445 results match your criteria Amyloidosis AA Inflammatory


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

Ann Hepatol 2018 Dec;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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http://dx.doi.org/10.5604/01.3001.0012.7936DOI Listing
December 2018

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
2 Reads

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

Pediatr Nephrol 2018 Nov 19. 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
November 2018
1 Read

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

Pediatr Nephrol 2018 Nov 19. 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
November 2018
1 Read

Rituximab Therapy in Renal Amyloidosis Secondary to Rheumatoid Arthritis.

Biomolecules 2018 Nov 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
10 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
1 Read

[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
7 Reads

First Nationwide Survey of 199 Patients with Amyloid A Amyloidosis in Japan.

Intern Med 2018 Dec 10;57(23):3351-3355. Epub 2018 Aug 10.

Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Japan.

Objective To clarify the underlying diseases, clinical manifestations, and treatment strategies for Amyloid A (AA) amyloidosis (AAA) in Japanese patients. Methods We conducted a survey on Japanese patients with AAA treated between January 1, 2012, and December 31, 2014. Results A total of 199 patients with AAA were included in the present study. Read More

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http://dx.doi.org/10.2169/internalmedicine.1099-18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6306533PMC
December 2018
2 Reads

Familial Mediterranean fever in Chinese adult patients.

Rheumatology (Oxford) 2018 Dec;57(12):2140-2144

Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

Objectives: FMF is the most frequent monogenic auto-inflammatory disease worldwide. There have been hardly any cases reported in the Chinese population. We aimed to describe the first cohort of adult FMF patients in China. Read More

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http://dx.doi.org/10.1093/rheumatology/key218DOI Listing
December 2018
2 Reads
4.475 Impact Factor

Heroin Use Is Associated with AA-Type Kidney Amyloidosis in the Pacific Northwest.

Clin J Am Soc Nephrol 2018 Jul 15;13(7):1030-1036. Epub 2018 Jun 15.

Division of Nephrology, Department of Medicine,

Background And Objectives: AA-type kidney amyloidosis is classically associated with chronic autoimmune or inflammatory disorders. However, some urban centers have reported a high prevalence of injection drug use among patients with kidney AA amyloidosis. Previous reports lack control groups to quantify associations and most predate the opioid epidemic in the United States. Read More

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http://dx.doi.org/10.2215/CJN.13641217DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6032593PMC
July 2018
15 Reads
1 Citation
4.613 Impact Factor

In Vitro and In Silico Studies of the Molecular Interactions of Epigallocatechin-3--gallate (EGCG) with Proteins That Explain the Health Benefits of Green Tea.

Molecules 2018 May 28;23(6). Epub 2018 May 28.

Graduate Division of Nutritional and Environmental Sciences, University of Shizuoka, Shizuoka 422-8526, Japan.

Green tea has been shown to have beneficial effects on many diseases such as cancer, obesity, inflammatory diseases, and neurodegenerative disorders. The major green tea component, epigallocatechin-3--gallate (EGCG), has been demonstrated to contribute to these effects through its anti-oxidative and pro-oxidative properties. Furthermore, several lines of evidence have indicated that the binding affinity of EGCG to specific proteins may explain its mechanism of action. Read More

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http://dx.doi.org/10.3390/molecules23061295DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6099932PMC
May 2018
1 Read

Profile of renal AA amyloidosis in older and younger individuals: a single-centre experience.

Amyloid 2018 Jun 18;25(2):115-119. Epub 2018 May 18.

a Department of Nephrology , Ankara University School of Medicine , Ankara , Turkey.

Objective: In epidemiological studies of amyloid A (AA) amyloidosis from Turkey, the most frequently cause was familial Mediterranean fever (FMF) and it occurs generally in young age population. However, there are no sufficient data regarding aetiology, clinical presentation and prognosis of renal AA amyloidosis in advanced age patients. In this study, we aimed to investigate demographic, clinical presentation, aetiology and outcomes of adults aged 60 years or older patients with biopsy-proven renal AA amyloidosis. Read More

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http://dx.doi.org/10.1080/13506129.2018.1474733DOI Listing
June 2018
17 Reads

Tocilizumab histologically improved AA renal amyloidosis in a patient with multicentric Castleman disease: A case report
.

