Pathogenesis of systemic and likalized amyloidoses  Page description

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Details of project

  
Identifier
46385
Type K
Principal investigator Bély, Miklós
Title in Hungarian A szisztémás és lokalizált amyloidosisok pathogenesise
Title in English Pathogenesis of systemic and likalized amyloidoses
Panel Physiology, Pathophysiology, Pharmacology and Endocrinology
Department or equivalent Polyclinic of the Hospitaller Brothers of St. John of God in Budapest
Participants Apáthy, Ágnes
Starting date 2004-01-01
Closing date 2008-12-31
Funding (in million HUF) 6.037
FTE (full time equivalent) 0.00
state closed project





 

Final report

  
Results in Hungarian
A kutatás célja a szisztémás és a lokalizált amyloidosisok kórlefolyásának a jellemzése volt. 1. Megállapítást nyert, hogy az amyloid lerakódása valamennyi vizsgált kórfolyamatban progresszív, kumulatív folyamat. 2. Igazoltuk, hogy a szisztémás amyloidosisok korai stádiumában csak néhány szerv, egy-egy szöveti struktúráján mutatható ki az amyloid lerakódása. Előrehaladott stádiumban szinte valamennyi szerv számos szöveti struktúráján igazolható az amyloid depositio. 3. Fentiek alapján megfogalmazást nyert a lerakódás sorrendiségére vonatkozó elv: „ami a leggyakoribb, az a legkorábbi”. 4. Igazoltuk (Bély és Apáthy 2006), hogy a korai „friss” depositumok könnyebben roncsolhatóak, mint a régi, „idősült” amyloid depositumok, azaz az amyloid depositumok szerkezetében – a kórfolyamat stádiumától függően – strukturális átalakulás zajlik. A friss és az idősült amyloid depositumok között mutatkozó histochemiai különbségek azt jelentik, hogy a lerakódott amyloid stadium fügő átalakulást (“maturáció”-t) mutat. Következtetés: Törekedni kell a korai diagnózisra, mert csak a korai stádiumban várható, hogy könnyebb a viszonylag lazább struktúrájú amyloid depozitumok mobilizációja, azaz a korai stádiumban felismert amyloidosisok esetében inkább várható a sikeres terápia. Előrehaladott stádiumra az idősült amyloid depositumokra a viszonylag stabil, terápia rezistens megejelenése jellemző. A resistencia, illetve szenzitivitás meghatározásának prognosztikus jelentősége van.
Results in English
The aim of this project was to characterize the pathogenesis of systemic and localized amyloidoses. 1. Amyloid deposition in all investigated diseases was found to be a progressive cumulative process. In its early stage amyloid involved only a few tissue structures in some organs, and increasingly more in later stages of the disease. 2. The early stage of amyloidosis is characterized by minimal or moderate amyloid deposits, involving only a few tissue structures in some organs. Massive amyloid deposits, extensively involving many tissue structures in most organs of the patients, represent a late (advanced) stage of amyloidosis. 3. During the progression of systemic amyloidosis, amyloid protein deposition begins in the organs and tissue structures that are frequently involved and later show marked deposition. Where deposits are infrequent or less marked, deposition starts later. 4. The early amyloid deposits may be distinguished histochemically from old ones. The histochemical differences between fresh and old amyloid deposits point to the age-dependent maturation of deposited amyloid. Conclusions In early stages, amyloid A deposits are fewer in number and smaller in size, as well as structurally looser and more soluble. For this reason, therapy is more likely to be successful in early stages of amyloidosis than in advanced stages when amyloid deposits are massive, dense, and less soluble. The sensitivity or resistance of amyloid deposits may be used as a prognostic marker in RA.
Full text http://real.mtak.hu/1411/
Decision
Yes





 

