Pulmonary Vasculitis: Clinical Presentation, Differential Diagnosis, and Management

Abstract

This review focuses on vasculitides with prominent pulmonary manifestations and discusses key contributions from the recent literature. Pulmonary vasculitis should be considered when clinical findings include alveolar hemorrhage, nodular and cavitary lung disease, airway stenosis, pulmonary artery aneurysms, or pulmonary artery stenosis. The differential diagnostic considerations for common clinical presentations of vasculitis in the lung are important, and several recent additions are discussed. Treatment for established pulmonary vasculitis is effective and has decreased the morbidity and mortality associated with these diseases while introducing an increased risk of infectious complications. Advances in immunosuppressive therapy have improved treatment of refractory disease and are likely to change initial treatment strategies in the future.

Dyspnea due to Pulmonary Vessel Arteritis

“…Cyclophosphamide and anti-tumour necrosis factor agents have been used in TA for individuals resistant to glucocorticoids (5). In patients unresponsive to medical therapy, balloon angioplasty with stent placement can be used (7,8). Pulmonary hypertension is a serious and life-threatening condition that should be treated aggressively with pharmacological and/or invasive approaches.…”

Pulmonary capillaritis leading to alveolar hemorrhage in a juvenile idiopathic arthritis patient: first description

“…The use of intravenous immunoglobulin, trimethoprimsulfamethoxazole, antilymphocyte monoclonal antibodies, tumor necrosis factor inhibitors, factor VIIa, mycophenolate mofetil and anti-CD20 monoclonal antibody are eventually used for the treatment of DAH [4,5].…”

“…Other conditions, like infectious diseases such as yellow fever, leptospirosis, hantavirus and blood dyscrasia which are the leading causes of alveolar hemorrhage, as well as autoantibodies which might suggest small-vessel vasculitis or antiphospholipid antibody syndrome [5] were discarded by clinical and laboratory criteria. This is the Wrst description of a JIA patient who evolved with alveolar hemorrhage due to pulmonary capillaritis.…”

Pathology

“…Other sites may also be involved, including the upper respiratory tract, skin, gastrointestinal tract, and the musculoskeletal and nervous systems. [208][209][210] The pathophysiology and clinical features of the various vasculitides are reviewed in more detail in Chapter 60.…”