519 results match your criteria Amyloidosis AA Inflammatory

Cardiovascular Sequelae and Genetics of Familial Mediterranean Fever: A Literature Review.

Pulse (Basel) 2021 Jun 3;8(3-4):78-85. Epub 2021 Jun 3.

Rawalpindi Institute of Cardiology, Rawalpindi, Pakistan.

Introduction: Familial Mediterranean fever (FMF) is an autoinflammatory fever syndrome distinguished by recurrent attacks of spontaneous peritonitis, pleuritis, fever, and arthritis. It is specifically seen in the ethnic groups of Mediterranean origin, but sporadic cases have been reported in Eastern Europe and America due to migrations. There is a number of cardiac manifestations associated with FMF. Read More

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Serum Amyloid A1 Induces Classically Activated Macrophages: A Role for Enhanced Fibril Formation.

Front Immunol 2021 30;12:691155. Epub 2021 Jun 30.

Institute of Pharmacology of Natural Products and Clinical Pharmacology, Ulm University, Ulm, Germany.

AA amyloidosis belongs to the group of amyloid diseases which can follow chronic inflammatory conditions of various origin. The disease is characterized by the deposition of insoluble amyloid fibrils formed by serum amyloid A1 (SAA1) leading eventually to organ failure. Macrophages are intimately involved in the fibrillogenesis as well as in the clearance of amyloid fibrils. Read More

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Renal involvement of a patient with Crohn´s disease: A case report.

Cesk Patol 2021 ;57(2):109-112

Inflammatory bowel disease (IBD) is a group of chronic relapsing intestinal inflammatory processes primarily represented by ulcerative colitis (UC) and Crohn´s disease (CD). Nearly half of IBD cases are followed by extraintestinal complications and renal involvement can occur independatly or along with other complications and are described with the patients sufferring from UC or CD. Most frequent renal involvement is nephrolithiasis, tubulointerstinal nephritis, different kinds of glomerulonephritis and AA amyloidosis. Read More

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AA Amyloidosis in the Course of HIV Infection: A Report of 19 Cases Including 4 New French Cases and a Comprehensive Review of Literature.

Nephron 2021 Jul 15:1-9. Epub 2021 Jul 15.

Department of Internal Medicine, Sorbonne University, AP-HP, Tenon Hospital, Centre De Référence Des Maladies Auto-Inflammatoires Et Des Amyloses D'origine Inflammatoire (CEREMAIA), Paris, France.

Introduction: HIV infection has been recently retained as an unclear cause of AA amyloidosis. Our aim was to investigate cases of AA amyloidosis associated with HIV infection to understand if it could be considered as a cause of AA amyloidosis.

Methods: A comprehensive literature review was conducted as well as retrospective study from French cases collected from our national reference center for AA amyloidosis. Read More

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Blockade of interleukin-6 as a possible therapeutic target for AA amyloidosis.

Nefrologia 2021 Jun 14. Epub 2021 Jun 14.

Servicio de Nefrología, Hospital Universitario Virgen Macarena, Sevilla, España.

Introduction: AA (secondary) amyloidosis is a severe complication of chronic inflammatory disorders. It is characterized by the systemic deposition of an abnormal protein called amyloid, affecting mainly renal function. IL-6 is a cytokine with a relevant role in this disease development. Read More

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Membranous nephropathy associated with multicentric Castleman's disease that was successfully treated with tocilizumab: a case report and review of the literature.

BMC Nephrol 2021 Jun 9;22(1):216. Epub 2021 Jun 9.

Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu, Mie, 514-8507, Japan.

Background: Multicentric Castleman's disease is a life-threatening disorder involving a systemic inflammatory response and multiple organ failure caused by the overproduction of interleukin-6. Although renal complications of Castleman's disease include AA amyloidosis, thrombotic microangiopathy, and membranoproliferative glomerulonephritis, membranous nephropathy is relatively rare. We experienced a case of secondary membranous nephropathy associated with Castleman's disease. Read More

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Two cases of secondary AA amyloidosis involving the skin and chronic kidney infection with a nephrotic syndrome in a high-income country.

BMJ Case Rep 2021 Jun 7;14(6). Epub 2021 Jun 7.

