490 results match your criteria Amyloidosis AA Inflammatory


[Local and systemic light chain amyloidosis in patients with rheumatic diseases].

Z Rheumatol 2020 Aug 7. Epub 2020 Aug 7.

Medizinische Klinik 5 und Amyloidose-Zentrum, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 410, 69115, Heidelberg, Deutschland.

Background: In patients with chronic inflammatory rheumatic diseases various types of amyloidosis diseases can occur. If amyloidosis is suspected a differentiation between local and systemic amyloid deposits needs to be made as well as between AL, AA and other forms of amyloidosis.

Objective: The aim is the characterization of local and systemic AL amyloidosis in rheumatic diseases, demonstration of diagnostic algorithms and prognostic factors as well as a discussion of the treatment options. Read More

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http://dx.doi.org/10.1007/s00393-020-00848-6DOI Listing

Amyloid A Amyloidosis After Renal Transplantation: An Important Cause of Mortality.

Transplantation 2020 Aug;104(8):1703-1711

Division of Nephrology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.

Background: There are limited data on the outcome of transplant recipients with familial Mediterranean fever (FMF)-associated AA amyloidosis. The aim of the present study is to evaluate demographic, clinical, laboratory, and prognostic characteristics and outcome measures of these patients.

Methods: Eighty-one renal transplant recipients with FMF-associated AA amyloidosis (group 1) and propensity score-matched transplant recipients (group 2, n = 81) with nonamyloidosis etiologies were evaluated in this retrospective, multicenter study. Read More

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http://dx.doi.org/10.1097/TP.0000000000003043DOI Listing

AA Amyloid Deposition in the Central and Peripheral Nervous Systems in Flamingos.

Vet Pathol 2020 Jul 17:300985820939976. Epub 2020 Jul 17.

Tokyo University of Agriculture and Technology, Tokyo, Japan.

AA amyloidosis is characterized by amyloid deposition in systemic organs, but amyloid deposition in the central nervous system (CNS) or peripheral nervous system (PNS) is rare. In this study, AA amyloidosis was observed in 31 of 48 flamingos that died at a Japanese zoo. Almost all cases developed AA amyloidosis secondary to inflammatory diseases such as enteritis. Read More

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http://dx.doi.org/10.1177/0300985820939976DOI Listing

Late Acute Cellular Rejection After Anakinra Treatment in a Kidney Transplant Patient, Is It a Coincidence?

Iran J Kidney Dis 2020 Jul;14(4):318-320

Department of Nephrology, Gazi University, Ankara, Turkey.

Familial mediterranean fever (FMF) is an autosomal recessive auto-inflammatory disorder, which could lead to secondary (AA) amyloidosis. Anakinra is an IL-1 receptor blocker and a treatment option for patients with FMF. There is no reported rejection episode associated with the use of Anakinra in the literature. Read More

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July 2020
0.979 Impact Factor

Secondary bladder amyloidosis due to Crohn's disease: a case report and literature review.

CEN Case Rep 2020 Jun 22. Epub 2020 Jun 22.

Department of Urology, S. Orsola-Malpighi University Hospital, Via P. Palagi 9, 40138, Bologna, Italy.

The presence of amyloid deposits in bladder walls is a rare histological finding. It can be linked to primary (limited to bladder) or secondary (systemic, associated with chronic inflammatory disorders) amyloidosis. Secondary bladder involvement is very uncommon; it usually presents with gross hematuria, which is challenging to manage, due to frail bladder mucosa and/or necrosis. Read More

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http://dx.doi.org/10.1007/s13730-020-00497-3DOI Listing

Solid Organ Transplantation in Amyloidosis.

Acta Haematol 2020 12;143(4):352-364. Epub 2020 Jun 12.

Plasma Cell Dyscrasia Unit, Department of Clinical Therapeutics, National and Kapodistrian University of Athens, Athens, Greece,

Amyloidosis comprises a diverse group of diseases characterized by misfolding of precursor proteins which eventually form amyloid aggregates and preceding intermediaries, which are deposited in target tissues causing progressive organ damage. In all forms of amyloidosis, vital organs may fail; depending on the specific amyloidosis type, this may occur rapidly or progress slowly. Beyond therapies to reduce the precursor protein (chemotherapy for light chain [AL] amyloidosis, anti-inflammatory therapy in serum A amyloid-osis [AA], and antisense RNA therapy in transthyretin amyloidosis [ATTR]), organ transplantation may also be a means to reduce amyloidogenic protein, e. Read More

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http://dx.doi.org/10.1159/000508262DOI Listing

A histopathological scoring and grading system to predict outcome for patients with AA amyloidosis.

Int Urol Nephrol 2020 Jul 11;52(7):1297-1304. Epub 2020 Jun 11.

Division of Nephrology, Department of Internal Medicine, School of Medicine, Ege University, Bornova, 35100, Izmir, Turkey.