Clin Nephrol 2018 Sep;90(3):232-236

Multicentric Castleman disease (MCD) is a rare systemic lymphoproliferative disorder and is infrequently associated with renal complications that include amyloid A (AA) amyloidosis. Although it has been reported that patients with MCD and amyloidosis usually have a poor prognosis, recently, tocilizumab, a humanized anti-interleukin-6 receptor antibody, has emerged as an effective and specific treatment for AA amyloidosis secondary to chronic inflammatory disorders. Here we report a case of an MCD patient with secondary AA renal amyloidosis who was successfully treated with tocilizumab. Read More

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http://dx.doi.org/10.5414/CN109273DOI Listing
September 2018
4 Reads

Apolipoprotein A-II induces acute-phase response associated AA amyloidosis in mice through conformational changes of plasma lipoprotein structure.

Sci Rep 2018 Apr 4;8(1):5620. Epub 2018 Apr 4.

Department of Aging Biology, Institute of Pathogenesis and Disease Prevention, Shinshu University Graduate School of Medicine, Matsumoto, 290-8621, Japan.

During acute-phase response (APR), there is a dramatic increase in serum amyloid A (SAA) in plasma high density lipoproteins (HDL). Elevated SAA leads to reactive AA amyloidosis in animals and humans. Herein, we employed apolipoprotein A-II (ApoA-II) deficient (Apoa2 ) and transgenic (Apoa2Tg) mice to investigate the potential roles of ApoA-II in lipoprotein particle formation and progression of AA amyloidosis during APR. Read More

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http://dx.doi.org/10.1038/s41598-018-23755-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5884826PMC
April 2018
7 Reads

Efficacy and safety of interleukin-1 inhibitors in familial Mediterranean fever patients complicated with amyloidosis.

Mod Rheumatol 2018 Apr 27:1-4. Epub 2018 Apr 27.

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

Background: Colchicine is the mainstay of the treatment of familial Mediterranean fever (FMF). However, 10% of FMF patients do not respond well to colchicine. Efficacy of interleukin (IL)-1 inhibitors in reducing attacks have been demonstrated in colchicine-resistant FMF (crFMF) patients recently. Read More

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http://dx.doi.org/10.1080/14397595.2018.1457469DOI Listing
April 2018
12 Reads

Recurrent fever and arthralgia as the presentation of tumor necrosis factor receptor-associated periodic syndrome (TRAPS) in a Chinese girl: a case report and review of the literature.

Clin Rheumatol 2018 May 15;37(5):1433-1438. Epub 2018 Feb 15.

Department of Clinical Laboratory, Zhejiang Provincial People's Hospital, Hangzhou Medical College, 158 Shangtang Road, Hangzhou, 310014, China.

Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is characterized by recurrent episodes of inflammation with fever, abdominal pain, chest pain, rash, myalgia, arthralgia, conjunctivitis, and periorbital edema. This condition is a rare autosomal dominant disease that is strongly associated with heterozygous mutations in the tumor necrosis factor (TNF) receptor super family 1A (TNFRSF1A) gene. This condition is believed to be more common in Western countries than in Asian countries, and the AA amyloidosis rate for European countries is estimated to be 10%. Read More

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http://dx.doi.org/10.1007/s10067-018-4023-4DOI Listing
May 2018
5 Reads

Obesity is a significant susceptibility factor for idiopathic AA amyloidosis.

Amyloid 2018 Mar 24;25(1):37-45. Epub 2018 Jan 24.

a Department of Medicine V, Amyloidosis Center and Division of Hematology, Oncology and Rheumatology , University of Heidelberg , Heidelberg , Germany.

Background: To investigate obesity as susceptibility factor in patients with idiopathic AA amyloidosis.

Methods: Clinical, biochemical and genetic data were obtained from 146 patients with AA amyloidosis. Control groups comprised 40 patients with long-standing inflammatory diseases without AA amyloidosis and 56 controls without any inflammatory disease. Read More

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http://dx.doi.org/10.1080/13506129.2018.1429391DOI Listing
March 2018
10 Reads

[Renal involvement in amyloidosis and sarcoidosis].

Dtsch Med Wochenschr 2018 Jan 22;143(2):101-109. Epub 2018 Jan 22.

Amyloidosis is a rare disease characterized by extracellular deposition of fibrils. Among the most common forms of systemic amyloidosis with renal involvement are AL-amyloidosis based on plasma cell dyscrasia and AA-amyloidosis in chronic inflammatory diseases. Depending on the affected renal compartment, the clinical appearance of renal amyloidosis varies. Read More

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http://dx.doi.org/10.1055/s-0043-106565DOI Listing
January 2018
4 Reads

Variation of amino acid sequences of serum amyloid a (SAA) and immunohistochemical analysis of amyloid a (AA) in Japanese domestic cats.

