What is the diagnosis and treatment for pulmonary vasculitis (inflammation of the blood vessels in the lungs)?

From the Guidelines

Pulmonary vasculitis should be treated with immunosuppressive therapy, with corticosteroids as the initial cornerstone of management, and rituximab or cyclophosphamide as steroid-sparing agents, according to the most recent guidelines 1. The diagnosis of pulmonary vasculitis involves clinical presentation, laboratory tests, and imaging studies. The clinical presentation may include symptoms such as cough, hemoptysis, and dyspnea, as well as signs of systemic inflammation. Laboratory tests may include ANCA testing, and imaging studies such as chest radiography and CT scans. Some key points to consider in the diagnosis and treatment of pulmonary vasculitis include:

• The use of biopsies to establish a diagnosis, with lung biopsies having a higher diagnostic yield than transbronchial biopsies 1
• The importance of testing for both PR3-ANCA and MPO-ANCA using a high-quality antigen-specific assay as the primary method of testing 1
• The recommendation for treatment with a combination of glucocorticoids and either rituximab or cyclophosphamide for induction of remission in patients with new-onset or relapsing GPA or MPA with organ-threatening or life-threatening disease 1
• The use of plasma exchange in the setting of rapidly progressive renal failure or severe diffuse pulmonary hemorrhage 1
• The importance of regular monitoring of pulmonary function, inflammatory markers, and medication side effects to assess treatment response and adjust therapy accordingly 1
• The recommendation for maintenance therapy to continue for 24-48 months following induction of remission of new-onset disease, with longer duration of therapy considered in relapsing patients or those with an increased risk of relapse 1

From the Research

Diagnosis of Pulmonary Vasculitis

• Pulmonary vasculitis is a group of disorders characterized by vascular destruction with cellular inflammation and necrosis, and can affect small, medium, and large vessels 2
• The diagnosis of pulmonary vasculitis can be challenging, and a high level of suspicion is essential to obtain a timely diagnosis 3
• Clinical examination of other organ systems typically affected by vasculitis, such as skin and kidney, and autoantibody measurements are complementary approaches to manage this situation 4
• Diffuse alveolar hemorrhage (DAH) is a clinical feature that often initiates the concern for an underlying vasculitis, but hemoptysis may not be apparent or its presentation can be mistaken for an alternative disease process 3

Treatment of Pulmonary Vasculitis

• Treatment of life-threatening pulmonary bleeding and irreversible damage to organs, especially the kidneys, requires rapid diagnosis of these conditions 5
• General principles for the treatment of vasculitis are reviewed, including the use of immunosuppressive therapy 6
• Therapies, including biologics, are evolving rapidly, requiring cooperation of specialties and with specialized centers to achieve best patient care 4
• All involved physicians should be aware of typical complications of immunosuppressive therapy 4
• The treatment options for patients with pulmonary vasculitis have advanced significantly over the past decade, and effective therapies can be implemented with a timely diagnosis 3