Secondary amyloidosis pathophysiology: Difference between revisions

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== Associated Conditions ==
== Associated Conditions ==
*[[Conditions]] associated with AA amyloidosis include:<ref name="BlankHegenbart2018">{{cite journal|last1=Blank|first1=Norbert|last2=Hegenbart|first2=Ute|last3=Dietrich|first3=Sascha|last4=Brune|first4=Maik|last5=Beimler|first5=Jörg|last6=Röcken|first6=Christoph|last7=Müller-Tidow|first7=Carsten|last8=Lorenz|first8=Hanns-Martin|last9=Schönland|first9=Stefan O.|title=Obesity is a significant susceptibility factor for idiopathic AA amyloidosis|journal=Amyloid|volume=25|issue=1|year=2018|pages=37–45|issn=1350-6129|doi=10.1080/13506129.2018.1429391}}</ref><ref name="van der HilstYamada2008">{{cite journal|last1=van der Hilst|first1=J. C. H.|last2=Yamada|first2=T.|last3=Op den Camp|first3=H. J. M.|last4=van der Meer|first4=J. W. M.|last5=Drenth|first5=J. P. H.|last6=Simon|first6=A.|title=Increased susceptibility of serum amyloid A 1.1 to degradation by MMP-1: potential explanation for higher risk of type AA amyloidosis|journal=Rheumatology|volume=47|issue=11|year=2008|pages=1651–1654|issn=1462-0324|doi=10.1093/rheumatology/ken371}}</ref><ref name="PapaDoglio2017">{{cite journal|last1=Papa|first1=Riccardo|last2=Doglio|first2=Matteo|last3=Lachmann|first3=Helen J.|last4=Ozen|first4=Seza|last5=Frenkel|first5=Joost|last6=Simon|first6=Anna|last7=Neven|first7=Bénédicte|last8=Kuemmerle-Deschner|first8=Jasmin|last9=Ozgodan|first9=Huri|last10=Caorsi|first10=Roberta|last11=Federici|first11=Silvia|last12=Finetti|first12=Martina|last13=Trachana|first13=Maria|last14=Brunner|first14=Jurgen|last15=Bezrodnik|first15=Liliana|last16=Pinedo Gago|first16=Mari Carmen|last17=Maggio|first17=Maria Cristina|last18=Tsitsami|first18=Elena|last19=Al Suwairi|first19=Wafaa|last20=Espada|first20=Graciela|last21=Shcherbina|first21=Anna|last22=Aksu|first22=Guzide|last23=Ruperto|first23=Nicolino|last24=Martini|first24=Alberto|last25=Ceccherini|first25=Isabella|last26=Gattorno|first26=Marco|title=A web-based collection of genotype-phenotype associations in hereditary recurrent fevers from the Eurofever registry|journal=Orphanet Journal of Rare Diseases|volume=12|issue=1|year=2017|issn=1750-1172|doi=10.1186/s13023-017-0720-3}}</ref>
*[[Conditions]] associated with AA amyloidosis include:<ref name="BlankHegenbart2018">{{cite journal|last1=Blank|first1=Norbert|last2=Hegenbart|first2=Ute|last3=Dietrich|first3=Sascha|last4=Brune|first4=Maik|last5=Beimler|first5=Jörg|last6=Röcken|first6=Christoph|last7=Müller-Tidow|first7=Carsten|last8=Lorenz|first8=Hanns-Martin|last9=Schönland|first9=Stefan O.|title=Obesity is a significant susceptibility factor for idiopathic AA amyloidosis|journal=Amyloid|volume=25|issue=1|year=2018|pages=37–45|issn=1350-6129|doi=10.1080/13506129.2018.1429391}}</ref><ref name="van der HilstYamada2008">{{cite journal|last1=van der Hilst|first1=J. C. H.|last2=Yamada|first2=T.|last3=Op den Camp|first3=H. J. M.|last4=van der Meer|first4=J. W. M.|last5=Drenth|first5=J. P. H.|last6=Simon|first6=A.