Pulmonary Manifestations of Vasculitis

Specks, Ulrich

Respiratory manifestations in patients with a primary systemic vasculitis syndrome need to be separated into those caused by the vasculitis itself, those related to treatment complications, and those caused by unrelated or only indirectly related comorbidities such as obstructive sleep apnea. Respiratory tract involvement is most common in the ANCA-associated vasculitides. Respiratory manifestations are less frequent and less prominent in other forms of systemic vasculitis, but they may nevertheless represent significant management challenges and carry a poor prognosis. This chapter reviews the tracheobronchial and pulmonary parenchymal disease manifestations, their clinical presentation, and differential diagnosis for each of the vasculitis syndromes. A systematic differential diagnostic approach to the patient with diffuse alveolar hemorrhage is also provided.