Purpose: Renal involvement is associated with significant morbidity and mortality in AA amyloidosis. Extend of amyloid deposition in kidney biopsies may be predictive for clinical manifestations and outcomes. The aim of our study is to assess clinical features of patients with biopsy-proven renal AA amyloidosis and to evaluate the relationship between histopathological scoring and grading of renal amyloid deposition with clinical findings and outcomes. Read More

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http://dx.doi.org/10.1007/s11255-020-02505-yDOI Listing

Familial Mediterranean Fever Complicated by a Triad of Adrenal Crisis: Amyloid Goiter and Cardiac Amyloidosis.

Case Rep Rheumatol 2020 19;2020:7865291. Epub 2020 May 19.

Medicine Department, Ahli Hospital, Hebron, Palestine, Israel.

Background: FMF is a common disease in the Mediterranean populations and may be complicated by AA amyloidosis. The coexistence of adrenal and thyroid amyloidosis in AA amyloidosis secondary to familial Mediterranean fever (FMF) is an extremely rare reported condition. We presented a previously unreported triad of adrenal, thyroid, and cardiac amyloidosis secondary to FMF. Read More

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http://dx.doi.org/10.1155/2020/7865291DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7256690PMC

AA amyloidosis associated with Fabry disease.

Int J Clin Pract 2020 Jun 9:e13577. Epub 2020 Jun 9.

Sorbonne Université, AP-HP, Hôpital Tenon, Centre de référence des maladies auto-inflammatoires et des amyloses d'origine inflammatoire (CEREMAIA), Service de médecine interne, F-75020, Paris, France.

Background: Fabry disease is the second most common lysosomal storage disorder, carrying a large morbidity and mortality. It has been recently reported that lysosomal storage disorders could cause inflammation and, subsequently, AA amyloidosis. Our aim was to describe AA amyloidosis cases occurring in the course of Fabry disease. Read More

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http://dx.doi.org/10.1111/ijcp.13577DOI Listing

Approach to the Diagnosis of Amyloidosis.

Indian J Hematol Blood Transfus 2020 Apr 9;36(2):246-253. Epub 2019 Oct 9.

Department of Hematology, AIIMS, New Delhi, India.

Amyloidosis is heterogeneous group of disorder characterized by extracellular deposition of misfolded insoluble proteinaceous material with cross beta pleated sheet structure leading to organ dysfunction. This disease is rare and indeed heterogeneous, as it may be hereditary (familial amyloidosis), secondary to spectrum of inflammatory conditions (AA amyloidosis) or member of plasma cell neoplasm family (AL amyloidosis). AL amyloidosis is the most common type of amyloid, however, is rarely accompanied by multiple myeloma or other lymphoproliferative disorder. Read More

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http://dx.doi.org/10.1007/s12288-019-01208-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7229097PMC

Revisiting TNF Receptor-Associated Periodic Syndrome (TRAPS): Current Perspectives.

Int J Mol Sci 2020 May 5;21(9). Epub 2020 May 5.

Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892, USA.

Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is an autosomal dominant autoinflammatory syndrome characterized by prolonged and recurrent episodes of fever, abdominal and/or chest pain, arthralgia, myalgia, and erythematous rash. TRAPS is associated with heterozygous variants in the gene, which encodes the TNFR1 (tumor necrosis factor receptor 1) receptor. Disease-causing variants are found exclusively in the extracellular domain of TNFR1 and affect receptor structure and binding to the TNF ligand. Read More

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http://dx.doi.org/10.3390/ijms21093263DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7246474PMC

Acquired and inherited amyloidosis: Knowledge driving patients' care.

J Peripher Nerv Syst 2020 06;25(2):85-101

National Reference Center for Familial Amyloid Polyneuropathy and Other Rare Neuropathies, APHP, Université Paris Saclay, INSERM U1195, Le Kremlin Bicêtre, France.

Until recently, systemic amyloidoses were regarded as ineluctably disabling and life-threatening diseases. However, this field has witnessed major advances in the last decade, with significant improvements in therapeutic options and in the availability of accurate and non-invasive diagnostic tools. Outstanding progress includes unprecedented hematological response rates provided by risk-adapted regimens in light chain (AL) amyloidosis and the approval of innovative pharmacological agents for both hereditary and wild-type transthyretin amyloidosis (ATTR). Read More

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http://dx.doi.org/10.1111/jns.12381DOI Listing

Soluble TREM-1 Levels in Familial Mediterranean Fever Related AA-Amyloidosis.

Immunol Invest 2020 Apr 22:1-9. Epub 2020 Apr 22.

Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul-Cerrahpasa, Istanbul, Turkey.