|title=Increased susceptibility of serum amyloid A 1.1 to degradation by MMP-1: potential explanation for higher risk of type AA amyloidosis|journal=Rheumatology|volume=47|issue=11|year=2008|pages=1651–1654|issn=1462-0324|doi=10.1093/rheumatology/ken371}}</ref><ref name="PapaDoglio2017">{{cite journal|last1=Papa|first1=Riccardo|last2=Doglio|first2=Matteo|last3=Lachmann|first3=Helen J.|last4=Ozen|first4=Seza|last5=Frenkel|first5=Joost|last6=Simon|first6=Anna|last7=Neven|first7=Bénédicte|last8=Kuemmerle-Deschner|first8=Jasmin|last9=Ozgodan|first9=Huri|last10=Caorsi|first10=Roberta|last11=Federici|first11=Silvia|last12=Finetti|first12=Martina|last13=Trachana|first13=Maria|last14=Brunner|first14=Jurgen|last15=Bezrodnik|first15=Liliana|last16=Pinedo Gago|first16=Mari Carmen|last17=Maggio|first17=Maria Cristina|last18=Tsitsami|first18=Elena|last19=Al Suwairi|first19=Wafaa|last20=Espada|first20=Graciela|last21=Shcherbina|first21=Anna|last22=Aksu|first22=Guzide|last23=Ruperto|first23=Nicolino|last24=Martini|first24=Alberto|last25=Ceccherini|first25=Isabella|last26=Gattorno|first26=Marco|title=A web-based collection of genotype-phenotype associations in hereditary recurrent fevers from the Eurofever registry|journal=Orphanet Journal of Rare Diseases|volume=12|issue=1|year=2017|issn=1750-1172|doi=10.1186/s13023-017-0720-3}}</ref>
** Monogenic [[Periodic fever syndrome|periodic fever syndromes]], such as:
{| class="wikitable"
***[[FMF]]
|+
***[[TNF receptor associated periodic syndrome|TNF Receptor associated periodic syndrome]] ([[TRAPS]])
| colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" + |'''Associated Conditions'''
***[[CAPS|Cryopyrin-associated periodic fever syndrome]]
|-
***[[Mevalonate kinase deficiency]]
| style="background: #DCDCDC; padding: 5px; text-align: left;" |'''Conditions'''
**[[Tuberculosis]]
|'''Examples'''
**[[Leprosy]]
|-
** Whipple Disease
| style="background: #DCDCDC; padding: 5px; text-align: left;" |'''Chronic infections'''
**[[Osteomyelitis]]
|
**[[Chronic pyelonephritis]]
*[[Tuberculosis]]
**[[Subacute bacterial endocarditis]]
*[[Leprosy]]
** Chronic cutaneous ulcers
*Whipple Disease
**[[Conditions]] Predisposing to chronic [[infections]] including:
*[[Osteomyelitis]]
***[[Cystic fibrosis]]
*[[Chronic pyelonephritis]]
***[[Bronchiectasis]]
*[[Subacute bacterial endocarditis]]
***[[Kartagener syndrome]]
*Chronic cutaneous ulcers
***[[Epidermolysis bullosa]]
|-
***[[IV drug abusers|Injected drug abuse]]
| style="background: #DCDCDC; padding: 5px; text-align: left;" |'''Monogenic [[Periodic fever syndrome|periodic fever syndromes]]'''
***[[Jejuno-ileal bypass]]
|<br />
***[[Paraplegia]]
*[[TNF receptor associated periodic syndrome|TNF Receptor associated periodic syndrome]] ([[TRAPS]])
***[[Sickle cell anemia]]
*[[CAPS|Cryopyrin-associated periodic fever syndrome]]
***[[Immunodeficiency]]
*[[Mevalonate kinase deficiency]]
***[[Common variable immunodeficiency]]
*[[Familial mediterranean fever]]
***[[Cyclic neutropenia]]
|-
***[[Hyperimmunoglobulin M syndrome]]
| style="background: #DCDCDC; padding: 5px; text-align: left;" |'''Conditions predisposing to recurrent infections'''