Nephrology Dialysis Apheresis, Hopital Universitaire de Nimes, CHU Caremeau, Nimes, France

We present two French cases of amyloid-associated (AA) amyloidosis secondary to chronic infections. Patient 1, a 51-year-old heroin addict, was hospitalised for chest pain and anasarca. During hospitalisation, a nephrotic syndrome with an inflammatory condition was discovered along with a chronic skin ulcer on his arm. Read More

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Amyloid Goiter in Familial Mediterranean Fever: Description of 42 Cases from a French Cohort and from Literature Review.

J Clin Med 2021 May 5;10(9). Epub 2021 May 5.

Internal Medicine Department and National Reference Center for Autoinflammatory Diseases and Inflammatory Amyloidosis (CEREMAIA), APHP, Tenon Hospital, Sorbonne University, 4 rue de la Chine, 75020 Paris, France.

Our aim was to describe the main features of amyloid goiter in adults with amyloidosis secondary to familial Mediterranean fever. Therefore, we analyzed cases from a French cohort of familial Mediterranean fever patients with amyloidosis and from literature review. Forty-two cases were identified: 9 from the French cohort and 33 from literature review. Read More

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Lung cancer combined with diffuse peritoneal and mesenteric amyloidosis detected on 18F-FDG PET/CT: A case report.

Medicine (Baltimore) 2021 May;100(21):e25961

Department of Neurosurgery, Aviation General Hospital of China Medical University, Chaoyang District, Beijing, People's Republic of China.

Rationale: Amyloidosis is a heterogeneous group of diseases characterized by extracellular deposition of amyloid fibrils. Lung carcinoma is rarely reported to be associated with AA amyloidosis. With regard to the manifestation of amyloidosis infiltrating organs, most of the cases focus on the heart, liver, kidneys, and peripheral nervous system. Read More

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Successful Treatment of AA Amyloidosis in Ankylosing Spondylitis Using Tocilizumab: Report of Two Cases and Review of the Literature.

Front Med (Lausanne) 2021 26;8:661101. Epub 2021 Apr 26.

Department of Biomedical and Clinical Sciences, Division of Inflammation and Infection, Linköping University, Linköping, Sweden.

Historically, secondary amyloidosis has been a feared complication of chronic inflammatory conditions. The fibril protein AA derives from the acute phase reactant serum amyloid A (SAA). Long-term elevation of SAA levels remains a major risk factor for the development of AA amyloidosis in rheumatic diseases, and the prognosis may be unpredictable. Read More

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[Familial Mediterranean fever in 2020].

Nephrol Ther 2021 Apr;17S:S119-S125

Service de médecine interne, hôpital Tenon, AP-HP, 4, rue de la Chine, 75020 Paris, France; Centre de référence des maladies auto-inflammatoires et des amyloses d'origine inflammatoire (Cerémaia), 4, rue de la Chine, 75020 Paris, France; Sorbonne université, 4, rue de la Chine, 75020 Paris, France.

Familial Mediterranean fever is the most frequent autoinflammatory disease with autosomal recessive transmission. Most patients carry mutations in the MEFV gene encoding the protein marenostrin/pyrin. It is characterised by short ant recurrent attacks of fever and serositis with abdominal or thoracic pain, usually lasting less than 3 days, raised inflammatory biologic markers in an individual of Mediterranean origin. Read More

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Species-barrier on the cross-species oral transmission of bovine AA amyloidosis in mice.

J Vet Med Sci 2021 Jul 28;83(6):962-967. Epub 2021 Apr 28.

Laboratory of Veterinary Toxicology, Cooperative Department of Veterinary Medicine, Tokyo University of Agriculture and Technology, 3-5-8 Saiwai-cho, Fuchu, Tokyo 183-8509, Japan.

In AA amyloidosis, cross-species oral transmission has been demonstrated in several animal models. While it is known that the transmission efficiency of AA amyloidosis between different species is lower than that among the same species, the mechanism of this species-barrier is unclear. In this study, we found at first that mice orally given a large amount of bovine AA simultaneously with inflammatory stimulation did not develop AA amyloidosis. Read More

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Tumor necrosis factor receptor-1 assciated periodic syndrome (TRAPS) related AA amyloidosis: a national case series and systematic review.

Rheumatology (Oxford) 2021 Mar 14. Epub 2021 Mar 14.

Sorbonne University, GRC GRAASU, Department of Internal Medicine, APHP, Tenon Hospital, Paris, France.