: Triggering Receptor Expressed on Myeloid cells-1 (TREM-1) is a monocyte and neutrophil receptor functioning in innate immunity. TREM-1 activity has been studied in various autoimmune diseases such as RA and SLE but there is no data in autoinflammatory pathologies. We studied soluble TREM-1 (sTREM-1) activity in Familial Mediterranean Fever (FMF) cases to evaluate the clinical role of TREM-1 in amyloidosis. Read More

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http://dx.doi.org/10.1080/08820139.2020.1751195DOI Listing

Colonoscopy Leading to the Diagnosis of AL Amyloidosis in the Gastrointestinal Tract Mimicking an Acute Ulcerative Colitis Flare.

ACG Case Rep J 2019 Nov 27;6(11):e00289. Epub 2019 Nov 27.

Department of Pathology, Kaiser Permanente Riverside Medical Center, Riverside, CA.

The 2 most common types of amyloidosis are light chain (AL) and reactive (AA). AL is associated with plasma cell dyscrasias; reactive (AA) is associated with chronic inflammatory conditions. A few cases have described AL amyloidosis mimicking colitis. Read More

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http://dx.doi.org/10.14309/crj.0000000000000289DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7145203PMC
November 2019

[Autoinflammatory syndromes and AA amyloidosis].

Z Rheumatol 2020 Apr 6. Epub 2020 Apr 6.

Amyloidose-Zentrum, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Deutschland.

Autoinflammatory syndromes (AIS) are characterized by uniform attacks often with febrile episodes, exanthema, abdominal pain, muscle and joint pain. Patients show markedly elevated levels of the inflammatory serum parameters C‑reactive protein (CRP) and systemic amyloid A (SAA) during an attack. The origin of the family of the patient and the duration of the attacks are helpful to find the appropriate diagnosis. Read More

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http://dx.doi.org/10.1007/s00393-020-00778-3DOI Listing

AA amyloidosis revealing mevalonate kinase deficiency: A report of 20 cases including two new French cases and a comprehensive review of literature.

Semin Arthritis Rheum 2020 Mar 19. Epub 2020 Mar 19.

Internal medicine department, Tenon hospital, Assistance Publique - Hôpitaux de Paris, Sorbonne University, 4 rue de la Chine, 75020 Paris, France. Electronic address:

Introduction: Mevalonate kinase deficiency (MKD) is a rare autosomal recessive autoinflammatory disease that can lead to an inflammatory A amyloidosis (AA).

Methods: To study the occurrence of AA in MKD patients we performed a systemic review of the literature and described two novel patients.

Results: Amyloidosis occurred in 20 MKD patients, renal impairment being always the revealing symptom of AA. Read More

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http://dx.doi.org/10.1016/j.semarthrit.2020.03.005DOI Listing

Kidney Transplantation in Systemic Amyloidosis.

Transplantation 2020 Feb 11. Epub 2020 Feb 11.

Renal Section, Department of Medicine, Boston University School of Medicine, Boston, MA, USA.

The present review discusses current developments and outcomes of renal transplantation in systemic amyloidosis. Amyloidosis can wreak havoc on the architecture and functioning of the kidneys, leading to end stage renal disease (ESRD). In recent years, the available treatments, especially for AL amyloidosis but also for several of the underlying inflammatory diseases that cause AA amyloidosis have expanded leading to prolonged survival albeit frequently with renal failure. Read More

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http://dx.doi.org/10.1097/TP.0000000000003170DOI Listing
February 2020

AA amyloidosis associated with Castleman disease: A case report and review of the literature.

Medicine (Baltimore) 2020 Feb;99(6):e18978

Penn Amyloidosis Program, Abramson Cancer Center.

Rationale: AA amyloidosis (AA) is caused by a wide variety of inflammatory states, but is infrequently associated with Castleman disease (CD). CD describes a heterogeneous group of hematologic disorders that share characteristic lymph node histopathology. CD can present with a solitary enlarged lymph node (unicentric CD, UCD) or with multicentric lymphadenopathy (MCD), constitutional symptoms, cytopenias, and multiple organ dysfunction due to an interleukin-6 driven cytokine storm. Read More

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http://dx.doi.org/10.1097/MD.0000000000018978DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7015640PMC
February 2020
5.723 Impact Factor

[Adult-onset Still's disease complications].

Rev Med Interne 2020 Mar 7;41(3):168-179. Epub 2020 Jan 7.

Service de médecine interne, hôpital de la Croix-Rousse, hospices civils de Lyon, université Claude-Bernard Lyon 1, Lyon, France. Electronic address:

Adult-onset Still's disease (AOSD), first described in 1971 by Bywaters, is a rare systemic auto-inflammatory disorder of unknown etiology, characterized by a symptomatic triad associating prolonged fever, polyarthritis and rash. The management of this disease has significantly improved since its first description, and, although the overall prognosis of the AOSD is good, with a low attributable mortality, below 3% (but up to 18% depending on the series), some rare complications are still possible, can be life-threatening and change the prognosis of the disease. A literature search was performed to review AOSD's complications: reactive hemophagocytic lymphohystiocytosis, coagulation disorders, fulminant hepatitis, cardiovascular (pericarditis, myocarditis, HTAP) or pulmonary complications, neurologic, renal complications, and AA amyloidosis. Read More

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http://dx.doi.org/10.1016/j.revmed.2019.12.003DOI Listing

Successful Pregnancies After Regression of AA Amyloidosis by Anti-inflammatory Therapy in Chronic Active Crohn's Disease.