***[[Hypogammaglobulinemia]]
|
***[[X-linked agammaglobulinemia|Sex-linked agammaglobulinemia]]
*[[Cystic fibrosis]]
***[[Human Immunodeficiency Virus|Human immunodeficiency virus]]/[[AIDS]]
*[[Bronchiectasis]]
**[[Neoplasia]]
*[[Kartagener syndrome]]
**[[Adenocarcinoma]]
*[[Epidermolysis bullosa]]
**[[Basal cell carcinoma]]
*[[IV drug abusers|Injected drug abuse]]
**[[Carcinoid tumors|Carcinoid tumor]]
*[[Jejuno-ileal bypass]]
**[[Castleman disease]]
*[[Paraplegia]]
**[[Gastrointestinal stromal tumor]]
*[[Sickle cell anemia]]
**[[Hairy cell leukemia]]
*[[Immunodeficiency]]
**[[Hepatic adenoma]]
*[[Common variable immunodeficiency]]
**[[Hodgkin disease]]
*[[Cyclic neutropenia]]
**[[Mesothelioma]]
*[[Hyperimmunoglobulin M syndrome]]
**[[Renal cell carcinoma]]
*[[Hypogammaglobulinemia]]
**[[Sarcoma]]
*[[X-linked agammaglobulinemia|Sex-linked agammaglobulinemia]]
**Inflammatory Arthritis
*[[Human Immunodeficiency Virus|Human immunodeficiency virus]]/[[AIDS]]
** Adult-onset Still disease
|-
**[[Ankylosing spondylitis]]
| style="background: #DCDCDC; padding: 5px; text-align: left;" |'''Neoplasia'''
**[[Juvenile idiopathic arthritis]]
|
**[[Psoriatic arthropathy]]
*[[Adenocarcinoma]]
**[[Reiter's syndrome|Reiter syndrome]]
*[[Basal cell carcinoma]]
**[[Rheumatoid arthritis]]
*[[Carcinoid tumors|Carcinoid tumor]]
**[[Gout]]
*[[Castleman disease]]
** Systemic [[Vasculitis]]
*[[Gastrointestinal stromal tumor]]
** Antineutrophil cytoplasmic antibody-associated vasculitis
*[[Hairy cell leukemia]]
**[[Behcet disease]]
*[[Hepatic adenoma]]
**[[Giant cell arteritis]]
*[[Hodgkin disease]]
**[[Polyarteritis nodosa]]
*[[Mesothelioma]]
**[[Polymyalgia rheumatica]]
*[[Renal cell carcinoma]]
**[[Systemic lupus erythematosus]]
*[[Sarcoma]]
**[[Takayasu arteritis]]
|-
**[[Inflammatory Bowel Disease]]
| style="background: #DCDCDC; padding: 5px; text-align: left;" |'''Inflammatory Arthritis'''
**[[Ulcerative colitis]]
|
**[[Crohn disease]]
*Adult-onset Still disease
* Others include:
*[[Ankylosing spondylitis]]
**[[Atrial myxoma]]
*[[Juvenile idiopathic arthritis]]
** Inflammatory abdominal aortic aneurism
*[[Psoriatic arthropathy]]
**[[Retroperitoneal fibrosis]]
*[[Reiter's syndrome|Reiter syndrome]]
**[[SAPHO syndrome|SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome]]
*[[Rheumatoid arthritis]]
**[[Sarcoidosis]]
*[[Gout]]
**[[Sinus histiocytosis with massive lymphadenopathy]]
|-
| style="background: #DCDCDC; padding: 5px; text-align: left;" |'''Systemic [[Vasculitis]]'''
|
*Antineutrophil cytoplasmic antibody-associated vasculitis
*[[Behcet disease]]
*[[Giant cell arteritis]]
*[[Polyarteritis nodosa]]
*[[Polymyalgia rheumatica]]
*[[Systemic lupus erythematosus]]
*[[Takayasu arteritis]]
*[[Inflammatory Bowel Disease]]
*[[Ulcerative colitis]]
*[[Crohn disease]]
|-
| style="background: #DCDCDC; padding: 5px; text-align: left;" |'''Others'''
|
*[[Atrial myxoma]]
*Inflammatory abdominal aortic aneurism
*[[Retroperitoneal fibrosis]]
*[[SAPHO syndrome|SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome]]
*[[Sarcoidosis]]
*[[Sinus histiocytosis with massive lymphadenopathy]]
|-
|+
 