Objectives: Tumor necrosis factor (TNF) receptor-1 associated periodic syndrome (TRAPS) is a rare autosomal dominant autoinflammatory disorder associated with mutations in the TNF receptor super family 1A (TNFRSF1A) gene. AA amyloidosis (AA) is the most severe complication of TRAPS. To study the occurrence and prognosis of AA in TRAPS, we conducted a retrospective study of all French cases and a systematic literature review. Read More

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IL-1 Inhibitors in the Treatment of Monogenic Periodic Fever Syndromes: From the Past to the Future Perspectives.

Front Immunol 2020 1;11:619257. Epub 2021 Feb 1.

Department of Paediatric and Adult Rheumatology, University Hospital Motol, Prague, Czechia.

Autoinflammatory diseases (AIDs) represent a rare and heterogeneous group of disorders characterized by recurrent episodes of inflammation and a broad range of clinical manifestations. The most common symptoms involve recurrent fevers, musculoskeletal symptoms, and serositis; however, AIDs can also lead to life-threatening complications, such as macrophage activation syndrome (MAS) and systemic AA amyloidosis. Typical monogenic periodic fever syndromes include cryopyrin-associated periodic fever syndrome (CAPS), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), mevalonate kinase deficiency/hyper IgD syndrome (MKD/HIDS), and familial Mediterranean fever (FMF). Read More

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Advanced systemic amyloidosis secondary to metastatic renal cell carcinoma.

Ecancermedicalscience 2020 15;14:1156. Epub 2020 Dec 15.

Servicio de Oncología Médica, Fundación Instituto Valenciano de Oncología (IVO), Calle Profesor Beltrán Báguena, 8, 46009, Valencia, Spain.

Secondary amyloidosis is a rare complex complication related to chronic inflammatory disease. This complication is sparsely associated to malignant neoplasms. Renal cell carcinoma (RCC) is the most common solid organ malignancy related with this paraneoplastic syndrome. Read More

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December 2020

Disruption of CCR5 signaling to treat COVID-19-associated cytokine storm: Case series of four critically ill patients treated with leronlimab.

J Transl Autoimmun 2021 6;4:100083. Epub 2021 Jan 6.

Amarex Clinical Research, 20201 Century Blvd, Germantown, MD, 20874, USA.

Coronavirus disease 2019 (COVID-19) is associated with considerable morbidity and mortality. The number of confirmed cases of infection with SARS-CoV-2, the virus causing COVID-19 continues to escalate with over 70 million confirmed cases and over 1.6 million confirmed deaths. Read More

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January 2021

Aggregation of Mouse Serum Amyloid A Protein Was Promoted by Amyloid-Enhancing Factors with the More Genetically Homologous Serum Amyloid A.

Int J Mol Sci 2021 Jan 21;22(3). Epub 2021 Jan 21.

Muroran Institute of Technology, Graduate School of Engineering, Muroran 050-8585, Japan.

Amyloid A (AA) amyloidosis is a condition in which amyloid fibrils characterized by a linear morphology and a cross-β structure accumulate and are deposited extracellularly in organs, resulting in chronic inflammatory diseases and infections. The incidence of AA amyloidosis is high in humans and several animal species. Serum amyloid A (SAA) is one of the most important precursor amyloid proteins and plays a vital step in AA amyloidosis. Read More

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January 2021

Reactive AA Amyloidosis in the setting of infective endocarditis manifesting as bilateral orbitopathy and choroidopathy.

Retin Cases Brief Rep 2020 Dec 18;Publish Ahead of Print. Epub 2020 Dec 18.

California Pacific Medical Center, Department of Ophthalmology, 711 Van Ness Avenue, Suite 250, San Francisco, CA 94102 West Coast Retina, 1445 Bush Street, San Francisco, CA 94109.

Purpose: To describe a case of AA amyloidosis which produced an orbital inflammatory response with a novel presentation.

Methods: Case report.

Results: A 24 year-old Caucasian female with a history of intravenous (IV) heroin use was hospitalized for tricuspid valve endocarditis and methicillin resistant Staphylococcus aureus bacteremia, as well as acute renal failure. Read More

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December 2020

Infections and AA amyloidosis: An overview.

Int J Clin Pract 2021 Jun 20;75(6):e13966. Epub 2021 Jan 20.