Dig Dis Sci 2020 Sep 21;65(9):2730-2734. Epub 2019 Dec 21.

Division of Gastroenterology and Hepatology, Department of Internal Medicine, Medical University of Graz, Auenbruggerplatz 15, 8036, Graz, Austria.

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http://dx.doi.org/10.1007/s10620-019-05988-7DOI Listing
September 2020

Tofacitinib for the treatment for colchicine-resistant familial Mediterranean fever: case-based review.

Rheumatol Int 2020 Jan 7;40(1):169-173. Epub 2019 Dec 7.

Division of Rheumatology, Department of Internal Medicine, Gazi University Faculty of Medicine, 06100, Ankara, Turkey.

Familial Mediterranean fever is characterized by self-limited attacks of serositis and arthritis. However, substantial number of patients suffer from chronic complications of this disease, primarily involving musculoskeletal system. Treatment for these complications is challenging due to limited evidence. Read More

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http://dx.doi.org/10.1007/s00296-019-04490-7DOI Listing
January 2020

Causes of AA amyloidosis: a systematic review.

Amyloid 2020 Mar 25;27(1):1-12. Epub 2019 Nov 25.

Departments of Rheumatology & Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands.

From a clinical perspective, there is a need for a reliable and comprehensive list of diseases causing AA amyloidosis. This list could guide clinicians in the evaluation of patients with AA amyloidosis in whom an obvious cause is lacking. In this systematic review, a PubMed, Embase and Web of Science literature search were performed on causes of AA amyloidosis published in the last four decades. Read More

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http://dx.doi.org/10.1080/13506129.2019.1693359DOI Listing
March 2020
4 Reads

AA Amyloidosis After Renal Transplantation: An Important Cause of Mortality.

Transplantation 2019 Oct 30. Epub 2019 Oct 30.

Division of Nephrology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.

Background: There is limited data on the outcome of transplant recipients with familial Mediterranean fever (FMF)-associated AA amyloidosis. The aim of the present study is to evaluate demographic, clinical, laboratory, prognostic characteristics and outcome measures of these patients.

Methods: Eighty-one renal transplant recipients with FMF-associated AA amyloidosis (group 1) and propensity score-matched transplant recipients (group 2, n = 81) with nonamyloidosis etiologies were evaluated in this retrospective, multicenter study. Read More

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http://dx.doi.org/10.1097/TP.0000000000003043DOI Listing
October 2019
1 Read

Recognising and understanding cryopyrin-associated periodic syndrome in adults.

Br J Nurs 2019 Oct;28(18):1180-1186

Honorary Research Associate, National Amyloidosis Centre, Division of Medicine, University College London.

Cryopyrin-associated periodic syndrome (CAPS) is a group of rare hereditary autoinflammatory diseases characterised by recurrent flares of mild to severe systemic inflammation and fever. CAPS is the umbrella term for a spectrum of individual conditions, namely familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and neonatal-onset multisystem inflammatory disease (NOMID), also known as chronic infantile neurologic, cutaneous and articular (CINCA) syndrome. The flare symptoms include fever, fatigue, rashes, headaches, arthralgia and myalgia that can last for a few hours or for several days. Read More

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http://dx.doi.org/10.12968/bjon.2019.28.18.1180DOI Listing
October 2019
1 Read

A Rare Case of Renal AA Amyloidosis Secondary to Sjogren's Syndrome.

Eur J Case Rep Intern Med 2019 16;6(9):001226. Epub 2019 Sep 16.

Nephrology, Hospital Pedro Hispano, Porto, Portugal.

AA amyloidosis is a rare complication of chronic inflammatory disorders and has been associated with rheumatoid arthritis and ankylosing spondylitis. We present a case of AA amyloidosis secondary to Sjogren's syndrome (SS). A 79-year-old woman presented with rapidly progressive renal failure and complaints of asthenia, anorexia and generalized oedema. Read More

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http://dx.doi.org/10.12890/2019_001226DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6774652PMC
September 2019
6 Reads

AA amyloidosis secondary to adult onset Still's disease: About 19 cases.

Semin Arthritis Rheum 2020 02 10;50(1):156-165. Epub 2019 Aug 10.