|-
|+
 
|-
|-
|}
== Gross Pathology ==
== Gross Pathology ==
On [[gross pathology]], the organs affected by amyloidosis can be characterized by the following features:
On [[gross pathology]], the organs affected by amyloidosis can be characterized by the following features:

Revision as of 21:20, 7 November 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]Shaghayegh Habibi, M.D. Sabawoon Mirwais, M.B.B.S, M.D.

Overview

AA amyloid is an abnormal insoluble extracellular protein derived from the spontaneous aggregation of fibrils composed of misfolded proteins, called amyloid precursors. In secondary amyloidosis, the amyloid precursor is SAA, an acute phase reactant, encoded by SAA 1&2 genes. In secondary amyloidosis, intermediate products of SAA metabolism tend to aggregate and form protofilaments that accumulate within extracellular space and form the insoluble amyloid deposits. Secondary amyloidosis is associated with chronic inflammation (such as tuberculosis or rheumatoid arthritis).

Pathophysiology

Associated Conditions

Associated Conditions
Conditions Examples
Chronic infections
Monogenic periodic fever syndromes
Conditions predisposing to recurrent infections
Neoplasia
Inflammatory Arthritis
Systemic Vasculitis
Others

Gross Pathology

On gross pathology, the organs affected by amyloidosis can be characterized by the following features:

  • Porcelain like or waxy appearance
  • Enlargement

Images

Nodular deposits of amyloid on the pleural surfaces.[7]
Cut section of an inguinal lymph node showing firm and waxy consistency.[8]
A slice of the affected node (left) has turned black after treatment with Lugol's solution. A piece of normal myometrium (right) treated similarly with no reaction is also shown.[9]


Microscopic Pathology

On microscopic histopathological analysis, aa amyloidosis is characterized by:[10][11]

  • Green birefringence under polarized light after Congo red staining (appears red under normal light)
  • Linear non-branching fibrils (indefinite length with an approximately same diameter)
  • Distinct X-ray diffraction pattern consistent with Pauling's model of a cross-beta fibril

On electron microscopy, amyloid fibrils have the following characteristics:[12]

  • 10 to 15 nm diameter
  • Straight, rigid, and nonbranching
  • Composed of twisted protofilaments

Images

Small bowel duodenum with amyloid deposition Congo red.[13]
Amyloidosis (black arrows) in a lymph node after staining with Congo Red.[14]
Green birefringence under polarized light.[15]


