diffuse Lungenblutung

Diffuse pulmonary hemorrhage (DPH) is a subtype of pulmonary hemorrhage where bleeding into the lung is diffuse. If the bleeding is into the alveolar spaces this can be further subclassified as diffuse alveolar hemorrhage (DAH).

Clinical presentation

While the exact presentation can vary is has been classically associated with dyspnea in the setting of hemoptysis +/- an iron deficiency anemia .

Pathology

Associations

• pulmonary vasculitides: especially small vessel vasculitides: alveolar hemorrhage secondary to extensive parenchymal small-vessel vasculitis is typically more diffuse and initially causes more widespread lobular ground-glass opacification with gravity-dependent density gradients through to air-space consolidation, often with interspersed areas of ground-glass opacity; these include
  • granulomatosis with polyangiitis 
  • other connective tissue disorders
    • systemic lupus erythematosus (SLE)
• post bone marrow transplantation
• all-trans-retinoic acid (ATRA) syndrome
• Goodpasture syndrome
• pulmonary hemosiderosis: idiopathic pulmonary hemosiderosis
• coagulative disorders: often require another precipitating source such as an infection to cause DPH
• widespread metastatic lung disease: has been described as a very rare cause of DPH

Radiographic features

Plain radiograph

While chest radiographic appearances are nonspecific, there may be evidence of bilateral air-space consolidation with relative apical sparing .

CT/HRCT

May show areas of widespread ground glass opacification +/- crazy paving pattern +/- areas of consolidation. In the subacute phase HRCT may show fine diffuse nodular densities . In later stage there may also be evidence of interlobular septal thickening due to intralymphatic accumulation of hemosiderin.

Differential diagnosis

For radiographic (plain radiograph and CT) in the acute phase of DPH appearances consider:

• multiple localized pulmonary hemorrhages from diffuse aspiration of blood
• pulmonary edema
• diffuse pulmonary infection / diffuse pneumonia

For HRCT appearances in the subacute phase (i.e. fine nodules) consider:

• hypersensitivity pneumonitis

Siehe auch:

• Lungenödem
• Hypersensitivitätspneumonitis
• Granulomatose mit Polyangiitis
• Goodpasture-Syndrom
• focal pulmonary haemorrhage
• diffuse Alveolarblutung

und weiter:

• Lungenblutung
• crazy paving-Muster
• diffuse pulmonary hemorrhage as the initial manifestation of Wegener granulomatosis