J Vet Med Sci 2018 Feb 4;80(1):164-172. Epub 2017 Dec 4.

Department of Veterinary Pathology, Graduate School of Agricultural and Life Sciences. The University of Tokyo, 1-1-1 Yayoi, Bunkyo-ku, Tokyo 113-8657, Japan.

Amyloid A (AA) amyloidosis, a fatal systemic amyloid disease, occurs secondary to chronic inflammatory conditions in humans. Although persistently elevated serum amyloid A (SAA) levels are required for its pathogenesis, not all individuals with chronic inflammation necessarily develop AA amyloidosis. Furthermore, many diseases in cats are associated with the elevated production of SAA, whereas only a small number actually develop AA amyloidosis. Read More

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http://dx.doi.org/10.1292/jvms.17-0447DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5797877PMC
February 2018
7 Reads

Clinical outcomes and survival in AA amyloidosis patients.

Rev Bras Reumatol Engl Ed 2017 Nov - Dec;57(6):535-544. Epub 2017 Mar 23.

Uludağ University Faculty of Medicine, Department of Nephrology, Bursa, Turkey.

Aim: Amyloid A amyloidosis is a rare complication of chronic inflammatory conditions. Most patients with amyloid A amyloidosis present with nephropathy and it leads to renal failure and death. We studied clinical characteristics and survival in patients with amyloid A amyloidosis. Read More

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http://dx.doi.org/10.1016/j.rbre.2017.02.002DOI Listing
March 2017
17 Reads

Other autoinflammatory disease genes in an FMF-prevalent population: a homozygous MVK mutation and a novel heterozygous TNFRSF1A mutation in two different Turkish families with clinical FMF.

Clin Exp Rheumatol 2017 Nov-Dec;35 Suppl 108(6):75-81. Epub 2017 Oct 27.

Division of Rheumatology, Department of Internal Medicine, Cerrahpaşa Faculty of Medicine, Istanbul University, Turkey.

Objectives: No MEFV mutations are detected in approximately 10% of the patients with clinical FMF in populations where the disease is highly prevalent. Causative mutations were searched in other genes in two such families with "MEFV negative clinical FMF".

Methods: Father and daughter of family A had attacks of fever, abdominal pain and AA amyloidosis. Read More

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February 2018
13 Reads

[Cryopyrin-associated periodic syndromes].

Rev Med Interne 2018 Apr 27;39(4):287-296. Epub 2017 Oct 27.

Filière maladies rares FAI2R, 75000 Paris, France; Assistance publique-Hôpitaux de Paris, 75000 Paris, France; Service de médecine interne, centre de référence national maladies rares pour les maladies auto-inflammatoires et l'amylose (CEREMAIA), hôpital Tenon, université Pierre-et-Marie-Curie, 4, rue de la Chine, 75020 Paris, France.

Cryopyrin-associated periodic syndromes (CAPS) are linked to one single gene mutations, however they are associated with 3 syndromes, which are, from the mildest to the most severe phenotype familial cold urticaria, Muckle-Wells syndrome and chronic, infantile, neurologic, cutaneous, articular (CINCA) syndrome also called neonatal-onset multisystem inflammatory disease (NOMID). Autosomic dominant inheritance is present in most cases but in CINCA/NOMID syndrome where neomutations are more common. Mutations in the gene encoding cryopyrin, NLRP3, are associated with deregulation of caspase-1 activity, excessive interleukin-1 production and an autoinflammatory syndrome, which in familial cold urticaria and Muckle-Wells syndrome may be triggered or worsened by exposure to coldness. Read More

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http://dx.doi.org/10.1016/j.revmed.2017.09.002DOI Listing
April 2018
2 Reads

Cardiac disease in familial Mediterranean fever.

Rheumatol Int 2018 Jan 20;38(1):51-58. Epub 2017 Oct 20.

Department of Nephrology, Sütçü İmam Üniversitesi Tıp Fakültesi Hastanesi, 46040, Onikişubat/Kahramanmaraş, Turkey.

