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Citation:
Weerakkody Y, Idiopathic pauci immune pulmonary capillaritis. Reference article, Radiopaedia.org (Accessed on 30 Mar 2025) https://doi.org/10.53347/rID-19687
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.
History and etymology
It was thought have been first described by Nierman et.al in 1995 6 and then fully charaterised by Jennings et.al in 1997 5.
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1. Ball GV, Bridges SL. Vasculitis. Oxford University Press, USA. (2008) ISBN:0198570198. Read it at Google Books - Find it at Amazon
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2. Schwarz MI, Brown KK. Small vessel vasculitis of the lung. Thorax. 2000;55 (6): 502-10. Thorax (link) - Free text at pubmed - Pubmed citation
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3. Brown KK. Pulmonary vasculitis. Proc Am Thorac Soc. 2006;3 (1): 48-57. doi:10.1513/pats.200511-120JH - Free text at pubmed - Pubmed citation
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4. Mehrotra AK, Gupta PR, Khublani TK et-al. Isolated pauci-immune pulmonary capillaritis. Lung India. 2015;32 (1): 56-9. doi:10.4103/0970-2113.148453 - Free text at pubmed - Pubmed citation
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5. Jennings CA, King TE, Tuder R et-al. Diffuse alveolar hemorrhage with underlying isolated, pauciimmune pulmonary capillaritis. Am. J. Respir. Crit. Care Med. 1997;155 (3): 1101-9. doi:10.1164/ajrccm.155.3.9116994 - Pubmed citation
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6. Nierman DM, Kalb TH, Ornstein MH et-al. A patient with antineutrophil cytoplasmic antibody-negative pulmonary capillaritis and circulating primed neutrophils. Arthritis Rheum. 1996;38 (12): 1855-8. Pubmed citation
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