List of publications

  
20. Apáthy Ágnes, Bély M: Amyloidosis, In: Klinikai reumatológia (Ed.: Gömör B) pp.:832-839. Medicina 2005., 2005
14. Bély M, Apáthy Ágnes: Prevalence and severity of AA amyloidosis in Rheumatoid Arthritis - A Retrospective Clinicopathologic Study of 161 Autopsy, Virchows Archiv, 447, 442-443 IF:2.224, 2005
15. Bély M, Apáthy Ágnes: Chronologic Sequence of AA amyloidosis in Rheumatoid Arthritis - A Retrospective Clinicopathologic Study of 161 Autopsy Patients, Virchows Archiv, 447, 498-499, IF:2.224, 2005
11. Bély M, Apáthy Ágnes: Associated Diseases in Rheumatoid Arthritis – A Retrospective Clinicopathologic Study of 234 Autopsy Patients, Ann Rheum Dis 64, Suppl 3, 177, (IF: 6,956) http://www.abstracts2view.com/eular/, 2005
12. Bély M, Apáthy Ágnes: Lethal complications of Rheumatoid Arthritis – A Retrospective Clinicopathologic Study of 234 Autopsy Patients, Ann Rheum Dis 64, Suppl 3, 178, (IF: 6,956) http://www.abstracts2view.com/eular/, 2005
27. Bély M, Apáthy Ágnes: Atherosclerosis, Hypertension and Diabetes Mellitus in Rheumatoid Arthritis - A Retrospective Clinicopathologic - Statistical Study of 234 Autopsy Patients (Abstract), Annals Rheum Dis 65. Suppl.11. 162-163 (2006) (IF:5.767) http://www.abstracts2view.com/eular/, 2006
28. Bély M, Apáthy Ágnes: Atherosclerosis, Vasculitis, Amyloidosis and Septic Infection in Rheumatoid Arthritis - A Retrospective Clinicopathologic-Statistical Study of 234 Autopsy Patients, Annals Rheum Dis 65. Suppl.11. 162 (2006).(IF:5.767) http://www.abstracts2view.com/eular/, 2006
25. Bély M, Apáthy Ágnes: Histology and immunohistochemical characteristics of temporal arteritis, Zdravniski vestnik, Suppl.1. 10, 76 (2006), 2006
26. Bély M, Apáthy Ágnes: Tumors in Rheumatoid Arthritis - A Retrospective Clinicopathologic Study of 234 Autopsy Patients. (Abstract), Annals Rheum Dis 65. Suppl.11. 162 (2006) (IF:5.767) http://www.abstracts2view.com/eular/, 2006
29. Bély M, Apáthy Ágnes: Systemic and Coronary Vasculitis in Rheumatoid Arthritis - A Retrospective Clinicopathologic - Statistical Study of 234 Autopsy Patients (Abstract), Annals Rheum Dis 65. Suppl.11. 165 (2006) (IF:5.767) http://www.abstracts2view.com/eular/, 2006
31. Bély M, Apáthy Ágnes: Vészhelyzetek rheumatoid arthritisben - 234 elhunyt beteg patológiai és klinikai adatainak retrospektív elemzése alapján, Magyar Reumatológia 47, 186-187 (2006), 2006
30. Bély M, Apáthy Ágnes: Pathogenesis Silent Myocardial Infarction in Rheumatoid Arthritis - A Retrospective Clinicopathologic - Statistical Study of 234 Autopsy Patients (Abstract), Annals Rheum Dis 65. Suppl.11. 164-165 (2006) (IF:5.767) http://www.abstracts2view.com/eular/, 2006
35. Gelley A, Kapp P, Bély M, Balázs C: Amyloid AA deposits induce an ischaemic chronic ulcer in rheumatoid arthritis (Abstract), Amyloid: J. Protein Folding Disord. 13, Suppl.1. 30 (2006) (IF:1.910), 2006
37. Bély M, Apáthy Ágnes: Characteristics of Systemic Vasculitis in Progressive Systemic Sclerosis – A retrospective clinicopathologic study of 11 autopsy patients (Abstract), Annals Rheum Dis 66. Suppl.II. 201 (2007) (IF:5.767) http://www.abstracts2view.com/eular/, 2007
36. Apáthy Ágnes, Bély M: Mortality and histological characteristics of SSc - A retrospective study of 12 autopsy patients (Abstract), Annals Rheum Dis 66. Suppl.II. 198-199 (2007) (IF:5.767) http://www.abstracts2view.com/eular/, 2007
