DefinitionThis section has been translated automatically.
Systemic amyloidosis with deposition of amyloid A protein (AA), a fragment of serum amyloid A protein (SAA). One speaks of idiopathic AA amyloidosis in the absence of a recognizable underlying disease.
Occurrence/EpidemiologyThis section has been translated automatically.
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EtiopathogenesisThis section has been translated automatically.
- Mostly caused by chronic inflammation, also occurring during long-term dialysis or hereditary diseases.
- Reactive overproduction of SAA (an acute phase protein formed in the liver) in chronic inflammatory processes, e.g. Tuberculosis, osteomyelitis, syphilis, rheumatoid arthritis, Reiter's syndrome, ulcerative colitis, chronic glomerulonephritis, bronchiectasis, empyema, acne conglobata, epidermolysis bullosa dystrophica, Hallopeau-Siemens, familial Mediterranean fever, Muckle-Wells syndrome In some patients, the increased SAA production is broken down by monocytes to amyloid A and deposited.
Clinical featuresThis section has been translated automatically.
- Integument: Skin symptoms are generally rather rare. The occurrence of bleeding and alopecia was described by amyloid deposits in the dermis and subcutis.
- The skin symptoms are accompanied by unspecific general symptoms caused by the primary underlying disease.
- Extracutaneous manifestation: Cardinal symptom: Progressive renal insufficiency due to amyloid deposits in the basal membrane of the glomerula. Deposits also in the liver, gastrointestinal tract, adrenal glands and CNS.
HistologyThis section has been translated automatically.
TherapyThis section has been translated automatically.
LiteratureThis section has been translated automatically.
- Akpolat T et al (2000) Behcet's disease and AA-type amyloidosis. At J Nephrol 20: 68-70
- Boorjian S (2002) A rare case of painless gross hematuria: primary localized AA-type amyloidosis of the urinary bladder. Urology 59: 137
- Cohen AS, Jones LA (1991) Amyloidosis. Curr Opin Rheumatol 3: 125-138
- Cohen AS, Jones LA (1993) Advances in Amyloidosis. Curr Opin Rheumatol 5: 62-76
- Csikos M et al (2003) Dystrophic epidermolysis bullosa complicated by cutaneous squamous cell carcinoma and pulmonary and renal amyloidosis. Clin Exp Dermatol 28: 163-166
- Escriba A et al (2000) Secondary (AA-type) amyloidosis in patients with polymyalgia rheumatica. At J Kidney Dis 35: 137-140
- Hazenberg BP et al (2007) Diagnostic performance of amyloid A protein quantification in fat tissue of patients with clinical AA amyloidosis. Amyloid 14: 133-140
- Komatsuda A et al (2003) Amyloid A-type renal amyloidosis in a patient with sarcoidosis: report of a case and review of the literature. Clin Nephrol 60: 284-288
- Lubarsch O (1899) Hyaline and amyloid degeneration. Erg allg Path 4: 449-460
- Picken MM (2007) New insights into systemic amyloidosis: the importance of diagnosis of specific type. Curr Opin Nephrol Hypertens 16: 196-203
- Ruzicka T et al (1990) Cutaneous amyloidosis. Dermatologist 41: 245-255
- Virchow R (1854) On the course of amyloid degeneration. Virch Arch 8: 364-368
Outgoing links (12)Acne conglobata; Amyloid; Amyloidosis (overview); Amyloidosis systemic (overview); Basal membrane; Colchicine; Cutaneous tuberculosis (overview); Epidermolysis bullosa dystrophica recessive, severe generalized; Muckle-wells syndrome; Reiter`s syndrome; ... Show all
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