Amyloidosis

A group of sporadic, familial and/or inherited, degenerative, and infectious disease processes, linked by the common theme of abnormal protein folding and deposition of AMYLOID. As the amyloid deposits enlarge they displace normal tissue structures, causing disruption of function. Various signs and symptoms depend on the location and size of the deposits.

Also Known As:

Networked: 7515 relevant articles (352 outcomes, 599 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Familial Mediterranean Fever (Periodic Disease)
2.	Rheumatoid Arthritis
3.	Nephrotic Syndrome (Syndrome, Nephrotic)
4.	Proteinuria
5.	Multiple Myeloma

Experts

1.	Merlini, Giampaolo: 106 articles (12/2015 - 03/2002)
2.	Gertz, Morie A: 100 articles (01/2016 - 01/2002)
3.	Dispenzieri, Angela: 96 articles (01/2016 - 01/2002)
4.	Hawkins, Philip N: 86 articles (12/2015 - 05/2002)
5.	Ando, Yukio: 70 articles (12/2015 - 06/2002)
6.	Seldin, David C: 64 articles (11/2015 - 06/2002)
7.	Palladini, Giovanni: 58 articles (11/2015 - 03/2002)
8.	Naiki, Hironobu: 55 articles (09/2015 - 11/2002)
9.	Gillmore, Julian D: 50 articles (11/2015 - 06/2002)
10.	Lacy, Martha Q: 48 articles (01/2016 - 01/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Amyloidosis:

