology (Oxford). 2021 Dec 24;61(1):319-330. doi: 10.1093/rheumatology/keab325.


Objectives: This study describes the spectrum and initial impact of pulmonary manifestations in the primary systemic vasculitides.

Methods: Description and comparison of pulmonary manifestations in adults with Takayasu's arteritis (TAK), GCA, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic GPA (EGPA), polyarteritis nodosa (PAN) and IgA vasculitis (IgAV), using data collected within the Diagnostic and Classification Criteria in Vasculitis study.

Results: Data from 1952 patients with primary vasculitides were included: 170 TAK, 657 GCA, 555 GPA, 223 MPA, 146 EGPA, 153 IgAV and 48 PAN. Pulmonary manifestations were observed in patients with TAK (21.8%), GCA (15.8%), GPA (64.5%), MPA (65.9%), EGPA (89.0%), PAN (27.1%) and IgAV (5.9%). Dyspnoea occurred in patients with TAK (14.7%), GCA (7.8%), GPA (41.8%), MPA (43.5%), EGPA (65.8%), PAN (18.8%) and IgAV (2.6%). Cough was reported in TAK (7.6%), GCA (9.3%), GPA (34.8%), MPA (37.7%), EGPA (55.5%), PAN (16.7%) and IgAV (3.3%). Haemoptysis occurred mainly in patients with ANCA-associated vasculitis (AAV). Fibrosis on imaging at diagnosis was documented in GPA (1.9%), MPA (24.9%) and EGPA (6.3%). Only patients with AAV (GPA 2.7%, MPA 2.7% and EGPA 3.4%) required mechanical ventilation. At 6 months, the presence of at least one pulmonary item in the Vasculitis Damage Index was observed in TAK (4.1%), GCA (3.3%), GPA (15.4%), MPA (28.7%), EGPA (52.7%), PAN (6.2%) and IgAV (1.3%).

Conclusion: Pulmonary manifestations can occur in all primary systemic vasculitides, but are more frequent and more often associated with permanent damage in AAV.

Keywords: ANCA-associated vasculitis; interstitial lung disease; pulmonary; vasculitis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / complications*
  • Female
  • Humans
  • IgA Vasculitis / complications*
  • Lung Diseases / etiology*
  • Male
  • Middle Aged
  • Polyarteritis Nodosa / complications*
  • Takayasu Arteritis / complications*
  • Young Adult