Familial Mediterranean fever (FMF) is an autoinflammatory disease manifested by inflammatory attacks of peritonitis, pleuritis, pericarditis accompanied by fever and arthritis. Mutations of MEFV gene results in pyrin dysfunction, which causes uncontrolled interleukin-1 beta production and triggers the inflammatory attacks. Inflammation persists even during attack-free periods in one-third of the FMF patients. Read More

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http://dx.doi.org/10.1007/s00296-017-3853-8DOI Listing
January 2018
15 Reads

Systemic Amyloidosis in an African Tiger Snake (Telescopus semiannulatus).

J Comp Pathol 2017 Aug - Oct;157(2-3):136-140. Epub 2017 Jul 29.

Disease Investigations, Institute for Conservation Research, San Diego Zoo Global, San Diego, CA, USA.

An adult male African tiger snake (Telescopus semiannulatus) was diagnosed with disseminated mycobacteriosis and a hepatic biliary cystadenocarcinoma. Histologically, the spleen was largely replaced by extracellular deposits of eosinophilic, fibrillar to hyaline material. Similar material was also present in the testicular interstitium and occasional blood vessel walls. Read More

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http://dx.doi.org/10.1016/j.jcpa.2017.07.001DOI Listing
May 2018
12 Reads

AA Amylodisis Associated with Jugular Paraganglioma as a Rare Cause of Chronic Diarrhea.

ACG Case Rep J 2017 16;4:e100. Epub 2017 Aug 16.

Department of Gastroenterology and Hepatology, Hospital Universitario Marqués de Valdecilla-IDIVAL, Universidad de Cantabria, Santander, Spain.

We report a case of a 64-year-old man with chronic diarrhea who was diagnosed of a systemic reactive (AA) amyloidosis associated with a jugular paraganglioma. This neoplasm was diagnosed 30 years previously and it was not removed after extensive evaluation by a multidisciplinary team. Chronic inflammatory diseases are the major cause of AA amyloidosis. Read More

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http://dx.doi.org/10.14309/crj.2017.100DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5559658PMC
August 2017
12 Reads

Spontaneous, Experimentally Induced, and Transmissible AA Amyloidosis in Japanese Quail ( Coturnix japonica).

Vet Pathol 2017 11 16;54(6):912-921. Epub 2017 Aug 16.

1 Laboratory of Veterinary Toxicology, Tokyo University of Agriculture and Technology, Fuchu-shi, Tokyo, Japan.

The authors describe a spontaneous case of amyloid A (AA) amyloidosis in an adult female Japanese quail ( Coturnix japonica). The bird developed AA amyloidosis secondary to chronic peritonitis caused by a Gram-negative bacillus infection. Mild amyloid deposition was also identified in the intestinal tract of apparently healthy adult individuals, suggesting that quail may develop intestinal amyloidosis with age. Read More

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http://dx.doi.org/10.1177/0300985817723692DOI Listing
November 2017
26 Reads

Safety and efficacy of empirical interleukin-1 inhibition using anakinra in AA amyloidosis of uncertain aetiology.

Amyloid 2017 Sep 26;24(3):189-193. Epub 2017 Jul 26.

a National Amyloidosis Centre, Centre for Amyloidosis and Acute Phase Proteins, Division of Medicine, University College London , London , UK.

Objective: AA amyloidosis is a serious complication of persistent inflammation, which, untreated will progress to renal failure and death. Effective suppression of the underlying inflammatory disease is the focus of treatment. However, in approximately 20% of cases the underlying condition remains uncertain, presenting a dilemma as to choice of treatment. Read More

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http://dx.doi.org/10.1080/13506129.2017.1352503DOI Listing
September 2017
13 Reads

[Autoinflammatory diseases and kidney involvement].

Ter Arkh 2017;89(6):4-20

I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia, Moscow, Russia.

Autoinflammatory disease (AID) is a new concept formulated from the results of studying the pathogenesis of familial periodic fevers, a heterogeneous group of genetically determined diseases characterized by causelessly recurrent exacerbations of the inflammatory process due to genetically determined disorders of innate immunity and accompanied by uncontrolled hypersecretion of interleukin-1 (IL-1). These mechanisms were a basic model for understanding a wide range of rheumatologic and other inflammatory diseases of the internal organs. The late diagnosis of AIDs and their ineffective treatment increase the risk for the development and progression of secondary AA amyloidosis. Read More

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http://dx.doi.org/10.17116/terarkh20178964-20DOI Listing
November 2017
5 Reads

Changing epidemiology of AA amyloidosis: clinical observations over 25 years at a single national referral centre.

