Idiopathic pauci immune pulmonary capillaritis

Idiopathic pauci immune pulmonary capillaritis (IPIPC) is considered a rare type of pulmonary vasculitis. Some authors consider this due be an organ specific subset of microscopic polyangiitis 3. It can result in diffuse alveolar hemorrhage.

It is an isolated small vessel vasculitis that by definition is isolated to the lungs and, hence, presents with diffuse alveolar hemorrhage as its primary clinical manifestation. Some (but not all) cases can have pANCA positivity 2.

It was thought have been first described by Nierman in 1995 6 and then fully charaterised by Jennings in 1997 5.

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Article information

rID: 19687
System: Vascular, Chest
Tag: stub, refs, cases
Synonyms or Alternate Spellings:
  • Idiopathic pauci immune pulmonary capillaritis (IPIPC)
  • Idiopathic, pauci immune pulmonary capillaritis

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