38. Bély M, Apáthy Ágnes: Vasculitis and Other Changes of Blood Vessels in Rheumatoid Arthritis and Systemic Sclerosis – A Comparative Histologic Study of 36 RA and 11 SSc Autopsy Patients with Systemic Vasculitis (Abstract), Annals Rheum Dis 66. Suppl.II. 375 (2007) (IF:5.767) http://www.abstracts2view.com/eular/, 2007
39. Bély M, Apáthy Ágnes: Clinical Significance of Stage-Dependent Qualitative Differences of (AA) Amyloid Deposits in Rheumatoid Arthritis - A retrospective clinicopathologic study of 161 autopsy patients (Abstract), Annals Rheum Dis 66. Suppl.II. 591 (2007) (IF:5.767) http://www.abstracts2view.com/eular/, 2007
40. Apáthy Ágnes, Bély M: Amyloidosis in Rheumatoid Arthritis - Clinical-laboratory parameters, demographics and duration of disease associated with AA amyloidosis - A retrospective clinicopathologic study of 161 autopsy patients (Abstract), Annals Rheum Dis 66. Suppl.II. 594 (2007) (IF:5.767) http://www.abstracts2view.com/eular/, 2007
41. Apáthy Ágnes, Bély M: Vasculitis in Rheumatoid Arthritis - Clinical-laboratory parameters, demographics and duration of disease associated with systemic vasculitis - A retrospective clinicopathologic study of 161 autopsy patients (Abstract), Annals Rheum Dis 66. Suppl.II. 594 (2007) (IF:5.767) http://www.abstracts2view.com/eular/, 2007
42. Bély M, Apáthy Ágnes: Systemic vasculitis in progressive systemic sclerosis: The frequency, severity and stages of vasculitis in various organs - A retrospective clinicopathologic study of 11 autopsy patients (Abstract), Annals Rheum Dis 66. Suppl.II. 609 (2007) (IF:5.767) http://www.abstracts2view.com/eular/, 2007
43. Bély M, Apáthy Ágnes: Lethal complications of Rheumatoid Arthritis -A Retrospective Clinicopathologic Study of 161 Autopsy Patients, Virchows Archiv, 449, 471 (2007) (IF:2.251), 2007
44. Bély M, Apáthy Ágnes: The Mortality and Clinically Missed Diagnosis of AA Amyloidosis in Rheumatoid Arthritis – A Retrospective Clinicopathologic Study of 161 Autopsy Patients, Virchows Archiv, 449, 509 (2007) (IF:2.251), 2007
45. Bély M, Apáthy Ágnes: Stage Dependent Quantitative Differences of (AA) Amyloidosis in Rheumatoid Arthritis – A Retrospective Clinicopathologic Study of 161 Autopsy Patients, Virchows Archiv, 449, 509-510 (2007) (IF:2.251), 2007
46. Apáthy Ágnes, Bély M: Clinical Significance of Stage-Dependent Qualitative Differences of Amyloid A Deposits in Rheumatoid Arthritis - A retrospective clinicopathologic study of 161 autopsy patients, Virchows Archiv, 449, 287 (2007) (IF:2.251), 2007
47. Bély M, Apáthy Ágnes: Systemic and Coronary Vasculitis in Rheumatoid Arthritis - A Retrospective Clinicopathologic - Statistical Study of 234 Autopsy Patients, Virchows Archiv, 449, 480 (2007) (IF:2.251), 2007
48. Bély M, Apáthy Ágnes: Pathogenesis and Silent Myocardial Infarction in Rheumatoid Arthritis - A Retrospective Clinicopathologic Study of 161 Autopsy Patients, Virchows Archiv, 449, 451 (2007) (IF:2.251), 2007
1. Bély M; Apáthy Ágnes: Clinical significance of stage-dependent qualitative differences of (AA) amyloid deposits in rheumatoid arthritis – a preliminary report, Magyar Reumatol, Supplementum 45: 74-75 (2004), 2004