Amyloid 2017 Sep 7;24(3):162-166. Epub 2017 Jul 7.

a National Amyloidosis Centre, Division of Medicine, Centre for Amyloidosis and Acute Phase Proteins , University College London , London , UK.

Objective: Systemic AA amyloidosis is a serious complication of chronic inflammation; however, there are relatively few published data on its incidence. We investigated the changing epidemiology of AA amyloidosis over a 25-year period at a single national referral centre.

Methods: We conducted a retrospective study of all patients diagnosed with AA amyloidosis who had attended the centre between 1990 and 2014 inclusive. Read More

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https://www.tandfonline.com/doi/full/10.1080/13506129.2017.1
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http://dx.doi.org/10.1080/13506129.2017.1342235DOI Listing
September 2017
16 Reads

Suppression of Lipopolysaccharide-Induced Inflammatory Response by Fragments from Serum Amyloid A.

J Immunol 2017 08 3;199(3):1105-1112. Epub 2017 Jul 3.

Engineering Research Center of Cell and Therapeutic Antibody, Ministry of Education, School of Pharmacy, Shanghai Jiao Tong University, Shanghai 200240, China; and

Serum amyloid A (SAA) is known as an acute-phase protein and a biomarker for inflammatory diseases. Published studies have shown that SAA possesses proinflammatory cytokine-like activity and is chemotactic for phagocytes, but the structural basis for these activities remains unidentified. In this article, we report that truncated SAA1 proteins lacking N- and C-terminal sequences exhibit reduced proinflammatory activity and strongly suppress LPS-induced expression of IL-1β, IL-6, and TNF-α in macrophages. Read More

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http://dx.doi.org/10.4049/jimmunol.1700470DOI Listing
August 2017
16 Reads

Gastrointestinal Amyloidosis: Review of the Literature.

Cureus 2017 May 8;9(5):e1228. Epub 2017 May 8.

Internal Medicine, University of Kansas School of Medicine - Wichita.

Gastrointestinal amyloidosis (GIA), a protein deposition disorder, represents a complex common pathway that encompasses multiple etiologies and presentations. It represents a significant diagnostic and treatment challenge. The disease results from the deposition of insoluble extracellular protein fragments that have been rendered resistant to digestion. Read More

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http://dx.doi.org/10.7759/cureus.1228DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5464793PMC
May 2017
15 Reads

Tocilizumab in the treatment of twelve cases with aa amyloidosis secondary to familial mediterranean fever.

Orphanet J Rare Dis 2017 05 30;12(1):105. Epub 2017 May 30.

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

Background: There is no established treatment of AA amyloidosis, a long-term complication of various chronic inflammatory diseases associated with increased mortality, such as familial Mediterranian fever (FMF). Recently there are few reports pointing out that tocilizumab(TCZ), an anti IL-6 agent may be effective in AA amyloidosis resistant to conventional treatments. We report our data on the effect of TCZ in patients with FMF complicated with AA amyloidosis. Read More

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http://dx.doi.org/10.1186/s13023-017-0642-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5450086PMC
May 2017
34 Reads

[AA amyloidosis].

Nephrol Ther 2017 Jun 24;13(4):258-264. Epub 2017 Apr 24.

Service de médecine interne, centre de référence des amyloses d'origine inflammatoire et de la fièvre méditerranéenne familiale, hôpital Tenon, 4, rue de la Chine, 75020 Paris, France; Département hospitalo-universitaire inflammation immunopathologie biothérapie (DHU I2B), faculté de médecine, université Pierre-et-Marie-Curie, 4, rue de la Chine, 75020 Paris, France. Electronic address:

AA amyloidosis remains one of the three main types of systemic amyloidosis with AL and ATTR. Its incidence has been however decreasing recently in Western countries. Chronic inflammatory diseases are currently the first cause of AA amyloidosis, including rheumatoid arthritis, spondyloarthritis and autoinflammatory diseases. Read More

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http://dx.doi.org/10.1016/j.nephro.2017.03.001DOI Listing
June 2017
25 Reads

[A rare concurrence of polymyalgia rheumatica and AA-amyloidosis].

Arkh Patol 2017;79(2):53-57

Moscow City Clinical Hospital Fifty-Two, Moscow Healthcare Department, Moscow, Russia; G.N. Gabrichevsky Moscow Research Institute of Epidemiology and Microbiology, Russian Federal Service for Supervision of Consumer Rights Protection and Human Welfare, Moscow, Russia; Branch, S.M. Kirov Military Medical Academy, Ministry of Defense of the Russian Federation, Moscow, Russia.

