18th February 2025

Pulmonary manifestations of other vasculitides

Rheumatoid arthritis

If longstanding, rheumatoid arthritis is associated with interstitial lung disease, inflammation of the pleura (pleuritis and pleural effusions) and, rarely, pulmonary vasculitis leading to pulmonary hypertension.

Takayasu arteritis

Pulmonary arteritis may cause pulmonary hypertension.

Giant cell arteritis

Pulmonary involvement is rare, but interstitial lung disease has been reported.

Behçet’s disease

Behçet’s may affect the pulmonary artery (the second most commonly affected site after the aorta), generally causing aneurysms rather than thrombosis. Haemoptysis is the most prevalent pulmonary presenting symptom. Behçet’s disease is also typically associated with orogenital ulceration, arthralgia, uveitis and pericarditis.

Epidemiologically, it is classically seen in people with Middle Eastern, Southeast Asian and Mediterranean ethnic origin. It is classically seronegative but can be investigated with a pathergy test.

Hughes-Stovin syndrome

This is thought to be a cardiovascular variant of Behçet’s disease, characterised by widespread pulmonary or bronchial artery aneurysms and deep vein thrombosis.

Idiopathic pauci-immune pulmonary capillaritis

IPIPC is a rare cause of pulmonary vasculitis. It doesn’t present with the systemic features of other vasculitides and is largely lung specific. Although pauci-immune, it may be associated with a weakly positive p-ANCA. Some consider this to be a lung-specific form of MPA. Treatment is with glucocorticoids and cyclophosphamide.