Secondary amyloidosis pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[2]Sabawoon Mirwais, M.B.B.S, M.D.[3]
Overview
Amyloid is an abnormal insoluble extracellular protein that deposits in the different tissues and causes organic dysfunction and a wide variety of clinical syndromes. In systemic amyloidosis, amyloid gradually accumulate and amyloid deposition is widespread in the viscera, blood vessel walls, and in the different connective tissues. Secondary amyloidosis is associated with chronic inflammation (such as tuberculosis or rheumatoid arthritis).
Pathophysiology
- Amyloid is an abnormal insoluble extracellular protein that deposits in the different tissues and causes organic dysfunction and a wide variety of clinical syndromes.[1]
- These abnormal amyloids are derived from misfolding and aggregation of normally soluble proteins.
- Amyloid deposition can disrupt tissue structure of involved organ and consequently leads to organ failure.[2]
Secondary Amyloidosis (AA)
- Secondary amyloidosis occurs as a reaction to an existing illness.
- Secondary amyloidosis is associated with chronic inflammation (such as tuberculosis or rheumatoid arthritis).[3]
- Secondary or reactive amyloidosis (AA) comprises approximately 45% of the systemic amyloidoses.
- Pathogenesis of secondary amyloidosis is multifactorial, including:
- Primary structure of the precursor protein
- Acute phase response
- Nonfibril proteins (amyloid P component, apo E, GAGs, proteoglycans and basement membrane proteins)
- Receptors
- Lipid metabolism
- Proteases
Associated Conditions
- Conditions associated with AA amyloidosis include:[4][5][6]
- Monogenic periodic fever syndromes, such as:
- Tuberculosis
- Leprosy
- Whipple Disease
- Osteomyelitis
- Chronic pyelonephritis
- Subacute bacterial endocarditis
- Chronic cutaneous ulcers
- Conditions Predisposing to chronic infections including:
- Cystic fibrosis
- Bronchiectasis
- Kartagener syndrome
- Epidermolysis bullosa
- Injected drug abuse
- Jejuno-ileal bypass
- Paraplegia
- Sickle cell anemia
- Immunodeficiency
- Common variable immunodeficiency
- Cyclic neutropenia
- Hyperimmunoglobulin M syndrome
- Hypogammaglobulinemia
- Sex-linked agammaglobulinemia
- Human immunodeficiency virus/AIDS
- Neoplasia
- Adenocarcinoma
- Basal cell carcinoma
- Carcinoid tumor
- Castleman disease
- Gastrointestinal stromal tumor
- Hairy cell leukemia
- Hepatic adenoma
- Hodgkin disease
- Mesothelioma
- Renal cell carcinoma
- Sarcoma
- Inflammatory Arthritis
- Adult-onset Still disease
- Ankylosing spondylitis
- Juvenile idiopathic arthritis
- Psoriatic arthropathy
- Reiter syndrome
- Rheumatoid arthritis
- Gout
- 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
- Others include:
- Atrial myxoma
- Inflammatory abdominal aortic aneurism
- Retroperitoneal fibrosis
- SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome
- Sarcoidosis
- Sinus histiocytosis with massive lymphadenopathy
Gross Pathology
On gross pathology, the organs affected by amyloidosis can be characterized by the following features:
- Porcelain like or waxy appearance
- Enlargement
Images
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Microscopic Pathology
On microscopic histopathological analysis, amyloidosis is characterized by:[3][10]
- 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
Images
References
- ↑ 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.
- ↑ 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.
- ↑ 3.0 3.1 Khan MF, Falk RH (November 2001). "Amyloidosis". Postgrad Med J. 77 (913): 686–93. PMC 1742163. PMID 11677276.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ By Yale Rosen from USA - Amyloidosis, CC BY-SA 2.0, https://commons.wikimedia.org/w/index.php?curid=31127928
- ↑ 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
- ↑ 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
- ↑ 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.
- ↑ 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
- ↑ 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
- ↑ 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