48,830
edits
(→Diagnoses - Table: more) |
(→Rare diseases: merge AGBM & Goodpasture disease) |
||
Line 692: | Line 692: | ||
*Abbreviated ''AGBM''. | *Abbreviated ''AGBM''. | ||
===General=== | ===General=== | ||
*Known as '''Goodpasture disease''' and '''Goodpasture syndrome''' if renal failure is accompanied by pulmonary hemorrhage.<ref>{{cite journal | author=Goodpasture EW | title=The significance of certain pulmonary lesions in relation to the etiology of influenza | journal=Am J Med Sci | year=1919 | volume=158 | pages=863–870 | doi=10.1097/00000441-191911000-00012 | issue=6}}</ref> | |||
*Rare - estimated incidence 1/1-2 million.<ref name=pmid20962523/> | *Rare - estimated incidence 1/1-2 million.<ref name=pmid20962523/> | ||
*[[Antibody mediated hypersensitivity]]. | *[[Antibody mediated hypersensitivity]]. | ||
Line 698: | Line 699: | ||
**Antibodies directed against COL4A3,<ref>{{OMIM|120070}}</ref> which is ''not'' mutated. | **Antibodies directed against COL4A3,<ref>{{OMIM|120070}}</ref> which is ''not'' mutated. | ||
Tx: | |||
*Immune suppression & plasma exchange.<ref name=pmid12597309>{{Cite journal | last1 = Shah | first1 = MK. | last2 = Hugghins | first2 = SY. | title = Characteristics and outcomes of patients with Goodpasture's syndrome. | journal = South Med J | volume = 95 | issue = 12 | pages = 1411-8 | month = Dec | year = 2002 | doi = | PMID = 12597309 }}</ref> | |||
* | |||
Clinical DDx: | Clinical DDx: | ||
*[[Wegener granulomatosis]]. | *[[Wegener granulomatosis]]. | ||
*Azathioprine toxicity.<ref>{{Cite journal | last1 = Stetter | first1 = M. | last2 = Schmidl | first2 = M. | last3 = Krapf | first3 = R. | title = Azathioprine hypersensitivity mimicking Goodpasture's syndrome. | journal = Am J Kidney Dis | volume = 23 | issue = 6 | pages = 874-7 | month = Jun | year = 1994 | doi = | PMID = 8203372 | *Azathioprine toxicity.<ref name=pmid8203372>{{Cite journal | last1 = Stetter | first1 = M. | last2 = Schmidl | first2 = M. | last3 = Krapf | first3 = R. | title = Azathioprine hypersensitivity mimicking Goodpasture's syndrome. | journal = Am J Kidney Dis | volume = 23 | issue = 6 | pages = 874-7 | month = Jun | year = 1994 | doi = | PMID = 8203372 }}</ref> | ||
===Microscopic=== | ===Microscopic=== | ||
Features: | Features: | ||
*RPGN. | *[[RPGN]]. | ||
**Crescentic glomerulonephritis. | **Crescentic glomerulonephritis. | ||
===IF=== | ===IF=== | ||
*Linear IgG deposits - ''' | *Linear IgG deposits - '''diagnostic'''. | ||
DDx: | DDx: | ||
*Goodpasture syndrome with the pulmonary hemorrhage. | |||
==Thin glomerular basement membrane disease== | ==Thin glomerular basement membrane disease== |
edits