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m (→Glomerulus: re-work, i don't believe the number i heard earlier) |
(→Rare diseases: +AGBM) |
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=Rare diseases= | =Rare diseases= | ||
==Antiglomerular basement membrane disease== | |||
*Abbreviated ''AGBM''. | |||
===General=== | |||
*Rare - estimated incidence 1/1-2 million.<ref name=pmid20962523/> | |||
*[[Antibody mediated hypersensitivity]]. | |||
*Thought to occur in genetically susceptible individuals.<ref name=pmid20962523>{{Cite journal | last1 = Zhou | first1 = XJ. | last2 = Lv | first2 = JC. | last3 = Zhao | first3 = MH. | last4 = Zhang | first4 = H. | title = Advances in the genetics of anti-glomerular basement membrane disease. | journal = Am J Nephrol | volume = 32 | issue = 5 | pages = 482-90 | month = | year = 2010 | doi = 10.1159/000321324 | PMID = 20962523 }}</ref> | |||
**Associated with a specific HLA type (HLA-DRB1*1501) and two gene families. | |||
**Antibodies directed against COL4A3,<ref>{{OMIM|120070}}</ref> which is ''not'' mutated. | |||
===Microscopic=== | |||
Features: | |||
*[[RPGN]]. | |||
**Crescentic glomerulonephritis. | |||
===IF=== | |||
*Linear IgG deposits - '''diagnostic'''. | |||
DDx: | |||
*[[Goodpasture syndrome]] - has pulmonary hemorrhage. | |||
==Goodpasture syndrome== | ==Goodpasture syndrome== | ||
*[[AKA]] Goodpasture disease. | *[[AKA]] Goodpasture disease. | ||
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===IF=== | ===IF=== | ||
*Linear IgG deposits. | *Linear IgG deposits - '''key feature'''. | ||
DDx: | DDx: |
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