1.	Amyloid (Amyloid Fibrils)IBA 07/31/1982 - "These observations support the view that major increases in SAA levels are a necessary condition for the deposition of this form of amyloid and suggest that prospective monitoring of the SAA concentration in predisposing diseases may help to identify those individuals who are most at risk for amyloidosis." 09/01/2003 - "Relapse occurred after 4 years of complete remission, and the second renal biopsy revealed amyloid light-chain (AL)-amyloidosis. " 01/01/2013 - "Noninvasive imaging techniques for amyloidosis are beneficial for early and definite diagnosis of amyloid deposition in the body. " 12/01/2008 - "On the other hand, antioxidative intervention studies in animal models of AD-like amyloidosis show a significant reduction in amyloid beta deposition and behavioral improvements. " 09/01/2000 - "The serum HGF level was significantly elevated in both amyloid light-chain and amyloid A amyloidosis and was a very useful indicator of suspected amyloidosis as well as a potential prognostic indicator. "
2.	ColchicineFDA LinkGeneric 08/01/2008 - "Colchicine is highly effective in the treatment of FMF and in preventing the development of recurrent attacks and amyloidosis, and it is essential to make the correct diagnosis and institute daily therapy with colchicine (0.5-0.6 mg bid). " 07/23/2005 - "The curative efficacy of colchicine on flares is debatable, although several studies report positive results against progression of early amyloidosis." 06/01/2015 - "Optimal colchicine dose is effective in preventing and reversing renal amyloidosis. " 05/01/2012 - "When used in proper doses and with compliance, colchicine is very effective in preclinical and proteinuric stages of FMF-related amyloidosis. " 07/31/2004 - "The indication for colchicine is important to consider particularly since the response of amyloidosis to the treatment of the causal disease does not, in the majority of cases, lead to the remission of the amyloidosis, the prognosis of which is determined by the extent of renal involvement."
3.	Melphalan (Alkeran)FDA LinkGeneric 03/01/2014 - "Safety and efficacy of high-dose melphalan and auto-SCT in patients with AL amyloidosis and cardiac involvement." 01/01/2010 - "Aggressive treatment with high-dose i.v. melphalan followed by auto-SCT (HDM/SCT) is effective in inducing hematological and clinical remissions, and in extending survival in AL amyloidosis. " 11/01/2013 - "We designed a trial using two sequential cycles of modified high-dose melphalan at 100 mg/m(2) and autologous SCT (mHDM/SCT) in AL amyloidosis (light-chain amyloidosis, AL), AL with myeloma (ALM) and host-based high-risk myeloma (hM) patients through SWOG-0115. " 11/01/2013 - "Modified high-dose melphalan and autologous SCT for AL amyloidosis or high-risk myeloma: analysis of SWOG trial S0115." 12/15/2012 - "The study group included all 74 patients with AL amyloidosis who underwent high-dose melphalan treatment supported by autologous SCT since the beginning of the Mayo Clinic's SCT program until prior to August 2001. "
4.	Dexamethasone (Maxidex)FDA LinkGeneric 06/10/2007 - "[Progressing primary AL-type amyloidosis benefited by dexamethasone]." 07/01/1999 - "Phase II trial of high-dose dexamethasone for untreated patients with primary systemic amyloidosis." 03/01/2010 - "At present, treatment regimes with high-dose dexamethasone are recommended for patients with primary systemic AL-amyloidosis. " 06/01/2001 - "High-dose dexamethasone (HD-Dex) has been reported to benefit AL amyloidosis patients with varying response rates. " 06/01/2001 - "A modified high-dose dexamethasone regimen for primary systemic (AL) amyloidosis."
5.	bortezomib (Velcade)FDA Link 02/01/2008 - "Bortezomib shows promise in the treatment of systemic AL amyloidosis." 02/01/2008 - "Efficacy of bortezomib in systemic AL amyloidosis with relapsed/refractory clonal disease." 07/01/2015 - "Recent studies support the use of bortezomib-based therapies in light chain amyloidosis (AL). " 10/16/2014 - "Long-term follow-up from a phase 1/2 study of single-agent bortezomib in relapsed systemic AL amyloidosis." 06/07/2012 - "The results of the present study show that RdC is an oral regimen with activity in primary systemic light chain amyloidosis and may be an additional treatment option, especially for patients with preserved organ function or for patients who cannot receive or who relapse after bortezomib. "
6.	tocilizumabFDA Link 01/01/2015 - "In published case reports, tocilizumab (TCZ) has shown good efficacy for AA amyloidosis in almost all patients. " 05/01/2014 - "In the present study, safety and efficacy of 1-year treatment with an anti-IL-6 receptor antibody tocilizumab (TCZ) on RA and AA amyloidosis were estimated. " 01/01/2015 - "AA amyloidosis treated with tocilizumab: case series and updated literature review." 01/01/2014 - "Therapeutic Benefits of Tocilizumab Vary in Different Organs of a Patient with AA Amyloidosis." 01/01/2013 - "Tocilizumab (TCZ) is a humanized anti-IL-6 receptor antibody that has emerged as an effective and specific treatment in AA amyloidosis secondary to chronic inflammatory disorders. "
7.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 03/01/2003 - "The study was undertaken to evaluate the efficacy of cyclophosphamide in primary localised cutaneous amyloidosis. " 02/01/2013 - "Primary localized cutaneous nodular amyloidosis successfully treated with cyclophosphamide." 03/01/2003 - "Treatment of primary localised cutaneous amyloidosis with cyclophosphamide." 01/01/1990 - "Results indicate the significance of immunodepression in amyloidogenesis and a possibility to use the cyclophosphamide-treated great gerbils as a convenient experimental model of amyloidosis." 07/01/2001 - "Renal type AA amyloidosis associated with rheumatoid arthritis: a cohort study showing improved survival on treatment with pulse cyclophosphamide."
8.	Prealbumin (Transthyretin)IBA 02/15/1995 - "Contrary to previous perceptions, there is much recent evidence of effective treatment for several different types of systemic and cardiac amyloidosis, including the most common hereditary form caused by mutations in the transthyretin gene. " 07/01/2015 - "This entity is especially noteworthy in light of emerging treatment options for hereditary transthyretin amyloidosis, which are likely to also be beneficial in wild-type disease." 08/29/2013 - "Safety and efficacy of RNAi therapy for transthyretin amyloidosis." 01/01/1987 - "Amyloidogenesis in the hereditary form of systemic amyloidosis may involve other factors in addition to the presence of a variant precursor prealbumin as indicated by the delayed onset of the disease. " 11/01/2015 - "Differential Myocyte Responses in Patients with Cardiac Transthyretin Amyloidosis and Light-Chain Amyloidosis: A Cardiac MR Imaging Study."
9.	Dimethyl Sulfoxide (DMSO)FDA LinkGeneric 02/15/1989 - "In the case of renal amyloidosis with a creatinin-clearance of more than 10 ml/min, a therapeutic attempt should be made with 1.0 to 1.5 mg/day of colchicin or 10 g/day dimethylsulphoxid (DMSO) for at least six months. " 07/01/2013 - "Dimethyl sulfoxide (DMSO) administered through intravenous,percutaneous or intravesical route has been shown to be effective for treating and preventing recurrence of the disease,but its effect on LUTS due to amyloidosis localized in the urinary bladder has not been demonstrated. " 08/01/1987 - "Amyloidosis presented with whitening and loss of hair which improved after dimethylsulfoxide (DMSO) treatment." 09/01/2012 - "Study on epidemiology of cutaneous amyloidosis in northern India and effectiveness of dimethylsulphoxide in cutaneous amyloidosis." 03/01/2002 - "To evaluate dimethylsulphoxide (DMSO) as a topical therapeutic agent for the treatment of two major variants of primary, localized, cutaneous amyloidosis -- macular amyloidosis (MA) and papular/lichen amyloidosis (LA) -- using a monocentric, open, prospective trial. "
10.	Immunoglobulin Light Chains (Immunoglobulin Light Chain)IBA 06/01/2011 - "Recent advances in the treatment of immunoglobulin light chain amyloidosis (AL) have dramatically improved survival. " 06/01/2012 - "Herein, we demonstrate a case of treated immunoglobulin light chain cardiac amyloidosis with echocardiographic wall thinning and cardiac biopsies demonstrating complete histologic remission. " 08/01/2014 - "Patient survival is superior to renal immunoglobulin light chain-derived amyloidosis and reactive amyloidosis largely due to the absence of cardiac involvement. " 04/01/2009 - "No therapy is uniformly effective in the management of immunoglobulin light chain amyloidosis (AL amyloidosis). " 06/01/2015 - "Natural history and outcomes in localised immunoglobulin light-chain amyloidosis: a long-term observational study."

