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

Pulmonary involvement in primary systemic vasculitides

Jean-Paul Makhzoum  1 , Peter C Grayson  2 , Cristina Ponte  3   4 , Joanna Robson  5   6 , Ravi Suppiah  7 , Richard A Watts  8   9 , Raashid Luqmani  8 , Peter A Merkel  10 , Christian Pagnoux  11 ; DCVAS Collaborators
Affiliations

Pulmonary involvement in primary systemic vasculitides

Jean-Paul Makhzoum et al. Rheumatology (Oxford). .

Abstract

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.

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References

    1. Frankel SK, Cosgrove GP, Fischer A, Meehan RT, Brown KK.. Update in the diagnosis and management of pulmonary vasculitis. Chest 2006;129:452–65. - PubMed
    1. Manganelli P, Fietta P, Carotti M, Pesci A, Salaffi F.. Respiratory system involvement in systemic vasculitides. Clin Exp Rheumatol 2006;24:S48–59. - PubMed
    1. Adams TN, Zhang D, Batra K, Fitzgerald J.. Pulmonary manifestations of large, medium, and variable vessel vasculitis. Respir Med 2018;145:182–91. - PubMed
    1. Lally L, Spiera RF.. Pulmonary vasculitis. Rheum Dis Clin North Am 2015;41:315–31. - PubMed
    1. Langford CA, Sneller MC, Hallahan CW. et al. Clinical features and therapeutic management of subglottic stenosis in patients with Wegener's granulomatosis. Arthritis Rheum 1996;39:1754–60. - PubMed

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