Polymyalgia rheumatica (PMR) is a rare chronic inflammatory disease. It predominantly affects the elderly. The disease has a slow onset, pain and stiffness in the muscles of the shoulder and pelvic girdle, fever, weight loss, and a high acute-phase inflammatory response. Read More

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http://dx.doi.org/10.17116/patol201779253-57DOI Listing
April 2018
5 Reads

CSP-1103 (CHF5074) stabilizes human transthyretin in healthy human subjects.

Amyloid 2017 Mar 10;24(1):42-51. Epub 2017 Apr 10.

c Department of Histology and Embryology , Harbin Medical University , Harbin , Heilongjiang , China.

Hereditary amyloid polyneuropathy is a type of protein misfolding disease. Transthyretin (TTR) is a homotetrameric serum protein and TTR tetramer dissociation is the limiting step in amyloid fibril formation. Thus, prevention of TTR dissociation is a promising therapeutic approach and some TTR stabilizers have been approved for the treatment of TTR amyloidosis. Read More

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http://dx.doi.org/10.1080/13506129.2017.1308348DOI Listing
March 2017
9 Reads

Novel evidences of atypical manifestations in cryopyrin-associated periodic syndromes.

Clin Exp Rheumatol 2017 Nov-Dec;35 Suppl 108(6):27-31. Epub 2017 Jan 9.

Department of Immunology-CDB, Hospital Clínic-IDIBAPS, Barcelona, Spain.

Objectives: Cryopyrin-associated periodic syndromes (CAPS) usually start during infancy as an urticarial-like rash and a marked acute phase response, with additional manifestations appearing during its evolution. The aim of this study was to expand the clinical diversity of CAPS by the description of novel atypical features.

Methods: Clinical data were collected from patients' medical charts. Read More

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February 2018
14 Reads

Colchicine in Renal Medicine: New Virtues of an Ancient Friend.

Blood Purif 2017 13;43(1-3):125-135. Epub 2016 Dec 13.

Division of Nephrology, Department of Internal Medicine, Sakarya University Training and Research Hospital, Sakarya, Turkey.

Colchicine is a plant-derived alkaloid that disrupts the cell microtubule system and accumulates in neutrophils, inhibiting neutrophil adhesion and recruitment. Colchicine has been used extensively in the prevention and treatment of gouty arthritis attacks, familial Mediterranean fever attacks and resultant AA amyloidosis, and recurrent pericarditis. Colchicine also disrupts the intracellular traffic of additional inflammatory and fibrosis mediators. Read More

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http://dx.doi.org/10.1159/000454669DOI Listing
March 2017
20 Reads

A Case Report Describing a Rare Presentation of Simultaneous Occurrence of MPO-ANCA-Associated Vasculitis and Rheumatoid Arthritis.

Case Rep Nephrol 2016 6;2016:9340524. Epub 2016 Nov 6.

Department of Pathology, Memorial Medical Center, 701 North 1st Street, Springfield, IL 62781, USA.

. Renal-limited myeloperoxidase vasculitis with simultaneous rheumatoid arthritis is reported as a rare occurrence. Review of literature suggests that most patients had a diagnosis of rheumatoid arthritis for several years prior to presenting with renal failure from myeloperoxidase vasculitis. Read More

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https://www.hindawi.com/journals/crin/2016/9340524/
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http://dx.doi.org/10.1155/2016/9340524DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5116358PMC
November 2016
9 Reads

Studies on the potential risk of amyloidosis from exposure to silk fibroin.

Biomed Mater 2016 11 21;11(6):065010. Epub 2016 Nov 21.

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

Amyloid A (AA) amyloidosis can be induced by the administration of amyloid fibrils to animals under inflammatory conditions. Silk fibroin (SF) is a main component protein of bombic silk and has amyloid-like features. The amyloidogenesis of SF solution in mice has been previously reported. Read More

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http://dx.doi.org/10.1088/1748-6041/11/6/065010DOI Listing
November 2016
8 Reads

Two diseases one remedy? Systemic amyloidosis secondary to hidradenitis suppurativa: Treatment with infliximab.

Dermatol Ther 2017 Mar 12;30(2). Epub 2016 Nov 12.

Department of Dermatology, Gazi University Faculty of Medicine, Ankara, Turkey.