2. Bély M; Apáthy Ágnes: Tuberculosis in Rheumatoid Arthritis - A Retrospective Clinicopathologic Study of 161 Autopsy Patients (Abstract), Ann Rheum Dis 63, Suppl 1, 478-479 (2004) (IF: 3.916). http://www.abstracts2view.com/eular/, 2004
3. Bély M; Apáthy Ágnes: Mortality of Tuberculosis in Rheumatoid Arthritis. A Retrospective Clinicopathologic Study of 161 Autopsy Patients. (Abstract), Ann Rheum Dis 63, Suppl 1: 308 (2004) (IF: 3.916). http://www.abstracts2view.com/eular/, 2004
4. Bély M; Apáthy Ágnes: Miliary Dissemination of Tuberculosis in Rheumatoid Arthritis - Clinical-laboratory parameters, demographics and duration of disease associated with miliary tuberculosis. A retrospective clinicopathologic study of 161 autopsy patients. (Abstract), Ann Rheum Dis 63, Suppl 1: 167 (2004) (IF: 3.916). http://www.abstracts2view.com/eular/, 2004
5. Bély M; Apáthy Ágnes: Tumors in Rheumatoid Arthritis - A Retrospective Clinicopathologic Study of 161 Autopsy Patients. (Abstract), Ann Rheum Dis 63, Suppl 1: 539 (2004) (IF: 3.916). http://www.abstracts2view.com/eular/, 2004
19. Bély M: Funkcionális patomorfológia, In: Klinikai reumatológia (Ed.: Gömör B) pp.:39-53. Medicina 2005, 2005
6. Bély M; Apáthy Ágnes: Szeptikus infekció rheumatoid arthritisben, LAM 14: 8857-866 (2004), 2004
17. Bély M; Apáthy Á; Lakatos T: Systemic b2-Microglobulin-Related Amyloidosis in a Patient with Chronic Renal Failure...–A Light and Electronmicroscopic Study, In: Grateau G, Kyle RA, Skinner M (ed.) Amyloid and Amyloidosis. London, New York, Washington: CRC Press, Boca Raton 2005. pp. 429-431, 2005
22. Bély M; Apáthy Ágnes: The incidence, and polarization optical characteristics of pulmonary alveolar calcospherities (corpora amylacea) in rheumatoid arthritis, – In: Grateau G, Kyle RA, Skinner M (ed.) Amyloid and Amyloidosis. London, New York, Washington: CRC Press, Boca Raton 2005. pp. 263-265, 2005
16. Bély M, Kapp P, Szentjóbi Szabó T, Lakatos T, Apáthy Á: Electron microscopic characteristics of b2-microglobulin amyloid deposits in long term haemodialysis, Ultrastructural Pathol 29, 1-9 (IF:0.694), 2005
13. Kiss G, Kelemen J, Bély M, Vértes P: Clinically diagnosed fatal cerebral vasculitis in long-standing juvenile rheumatoid arthritis, Virchows Arch 448, 381-383 (2005). (IF:2.442), 2005
24. Bély M, Makovitzky J: Sensitivity and Specificity of Congo red Staining According to Romhányi - Comparison with Puchtler's or Bennhold's Methods, Acta Histochemica 108, 175-180 (2006) (IF:0.920), 2006
23. Bély M: Histochemical differential diagnosis and polarization optical analysis of amyloid and amyloidosis: in memoriam Professor G Romhányi (09. 15. 1905 – 08. 29. 1991), TheScientificWorldJOURNAL 6, 154–168. DOI, 2006
32. Bély M, Apáthy Ágnes: Szövődmények és társult megbetegedések rheumatoid arthritisben - A 234 elhunyt beteg patológiai és klinikai adatainak retrospektív elemzése alapján, Orvosi Hetilap 147, 1063-1076 (2006), 2006
33. Bély M, Apáthy Ágnes: Histochemical and Immunohistochemical Characteristics of Amyloid Deposits (Abstract), Amyloid: J. Protein Folding Disord. 13, Suppl.1. 16 (2006) (IF:1.910), 2006
34. Bély M, Apáthy Ágnes, Kapp P: Ultrastructural Characteristics of Amyloid Deposits (Abstract), Amyloid: J. Protein Folding Disord. 13, Suppl.1. 18 (2006) (IF:1.910), 2006



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