Sorbonne Université, Service de médecine interne, GRC-28 (GRAASU), Centre national de référence des maladies autoinflammatoires et des amyloses inflammatoires (CEREMAIA), hôpital Tenon, AP-HP, Paris, France.

Background: Amyloidoses are a heterogeneous group of systemic diseases characterised by extracellular accumulation of insoluble amyloid fibrils derived from unfolded proteins. Inflammatory (AA) amyloidosis can complicate various inflammatory disorders that are associated with a sustained acute phase response and serum amyloid A (SAA) protein overproduction. Chronic infections were the first recognised cause of amyloidoses. Read More

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Hidradenitis Suppurativa: a lesser-known cause of AA amyloidosis.

Hippokratia 2020 Jan-Mar;24(1):33-37

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

Background: Hidradenitis suppurativa (HS) is a chronic, disabling skin disease. The estimated prevalence is 1-4 % worldwide. HS is a systemic inflammatory disease and can cause AA amyloidosis. Read More

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December 2020

Schnitzler Syndrome: the paradigm of an acquired adult-onset auto-inflammatory disease.

G Ital Dermatol Venereol 2020 Oct;155(5):567-573

Department of Dermatology, University Hospital of Strasbourg, Strasbourg, France.

Schnitzler Syndrome is a rare acquired auto-inflammatory syndrome defined by an urticarial eruption and a monoclonal gammopathy, mainly of the IgM kappa isotype. It shares many clinical and biological features with other autoinflammatory disorders such as NLRP3-auto-inflammatory disorders (NLRP3-AID, formerly cryopyrin associated periodic syndromes or CAPS) or adult-onset Still disease (AOSD). Hence, recurrent fever, urticarial rash with a neutrophilic infiltrate on skin biopsy (i. Read More

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October 2020

[Retrospective clinical and morphological analysis of patients with AL amyloidosis (the 2008 to 2015 nephrobiopsies)].

Arkh Patol 2020 ;82(6):29-35

Acad. I.P. Pavlov First Saint Petersburg State Medical University of the Ministry of Health of Russia, Saint Petersburg, Russia.

Objective: To study correlations between the clinical signs of dysfunction and pathological structural changes in the renal parenchyma in a group of Russian patients with AL amyloidosis in 2008-2015.

Subjects And Methods: A total group (At) including Group 1 with AL (kappa + lambda light chains) (=46) was divided into subgroups: 2λ) 40 patients with AL-lambda (AL-λ); 3κ) 6 patients with AL-kappa (AL-κ). All the patients underwent standard laboratory and instrumental studies: determinations of the peak systolic and diastolic blood pressures (SBP and DBP, respectively, mm Hg), glomerular filtration rate (GFR) (ml/min/1. Read More

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December 2020

INSAID Variant Classification and Eurofever Criteria Guide Optimal Treatment Strategy in Patients with TRAPS: Data from the Eurofever Registry.

J Allergy Clin Immunol Pract 2021 02 9;9(2):783-791.e4. Epub 2020 Nov 9.

National Amyloidosis Centre, UCL Division of Medicine, Royal Free Campus, University College London, London, United Kingdom. Electronic address:

Background: TNF receptor-associated periodic syndrome (TRAPS) is a rare autoinflammatory disease caused by dominant mutation of the TNF super family receptor 1A (TNFRSF1A) gene. Data regarding long-term treatment outcomes are lacking.

Objective: To assess correlations of genotype-phenotypes in patients with TRAPS, as defined by the International Study Group for Systemic Autoinflammatory Diseases (INSAID) classification and Eurofever criteria, with treatment responses. Read More

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February 2021

[Periodic fever syndrome associated with mutations in the TNF type 1 receptor gene: A differential diagnosis of familial Mediterranean fever that should not be overlooked in patients of Mediterranean origin].

Rev Med Interne 2021 Jul 31;42(7):459-464. Epub 2020 Oct 31.

Service de médecine interne, centre de référence des maladies auto-inflammatoires et des amyloses d'origine inflammatoire (CEREMAIA), Sorbonne université, hôpital Tenon, AP-HP, 20, rue de la Chine, 75020 Paris, France. Electronic address:

Introduction: Tumor Necrosis Factor Type 1 Receptor Associated Periodic Syndrome (TRAPS) is a rare autosomal dominant autosomal autoinflammatory disease associated with mutations in the TNF type 1 receptor gene (TNFRSF1A). It is characterized by relatively long recurrent febrile seizures with an average duration of 7 days accompanied by arthralgia, myalgia, and usually a rash. In a patient of Mediterranean origin with recurrent fever, familial Mediterranean fever is the first diagnosis to be suspected by argument of frequency. Read More

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Amyloid A amyloidosis in a patient with Caplan's syndrome, with special reference to genetic predisposition.