Sorbonne Université, AP-HP, Hôpital Tenon, Service de médecine interne, Centre de référence des maladies auto-inflammatoires et des amyloses d'origine inflammatoire (CEREMAIA), F-75020, Paris, France. Electronic address:

Objective: Adult onset Still's disease (AOSD) is an inflammatory disorder characterized by high spiking fever, evanescent rash, polyarthritis, and many other systemic manifestations. Recurrent or persistent disease can lead to AA amyloidosis (AAA). Our objectives were to present 3 French cases and perform a systematic review of the literature, in order to determine the prevalence, characteristics, predisposing factors, and therapeutic response of AOSD-related AAA. Read More

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http://dx.doi.org/10.1016/j.semarthrit.2019.08.005DOI Listing
February 2020
2 Reads

[AA amyloidosis. A single institution cohort study].

Medicina (B Aires) 2019 ;79(4):276-279

Servicio de Clínica Médica, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.

There is limited epidemiological information on AA amyloidosis in Argentina, so the objective of this study was to describe the epidemiological characteristics of this disease in a tertiary hospital in our country. We designed a prospective clinical cohort of all consecutive patients with AA amyloidosis confirmed by immunohistochemistry in tissue from the Institutional Registry of Amyloidosis of the Hospital Italiano de Buenos Aires, in the period 04/01/2012- 12/31/2017. Of the 121 patients in the registry, 18 were included with AA for the analysis. Read More

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September 2019
2 Reads

Specific changes in faecal microbiota are associated with familial Mediterranean fever.

Ann Rheum Dis 2019 10 3;78(10):1398-1404. Epub 2019 Aug 3.

Service de Médecine Interne, Centre de référence des maladies auto-inflammatoires et des amyloses inflammatoires (CEREMAIA), Sorbonne Université, Assistance Publique des Hôpitaux de Paris, Hôpital Tenon, Paris, France

Objectives: Familial Mediterranean fever (FMF) can be complicated by AA amyloidosis (AAA), though it remains unclear why only some patients develop amyloidosis. We examined the gut microbiota composition and inflammatory markers in patients with FMF complicated or not by AAA.

Methods: We analysed the gut microbiota of 34 patients with FMF without AAA, 7 patients with FMF with AAA, 19 patients with AAA of another origin, and 26 controls using 16S ribosomal RNA gene sequencing with the Illumina MiSeq platform. Read More

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http://dx.doi.org/10.1136/annrheumdis-2019-215258DOI Listing
October 2019
8 Reads
10.377 Impact Factor

[Renal amyloidosis complicating adult onset Still's disease: about three cases].

Pan Afr Med J 2019 8;32:158. Epub 2019 Apr 8.

Service de Médecine Interne, CHU Hédi Chaker, Sfax, Tunisie.

Renal amyloidosis is a rare complication of adult onset Still's disease. We here report three cases of renal amyloidosis in a series of 33 cases of adult onset Still's disease. The three patients enrolled had an average age of 43 years (with a range from 33 to 58 years). Read More

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http://dx.doi.org/10.11604/pamj.2019.32.158.6285DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6607293PMC
July 2019
5 Reads

Familial Mediterranean Fever: Assessing the Overall Clinical Impact and Formulating Treatment Plans.

Mediterr J Hematol Infect Dis 2019 1;11(1):e2019027. Epub 2019 May 1.

Institute of Pediatrics, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.

Recurrent self-limited attacks of fever and short-lived inflammation in the serosal membranes, joints, and skin are the leading features of familial Mediterranean fever (FMF), the most common autoinflammatory disorder in the world, transmitted as autosomal recessive trait caused by gene mutations. Their consequence is an abnormal function of pyrin, a natural repressor of inflammation, apoptosis, and release of cytokines. FMF-related mutant pyrins are hypophosphorylated following RhoA GTPases' impaired activity and show a propensity to relapsing uncontrolled systemic inflammation with inappropriate response to inflammatory stimuli and leukocyte spread to serosal membranes, joints or skin. Read More

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http://dx.doi.org/10.4084/MJHID.2019.027DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6548206PMC
May 2019
20 Reads

Experimental model of oral transmissible AA amyloidosis in quails.

Avian Pathol 2019 Dec 28;48(6):521-527. Epub 2019 Aug 28.

Laboratory of Veterinary Toxicology, Cooperative Department of Veterinary Medicine, Tokyo University of Agriculture and Technology , Tokyo , Japan.

In poultry and zoo birds, mass outbreaks of amyloid A (AA) amyloidosis are often reported, and horizontal transmission is considered as one of the causes. However, oral transmission of avian AA amyloidosis in nature has been unclear. In order to clarify the horizontal transmission of avian AA amyloidosis, basic research using an appropriate oral transmission model is necessary. Read More

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http://dx.doi.org/10.1080/03079457.2019.1632414DOI Listing
December 2019
6 Reads

Systemic Amyloidosis with Renal Failure: A Challenging Diagnosis of SAPHO Syndrome.