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 01/01/2001 - "It was found that high-dose chemotherapy with autologous transpantation of haematopoietic cells is at present the most effective treatment for selected patients with not advanced forms of primary systemic AL-amyloidosis. " 01/01/2015 - "Although developments in chemotherapy have greatly improved the outcomes in AL amyloidosis, the prognosis of patients with severe cardiac involvement remains very poor. " 01/01/2014 - "Our results suggest that light chain AL-amyloidosis patients considered fit to undergo HDCT and ASCT may have a better outcome than patients treated exclusively with conventional chemotherapy regimens; however, the better performance status of patients receiving HDCT may have added to this treatment effect." 08/01/2009 - "We performed a systematic review and meta-analysis to evaluate the efficacy of AHCT versus conventional chemotherapy (CC) in patients with AL amyloidosis using methodology recommended by the Cochrane Collaboration. " 12/01/2004 - "These chemotherapies may be effective for reduction of M-protein and are also useful in improving of amyloidosis-induced organ dysfunction. "
2.	Stem Cell Transplantation 12/01/1999 - "Autologous stem cell transplantation (ASCT) is a new treatment option in patients with systemic AL amyloidosis (AL). " 09/01/2010 - "AL amyloidosis in a young adult: remission with autologous stem cell transplantation." 01/01/2008 - "The efficacy of stem cell transplantation in amyloidosis largely is determined by treatment-related mortality rates (range, 11-25%). " 03/20/2012 - "We developed a prognostic model based on 810 patients with newly diagnosed AL amyloidosis, which was further examined in two other datasets: 303 patients undergoing stem-cell transplantation, and 103 patients enrolled onto different clinical trials. " 08/01/2005 - "All patients with AL amyloidosis who underwent autologous stem cell transplantation between 1996 and December 2002 were selected for study. "
3.	Transplants (Transplant) 09/01/2015 - "Our results support the efficacy of this regimen, with high quality responses and prolonged survival, as well as its tolerability, in patients with AL amyloidosis not eligible for stem cell transplant and without advanced cardiac involvement (clinicaltrials.gov identifier: NCT01194791). " 05/01/2012 - "Improvement in myocardial delayed enhancement after autologous stem cell transplant in a patient with light chain amyloidosis." 05/01/2012 - "Quality of life and prognosis of some forms of hereditary systemic amyloidosis can be improved by liver and other organ transplants. " 06/01/2014 - "In conclusion, our study indicates that patients with AL amyloidosis fare well post-transplant relapse/progression. " 01/01/2013 - "Autologous stem cell transplant (ASCT) is now being increasingly used for the treatment of amyloidosis, but most studies are small case series. "
4.	Liver Transplantation 01/01/2008 - "The only effective treatment for the familial TTR amyloidoses is liver transplantation; however, this strategy has a number of limitations, including a shortage of donors, a requirement for surgery for both the recipient and living donor, and the high cost. " 09/01/2015 - "The current standard first-line treatment for hereditary ATTR amyloidosis is liver transplantation, which allows suppression of the main source of variant TTR. " 09/01/2002 - "Liver transplantation: an effective treatment for familial ATTR amyloidosis." 06/01/2014 - "The current standard first-line treatment of familial TTR amyloidosis is liver transplantation. " 05/27/2003 - "Efficacy of liver transplantation in patients with oculoleptomeningeal amyloidosis remains unknown. "
5.	Renal Dialysis (Hemodialysis) 12/01/2007 - "Vertebral involvement with neurological deterioration in hemodialysis-associated amyloidosis patients prompts for early diagnosis, surgical and rehabilitation management, in this instance with good outcome.Spinal" 06/30/2004 - "Daily hemodialysis seems to be more effective in preventing amyloidosis than dialysis three times a week. " 10/01/2001 - "The results indicated that hemodiafiltration therapy and simultaneous hemodialysis with beta(2)-microglobulin adsorption therapy were more effective treatment for dialysis-related amyloidosis." 07/01/2006 - "Beta-2 microglobulin adsorption columns when used along with hemodialysis may lead to lower beta-2 microglobulin levels and improvement in articular manifestations of beta-2 microglobulin amyloidosis. " 07/01/2006 - "The purpose of the study was to obtain information on the survival and the hemodialysis (HD) of patients with amyloidosis. "