Hidradenitis suppurativa, known as acne inversa, is a relapsing and chronic inflammatory skin disease affecting the skin folds. During the chronic course of the disease many local complications like fistulae to other tissues or systemic complications including anemia, secondary amyloidosis, lymphedema, nephrotic syndrome, artropathy may take place. Amyloid A amyloidosis is a rare complication of hidradenitis suppurativa, which has been described in a limited number of case reports. Read More

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http://dx.doi.org/10.1111/dth.12445DOI Listing
March 2017
20 Reads

Experimental transmission of systemic AA amyloidosis in autoimmune disease and type 2 diabetes mellitus model mice.

Exp Anim 2016 Nov 16;65(4):427-436. Epub 2016 Jun 16.

Laboratory of Food and Environmental Hygiene, Department of Veterinary Medicine, Faculty of Applied Biological Sciences, Gifu University, Gifu 501-1193, Japan.

AA amyloidosis is a protein misfolding disease characterized by extracellular deposition of amyloid A (AA) fibrils. AA amyloidosis has been identified in food animals, and it has been postulated that AA amyloidosis may be transmissible to different animal species. Since the precursor protein of AA fibrils is serum amyloid A (SAA), which is an inflammatory acute phase protein, AA amyloidosis is considered to be associated with inflammatory diseases such as rheumatoid arthritis. Read More

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http://dx.doi.org/10.1538/expanim.16-0037DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5111846PMC
November 2016
10 Reads

MEFV and SAA1 genotype associations with clinical features of familial Mediterranean fever and amyloidosis in Armenia.

Clin Exp Rheumatol 2016 Sep-Oct;34(6 Suppl 102):72-76. Epub 2016 Oct 25.

Rheumatology Unit, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.

Objectives: Familial Mediterranean fever (FMF) is a hereditary periodic disease characterised by recurrent attacks of fever and serositis. The most devastating complication of FMF is amyloidosis (AA) affecting mainly the kidneys. Aim of the study is to search for correlations between the MEFV genotype and the SAA polymorphisms with the clinical manifestations of FMF and the occurrence of amyloidosis in a large cohort of Armenian patients. Read More

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February 2017
13 Reads

The Phenotype and Genotype of Mevalonate Kinase Deficiency: A Series of 114 Cases From the Eurofever Registry.

Arthritis Rheumatol 2016 11;68(11):2795-2805

Istituto Giannina Gaslini, Genoa, Italy.

Objective: Mevalonate kinase deficiency (MKD) is a rare metabolic disease characterized by recurrent inflammatory episodes. This study was undertaken to describe the genotype, phenotype, and response to treatment in an international cohort of MKD patients.

Methods: All MKD cases were extracted from the Eurofever registry (Executive Agency for Health and Consumers project no. Read More

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http://dx.doi.org/10.1002/art.39763DOI Listing
November 2016
38 Reads

Secondary bladder amyloidosis with familial Mediterranean fever in a living donor kidney transplant recipient: a case report.

BMC Res Notes 2016 Oct 19;9(1):473. Epub 2016 Oct 19.

Department of Urology, Akita University School of Medicine, 1-1-1 Hondo, Akita, 010-8543, Japan.

Background: Secondary bladder amyloidosis is an extremely rare disease, resulting from a chronic systematic inflammatory disorder associated with amyloid deposits. Although uncommon in Japan, familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease characterized by recurrent episodes of fever of short duration and serositis and is frequently associated with systemic amyloidosis. Here, we present a case of a Japanese patient complaining of fever and macroscopic hematuria after a living donor renal transplantation. Read More

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http://dx.doi.org/10.1186/s13104-016-2273-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5070197PMC
October 2016
26 Reads

Anti-TNFα therapy for chronic inflammatory disease in kidney transplant recipients: Clinical outcomes.

Medicine (Baltimore) 2016 Oct;95(41):e5108

aCHU Clermont-Ferrand, Department of Nephrology, Clermont-Ferrand bNecker Hospital, Assistance Publique-Hôpitaux de Paris, Department of Nephrology and Kidney Transplantation, Paris cCHU Rangueil, Nephrology, Dialysis, Transplantation, Toulouse dCHU Pontchaillou, Department of Nephrology, Rennes eCHU Clermont-Ferrand, Biostatistics Unit (DRCI), Clermont-Ferrand fUniversity Hospital, Nephrology-Transplantation Department, Strasbourg gCHRU and FHU Transplantation, Department of Nephrology and Clinical Immunology, Tours hCHU Clermont-Ferrand, Department of Rheutamology, Clermont-Ferrand iService de Néphrologie-Dialyse-Transplantation, CHU Angers, Angers jHôpital Maison Blanche-CHU Reims, Department of Nephrology, Reims kGastroenterology Department, University Hospital Estaing, Clermont-Ferrand lNephrology Department, Hôpital Européen Georges Pompidou, Assistance Publique Hôpitaux de Paris, Paris mDepartment of Nephrology, CHU de Caen, Caen, France.

