pulmonale Vaskulitis

Pulmonary vasculitis refers to vasculitides that affect the lung or pulmonary vessels. If this definition is used, a large group of conditions can fall into this category. The respiratory system may be potentially involved in all systemic vasculitides, although to a variable degree.

Pathology

When using the term "vasculitis" this implies that an inflammatory component is present within the pulmonary vessels.

Small vessel vasculitides

The group that classically forms the dominant pulmonary vasculitis tends to include the small vessel inflammatory processes that affect the pulmonary vessels:

• ANCA associated vasculitides
  • granulomatosis with polyangiitis: ANCA associated pulmonary renal syndrome
  • microscopic polyangiitis
  • eosinophilic granulomatosis with polyangiitis
• idiopathic, pauci-immune pulmonary capillaritis: may or may not be ANCA associated
• non-ANCA associated pulmonary renal syndrome: Goodpasture syndrome 
• rheumatoid pulmonary vasculitis : rare
• cryoglobulinaemic vasculitis (affecting the lungs)

Large vessel vasculitides

Large vessel vasculitides can also affect the lung and pulmonary vessels:

• Takayasu arteritis: 50-80% pulmonary involvement
• giant cell arteritis
• Behçet disease: 1-8% thoracic involvement
• Hugh Stovin syndrome

Granulomatous disease

In addition, certain granulomatous processes in the lung can also have an angiocentric component :

• necrotizing sarcoid granulomatosis
• bronchocentric granulomatosis
• lymphoid granulomatosis

Classification

Various classification systems have been published including the popular Chapel Hill Consensus Conference classification system .

Siehe auch:

• Vaskulitis
• Churg-Strauss-Syndrom
• Granulomatose mit Polyangiitis
• Morbus Behçet
• Takayasu-Arteriitis
• Goodpasture-Syndrom
• Riesenzellarteriitis
• bronchocentric granulomatosis
• Hughes-Stovin-Syndrom
• idiopathic, pauci-immune pulmonary capillaritis (IPIPC)
• Mikroskopische Polyangiitis
• pulmonary capillaritis
• Großgefäßvaskulitis
• lymphoid granulomatosis

und weiter:

• Kryptogene organisierende Pneumonie (COP)
• Bronchiolitis obliterans
• Kavernöse Lungenläsionen
• pulmonary interstitium
• zentrilobuläre Lungennoduli
• feeding vessel sign
• idiopathic pauci immune pulmonary capillaritis
• Aneurysma der Arteria pulmonalis
• Chronische Lungeninfiltrate
• pulmonary manifestations of lymphomatoid granulomatosis
• diffuse Alveolarblutung