Eur J Case Rep Intern Med 2019 27;6(4):001087. Epub 2019 Mar 27.

Internal Medicine Department, Centro Hospitalar São João, Porto, Portugal.

Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a rare, unrecognized and chronic inflammatory disorder characterized by distinct cutaneous and osteoarticular manifestations. Renal complications are uncommon. We describe the unusual case of a patient with severe refractory and chronic hidradenitis suppurativa (HS) that progressed to chronic renal failure due to secondary amyloidosis, presenting with mandibular pain and renal failure. Read More

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http://dx.doi.org/10.12890/2019_001087DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6499096PMC
March 2019
25 Reads

Synchronous Unicentric Castleman Disease and Inflammatory Hepatocellular Adenoma: a Case Report.

Ann Hepatol 2019 Jan - Feb;18(1):263-268

Institute of Liver Studies, King's College Hospital, London, UK.

Systemic symptoms such as fever and fatigue are non-specific manifestations spanning from inflammation to neoplasia. Here we report the case of a 34 year-old man who presented with systemic symptoms for four months. CT-scan and MRI revealed a 3. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S16652681193033
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http://dx.doi.org/10.5604/01.3001.0012.7936DOI Listing
April 2020
24 Reads

Systemic Amyloidosis in a Patient With Familial Mediterranean Fever and Hodgkin Lymphoma: A Case Report.

J Pediatr Hematol Oncol 2020 04;42(3):234-237

Departments of Pediatric Rheumatology.

Systemic amyloidosis is a clinical manifestation of the accumulation of amyloid fibrils in tissues because of persistent acute phase elevation and chronic inflammation. Its most common causes are inflammatory diseases and malignancies. Here, we present a 12-year-old girl diagnosed with systemic amyloidosis and Hodgkin lymphoma (HL) who was also previously diagnosed with familial Mediterranean fever (FMF). Read More

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http://Insights.ovid.com/crossref?an=00043426-900000000-9755
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http://dx.doi.org/10.1097/MPH.0000000000001504DOI Listing
April 2020
9 Reads

Muckle-Wells Syndrome Across Four Generations in One Czech Family: Natural Course of the Disease.

Front Immunol 2019 16;10:802. Epub 2019 Apr 16.

Paediatric Rheumatology and Autoinflammatory Diseases Unit, Department of Paediatrics and Adolescent Medicine, First Faculty of Medicine, General University Hospital in Prague, Charles University, Prague, Czechia.

Muckle-Wells syndrome (MWS) represents a moderate phenotype of cryopyrinopathies. Sensorineural hearing loss and AA amyloidosis belong to the most severe manifestations of uncontrolled disease. Simultaneous discovery of MWS in four generations of one large kindred has enabled us to document natural evolution of untreated disease and their response to targeted therapy. Read More

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http://dx.doi.org/10.3389/fimmu.2019.00802DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6477140PMC
April 2019
9 Reads

Novel Therapeutic Potentials of Taxifolin for Amyloid-β-associated Neurodegenerative Diseases and Other Diseases: Recent Advances and Future Perspectives.

Int J Mol Sci 2019 Apr 30;20(9). Epub 2019 Apr 30.

Department of Neurology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka 565-8565, Japan.

Amyloid-β (Aβ) has been closely implicated in the pathogenesis of cerebral amyloid angiopathy (CAA) and Alzheimer's disease (AD), the major causes of dementia. Thus, Aβ could be a target for the treatment of these diseases, for which, currently, there are no established effective treatments. Taxifolin is a bioactive catechol-type flavonoid present in various plants, such as herbs, and it exhibits pleiotropic effects including anti-oxidant and anti-glycation activities. Read More

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http://dx.doi.org/10.3390/ijms20092139DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6539020PMC
April 2019
8 Reads

Pleiotropic neuroprotective effects of taxifolin in cerebral amyloid angiopathy.

Proc Natl Acad Sci U S A 2019 05 29;116(20):10031-10038. Epub 2019 Apr 29.

Department of Endocrinology, Metabolism, and Hypertension Research, Clinical Research Institute, National Hospital Organization Kyoto Medical Center, Fushimi-ku, 612-8555 Kyoto, Japan.

Cerebral amyloid angiopathy (CAA) results from amyloid-β deposition in the cerebrovasculature. It is frequently accompanied by Alzheimer's disease and causes dementia. We recently demonstrated that in a mouse model of CAA, taxifolin improved cerebral blood flow, promoted amyloid-β removal from the brain, and prevented cognitive dysfunction when administered orally. Read More

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http://dx.doi.org/10.1073/pnas.1901659116DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6525485PMC
May 2019
4 Reads

Treating TNF Receptor Associated Periodic Fever Syndrome in End-Stage Renal Failure.

Case Rep Nephrol 2019 17;2019:6819476. Epub 2019 Mar 17.