Anti-tumor necrosis factor-α (TNFα) therapy has improved the prognosis of many chronic inflammatory diseases. It appears to be well-tolerated by liver-transplant patients. However, their use and their safety in kidney-transplant patients have yet to be determined. Read More

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http://dx.doi.org/10.1097/MD.0000000000005108DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5072954PMC
October 2016
29 Reads

The Schnitzler syndrome: chronic urticaria in disguise: a single-centre report of 11 cases and a critical reappraisal of the literature.

Clin Exp Rheumatol 2017 Jan-Feb;35(1):69-73. Epub 2016 Aug 31.

Department of General Internal Medicine, University Hospitals Leuven, Belgium.

Objectives: The Schnitzler syndrome is a rare inflammatory disorder, with a chronic urticaria-like rash and an IgM (rarely IgG) monoclonal gammopathy as cardinal features. Interleukin-1 β is regarded as the key mediator and the interleukin-1 receptor antagonist anakinra has been proposed as first-line treatment. This case series of eleven patients is intended to enhance disease awareness and to compare our centre's experience with that of literature. Read More

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June 2017
13 Reads

Deposition, Clearance, and Reinduction of Amyloid A Amyloid in Interleukin 1 Receptor Antagonist Knockout Mice.

Vet Pathol 2017 01 29;54(1):99-110. Epub 2016 Sep 29.

1 Department of Veterinary Pathology, Graduate School of Agricultural and Life Sciences, University of Tokyo, Tokyo, Japan.

Amyloid A (AA) amyloidosis is characterized by the extracellular deposition of AA amyloid and results in the irreversible dysfunction of parenchymal organs. In experimental models, AA amyloid deposits are cleared following a decrease in circulating serum amyloid A (SAA) concentrations. Additional inflammatory stimuli during this recovery process may induce more severe amyloid redeposition. Read More

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http://dx.doi.org/10.1177/0300985816658772DOI Listing
January 2017
4 Reads

Systemic amyloidosis: novel therapies and role of biomarkers.

Nephrol Dial Transplant 2017 May;32(5):770-780

Amyloidosis Research and Treatment Center, Foundation Scientific Institute Policlinico San Matteo, Department of Molecular Medicine, University of Pavia, Pavia, Italy.

Systemic amyloidosis is caused by misfolding and extracellular deposition of one of an ever-growing list of circulating proteins, resulting in vital organ dysfunction and eventually death. Despite different predisposing conditions, including plasma cell dyscrasias [immunoglobulin light chain (AL) amyloidosis], long-lasting inflammation [reactive (AA) amyloidosis] or mutations (hereditary amyloidoses), clinical manifestations are conspicuously overlapping and mimic more prevalent conditions, significantly complicating and often delaying the recognition of these rare, complex diseases. However, refined diagnostic and imaging approaches and the increasing role of biomarkers, which help in establishing the diagnosis, assessing the prognosis and evaluating the response to therapy, have considerably improved the management of these conditions. Read More

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http://ndt.oxfordjournals.org/content/early/2016/08/18/ndt.g
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http://dx.doi.org/10.1093/ndt/gfw305DOI Listing
May 2017
10 Reads

Hemodynamic deterioration after aortic valve replacement in a patient with mixed systemic amyloidosis.

Gen Thorac Cardiovasc Surg 2017 Aug 4;65(8):470-473. Epub 2016 Aug 4.

Department of Cardiovascular Surgery, Japan Community Health Care Organization (JCHO) Hokkaido Hospital, 1 jo 8 chome 3-18, Toyohira-ku, Sapporo, Japan.

We report a case of hemodynamic deterioration after aortic valve replacement in a patient with mixed systemic amyloidosis. A 77-year-old male with severe aortic valve stenosis and 19 years hemodialysis underwent aortic valve replacement. Postoperatively, the patient died of hemodynamic deterioration. Read More

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http://dx.doi.org/10.1007/s11748-016-0703-7DOI Listing
August 2017
6 Reads