References

  1. ↑ Jayaraman, Shobini; Gantz, Donald L.; Haupt, Christian; Gursky, Olga (2017). "Serum amyloid A forms stable oligomers that disrupt vesicles at lysosomal pH and contribute to the pathogenesis of reactive amyloidosis". Proceedings of the National Academy of Sciences. 114 (32): E6507–E6515. doi:10.1073/pnas.1707120114. ISSN 0027-8424.
  2. ↑ Kisilevsky, Robert; Raimondi, Sara; Bellotti, Vittorio (2016). "Historical and Current Concepts of Fibrillogenesis and In vivo Amyloidogenesis: Implications of Amyloid Tissue Targeting". Frontiers in Molecular Biosciences. 3. doi:10.3389/fmolb.2016.00017. ISSN 2296-889X.
  3. ↑ Wechalekar AD, Gillmore JD, Hawkins PN (June 2016). "Systemic amyloidosis". Lancet. 387 (10038): 2641–2654. doi:10.1016/S0140-6736(15)01274-X. PMID 26719234.
  4. ↑ Blank, Norbert; Hegenbart, Ute; Dietrich, Sascha; Brune, Maik; Beimler, Jörg; Röcken, Christoph; Müller-Tidow, Carsten; Lorenz, Hanns-Martin; Schönland, Stefan O. (2018). "Obesity is a significant susceptibility factor for idiopathic AA amyloidosis". Amyloid. 25 (1): 37–45. doi:10.1080/13506129.2018.1429391. ISSN 1350-6129.
  5. ↑ van der Hilst, J. C. H.; Yamada, T.; Op den Camp, H. J. M.; van der Meer, J. W. M.; Drenth, J. P. H.; Simon, A. (2008). "Increased susceptibility of serum amyloid A 1.1 to degradation by MMP-1: potential explanation for higher risk of type AA amyloidosis". Rheumatology. 47 (11): 1651–1654. doi:10.1093/rheumatology/ken371. ISSN 1462-0324.
  6. ↑ Papa, Riccardo; Doglio, Matteo; Lachmann, Helen J.; Ozen, Seza; Frenkel, Joost; Simon, Anna; Neven, Bénédicte; Kuemmerle-Deschner, Jasmin; Ozgodan, Huri; Caorsi, Roberta; Federici, Silvia; Finetti, Martina; Trachana, Maria; Brunner, Jurgen; Bezrodnik, Liliana; Pinedo Gago, Mari Carmen; Maggio, Maria Cristina; Tsitsami, Elena; Al Suwairi, Wafaa; Espada, Graciela; Shcherbina, Anna; Aksu, Guzide; Ruperto, Nicolino; Martini, Alberto; Ceccherini, Isabella; Gattorno, Marco (2017). "A web-based collection of genotype-phenotype associations in hereditary recurrent fevers from the Eurofever registry". Orphanet Journal of Rare Diseases. 12 (1). doi:10.1186/s13023-017-0720-3. ISSN 1750-1172.
  7. ↑ By Yale Rosen from USA - Amyloidosis, CC BY-SA 2.0, https://commons.wikimedia.org/w/index.php?curid=31127928
  8. ↑ By Ed Uthman, MD - https://www.flickr.com/photos/euthman/377537238/, CC BY-SA 2.0, https://commons.wikimedia.org/w/index.php?curid=1629764
  9. ↑ By Ed Uthman, MD - https://www.flickr.com/photos/euthman/377538012/, CC BY-SA 2.0, https://commons.wikimedia.org/w/index.php?curid=1629740
  10. ↑
  11. ↑ Röcken C, Shakespeare A (February 2002). "Pathology, diagnosis and pathogenesis of AA amyloidosis". Virchows Arch. 440 (2): 111–122. doi:10.1007/s00428-001-0582-9. PMID 11964039.
  12. ↑ Close, William; Neumann, Matthias; Schmidt, Andreas; Hora, Manuel; Annamalai, Karthikeyan; Schmidt, Matthias; Reif, Bernd; Schmidt, Volker; Grigorieff, Nikolaus; Fändrich, Marcus (2018). "Physical basis of amyloid fibril polymorphism". Nature Communications. 9 (1). doi:10.1038/s41467-018-03164-5. ISSN 2041-1723.
  13. ↑ By Michael Feldman, MD, PhDUniversity of Pennsylvania School of Medicine - http://www.healcentral.org/healapp/showMetadata?metadataId=38717, CC BY 2.0, https://commons.wikimedia.org/w/index.php?curid=870218
  14. ↑ By Ed Uthman, MD - https://www.flickr.com/photos/euthman/377559787/, CC BY-SA 2.0, https://commons.wikimedia.org/w/index.php?curid=1629716
  15. ↑ By Ed Uthman, MD - https://www.flickr.com/photos/euthman/377559955/, CC BY-SA 2.0, https://commons.wikimedia.org/w/index.php?curid=1629705

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