Hospital Amato Lusitano, Castelo Branco, Portugal.

Tumor necrosis factor receptor associated periodic syndrome (TRAPS) is a rare monogenic autoinflammatory disease. Its most severe manifestation is secondary amyloidosis. A 44-year-old male presented with nephrotic syndrome. Read More

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http://dx.doi.org/10.1155/2019/6819476DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6441500PMC
March 2019
10 Reads

Metformin inhibits IL-6 signaling by decreasing IL-6R expression on multiple myeloma cells.

Leukemia 2019 11 15;33(11):2695-2709. Epub 2019 Apr 15.

Division of Hematology, Mayo Clinic, Rochester, MN, 55905, USA.

IL-6 signaling plays a crucial role in the pathogenesis of a number of diseases, including multiple myeloma, primary amyloidosis, cytokine release syndrome and other inflammatory conditions. It is central for the growth and survival of malignant plasma cells. IL-6R and IL-6ST receptors transduce IL-6 signaling. Read More

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http://dx.doi.org/10.1038/s41375-019-0470-4DOI Listing
November 2019
5 Reads

Severe cryopyrin-associated periodic syndrome first characterized by early childhood-onset sensorineural hearing loss - Case report and literature review.

Int J Pediatr Otorhinolaryngol 2019 May 28;120:68-72. Epub 2019 Jan 28.

Division of Pediatric Rheumatology, Department of Pediatrics, IWK Health Centre, 5850/5980 University Ave, PO Box 9700, Halifax, NS, B3K 6R8, Canada. Electronic address:

Infant-onset bilateral sensorineural hearing loss is a key presenting symptom of the autoinflammatory cryopyrin-associated periodic syndrome. Other symptoms include periodic fever, cold-induced urticaria-like rash, chronic aseptic meningitis, polyarticular arthralgias, and renal AA amyloidosis. Early recognition and treatment with interleukin-1 blockade are critical for preventing disabling or fatal complications. Read More

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http://dx.doi.org/10.1016/j.ijporl.2019.01.037DOI Listing
May 2019
10 Reads

Cell assay for the identification of amyloid inhibitors in systemic AA amyloidosis.

Amyloid 2019 Mar 9;26(1):24-33. Epub 2019 Feb 9.

a Institute of Protein Biochemistry, Ulm University , Ulm , Germany.

Systemic AA amyloidosis is still, up to this day, a life-threatening complication of chronic inflammatory diseases. Despite the success of anti-inflammatory treatment, the prognosis of some AA patients is still poor, which is why therapies directed at the amyloidogenic pathway in AA amyloidosis are being sought after. The cell culture model of amyloid formation from serum amyloid A1 (SAA1) protein remodels crucial features of AA amyloid deposit formation in vivo. Read More

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https://www.tandfonline.com/doi/full/10.1080/13506129.2019.1
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http://dx.doi.org/10.1080/13506129.2019.1568978DOI Listing
March 2019
29 Reads

Secondary amyloidosis associated with heroin use and recurrent infections - A case report.

Authors:
Deepthi Mani

Ann Med Surg (Lond) 2019 Jan 22;37:38-41. Epub 2018 Nov 22.

Multi Care Good Samaritan Hospital, Puyallup, WA, USA.

A 49-year-old lady with history of polysubstance use disorder, recurrent cutaneous abscesses, spinal diskitis and septic thrombophlebitis presented to the emergency room with complaints of intermittent fevers, worsening right hip pain and bilateral lower extremity edema. A month before the presentation, she had left another hospital against medical advice after being diagnosed with Methicillin-resistant bacteremia and right hip septic arthritis. Post discharge, she was off antibiotics, but continued heroin and methamphetamine use. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S20490801183026
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http://dx.doi.org/10.1016/j.amsu.2018.11.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6297111PMC
January 2019
12 Reads

Combined treatment with the phenolics (-)-epigallocatechin-3-gallate and ferulic acid improves cognition and reduces Alzheimer-like pathology in mice.

J Biol Chem 2019 02 18;294(8):2714-2731. Epub 2018 Dec 18.

the Zilkha Neurogenetic Institute, Department of Physiology and Neuroscience, Keck School of Medicine, University of Southern California, Los Angeles, California 90089-2821

"Nutraceuticals" are well-tolerated natural dietary compounds with drug-like properties that make them attractive as Alzheimer's disease (AD) therapeutics. Combination therapy for AD has garnered attention following a recent National Institute on Aging mandate, but this approach has not yet been fully validated. In this report, we combined the two most promising nutraceuticals with complementary anti-amyloidogenic properties: the plant-derived phenolics (-)-epigallocatechin-3-gallate (EGCG, an α-secretase activator) and ferulic acid (FA, a β-secretase modulator). Read More

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http://dx.doi.org/10.1074/jbc.RA118.004280DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6393618PMC
February 2019
14 Reads

A rare cause of AA amyloidosis and end-stage kidney failure: Answers.

Pediatr Nephrol 2019 09 19;34(9):1537-1539. Epub 2018 Nov 19.

Division of Pediatric Nephrology, Department of Pediatrics, Koç University School of Medicine, Davutpaşa cad no: 4 Topkapı, 34010, Istanbul, Turkey.

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http://dx.doi.org/10.1007/s00467-018-4153-6DOI Listing
September 2019
6 Reads

A rare cause of AA amyloidosis and end-stage kidney failure: Questions.

Pediatr Nephrol 2019 09 19;34(9):1533-1535. Epub 2018 Nov 19.

Division of Pediatric Nephrology, Department of Pediatrics, Koç University School of Medicine, Davutpaşa cad no: 4 Topkapı, 34010, Istanbul, Turkey.

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http://dx.doi.org/10.1007/s00467-018-4152-7DOI Listing
September 2019
7 Reads

Rituximab Therapy in Renal Amyloidosis Secondary to Rheumatoid Arthritis.

Biomolecules 2018 11 5;8(4). Epub 2018 Nov 5.

Department of Internal Medicine, Division of Rheumatology, Hacettepe University Faculty of Medicine, 06100 Ankara, Turkey.

Secondary amyloid A (AA) amyloidosis is a late and serious complication of poorly controlled, chronic inflammatory diseases. Rheumatoid arthritis (RA) patients with poorly controlled, longstanding disease and those with extra-articular manifestations are under risk for the development of AA amyloidosis. Although new drugs have proven to be significantly effective in the treatment of secondary AA amyloidosis, no treatment modality has proven to be ideal. Read More

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http://www.mdpi.com/2218-273X/8/4/136
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http://dx.doi.org/10.3390/biom8040136DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6316109PMC
November 2018
40 Reads

Azelnidipine Attenuates the Oxidative and NFκB Pathways in Amyloid-β-Stimulated Cerebral Endothelial Cells.

ACS Chem Neurosci 2019 01 8;10(1):209-215. Epub 2018 Nov 8.

Richard and Loan Hill Department of Bioengineering , University of Illinois at Chicago , 851 South Morgan Street, MC 063 , Chicago , Illinois 60607 , United States.

Cerebral amyloid angiopathy (CAA), a condition depicting cerebrovascular accumulation of amyloid β-peptide (Aβ), is a common pathological manifestation in Alzheimer's disease (AD). In this study, we investigated the effects of Azelnidipine (ALP), a dihydropyridine calcium channel blocker known for its treatment of hypertension, on oligomeric Aβ (oAβ)-induced calcium influx and its downstream pathway in immortalized mouse cerebral endothelial cells (bEND3). We found that ALP attenuated oAβ-induced calcium influx, superoxide anion production, and phosphorylation of extracellular signal-regulated kinase 1/2 (ERK1/2) and calcium-dependent cytosolic phospholipase A (cPLA). Read More

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http://pubs.acs.org/doi/10.1021/acschemneuro.8b00368
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http://dx.doi.org/10.1021/acschemneuro.8b00368DOI Listing
January 2019
6 Reads

Secondary, AA, Amyloidosis.

Rheum Dis Clin North Am 2018 11 7;44(4):585-603. Epub 2018 Sep 7.

National Amyloidosis Centre, Royal Free Campus, University College Medical School, Rowland Hill Street, London NW3 2PF, UK.

Secondary, AA, amyloidosis is a rare systemic complication that can develop in any long-term inflammatory disorder, and is characterized by the extracellular deposition of fibrils derived from serum amyloid A (SAA) protein. SAA is an acute-phase reactant synthetized largely by hepatocytes under the transcriptional regulation of proinflammatory cytokines. The kidney is the major involved organ with proteinuria as first clinical manifestation; renal biopsy is the commonest diagnostic investigation. Read More

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http://dx.doi.org/10.1016/j.rdc.2018.06.004DOI Listing
November 2018
14 Reads

[Renal amyloidosis revealing a cryopyrin associated periodic syndrome].

Ann Pathol 2018 Dec 17;38(6):401-406. Epub 2018 Sep 17.

Service d'anatomie et cytologie pathologiques, CHU de Pontchaillou, 2, rue Henri-le-Guilloux, 35033 Rennes cedex 9, France.

Introduction: Cryopyrin associated periodic syndrome is a rare auto inflammatory disease including three clinical entities with a common genetic cause. Among these three entities, Muckle-Wells syndrome is described as an intermediate phenotype associated with a progressive sensorineural hearing loss and AA amyloidosis. The present case reports a renal AA amyloidosis associated with an IgA nephropathy, revealing a Muckle-Wells syndrome. Read More

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http://dx.doi.org/10.1016/j.annpat.2018.07.001DOI Listing
December 2018
22 Reads