Mod Rheumatol Case Rep 2020 07 20;4(2):212-217. Epub 2020 Apr 20.

Section of Pneumonology, Sakurajyuji Hospital, Kumamoto, Japan.

Secondary amyloid A (AA) amyloidosis, which is a disorder of protein conformation and metabolism, is an important serious complication of inflammatory diseases, especially rheumatoid arthritis (RA). AA amyloidosis develops when AA fibrils, which are derived from the acute-phase reactant, serum amyloid AA (SAA) protein, in the circulation, are deposited in organs and cause systemic organ dysfunction. Caplan's syndrome, or rheumatoid pneumoconiosis, is a rare type of lung disease in which individuals suffering from RA develop lung nodules that are associated with occupational exposure to silica and coal dust. Read More

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The role of azurocidin in patients with familial Mediterranean fever and AA amyloidosis and its association with cardiovascular risk factors.

Int Urol Nephrol 2021 Mar 15;53(3):531-538. Epub 2020 Oct 15.

Department of Nephrology, University of Health Sciences Bozyaka Training and Research Hospital, Saim Cikrikci Street, No: 59, Karabaglar, 35360, Izmir, Turkey.

Background: Familial Mediterranean fever (FMF) is characterized by sporadic, recurrent attacks of fever and serosal inflammation. AA amyloidosis (AAA) is a disorder characterized by the extracellular tissue deposition of serum amyloid A protein (SAA). Azurocidin is a neutrophil-derived granule protein. Read More

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Investigating the pernicious effects of heparan sulfate in serum amyloid A1 protein aggregation: a structural bioinformatics approach.

J Biomol Struct Dyn 2020 Oct 14:1-15. Epub 2020 Oct 14.

Bioinformatics Lab, Department of Biotechnology, School of Bio Sciences and Technology, Vellore Institute of Technology (Deemed to be University), Vellore, Tamil Nadu, India.

Amyloid-A mediated (AA) amyloidosis is the pathogenic byproduct of body's prolonged exposure to inflammatory conditions. It is described by the aggregation of mutated/misfolded serum amyloid A1 (SAA1) protein in various tissues and organs. Genetic polymorphism G90D is suspected to cause AA amyloidosis, although the causal mechanism remains cryptic. Read More

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October 2020

AA Amyloidosis Secondary to Primary Immune Deficiency: About 40 Cases Including 2 New French Cases and a Systematic Literature Review.

J Allergy Clin Immunol Pract 2021 02 30;9(2):745-752.e1. Epub 2020 Sep 30.

Sorbonne Université, AP-HP, Hôpital Tenon, Service de médecine interne, Centre de référence des maladies auto-inflammatoires et des amyloses d'origine inflammatoire (CEREMAIA), GRC-28 (Groupe de recherche clinique amylose AA Sorbonne univeristé), Paris, France. Electronic address:

Background: Primary immune deficiencies (PIDs) are a heterogeneous group of disorders resulting from defects in immune system. They lead to increased susceptibility to infections and immune dysregulation. The resulting chronic inflammation can induce long-term complications, including AA amyloidosis (AAA). Read More

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February 2021

The systemic autoinflammatory disorders for dermatologists. Part 2: disease examples.

Clin Exp Dermatol 2020 Dec 3;45(8):967-973. Epub 2020 Sep 3.

Portsmouth Hospitals NHS Trust, National Amyloidosis Centre, UCL Division of Medicine and Royal Free London NHS Foundation Trust, Portsmouth, Hampshire, UK.

The systemic autoinflammatory disorders (SAIDS) or periodic fever syndromes are disorders of innate immunity, which can be inherited or acquired. They are almost all very rare and easily overlooked; typically, patients will have seen multiple specialities prior to diagnosis, so a high level of clinical suspicion is key. It is important to note that these are 'high-value' diagnoses as the majority of these syndromes can be very effectively controlled, dramatically improving quality of life and providing protection against the development of irreversible complications such as AA amyloidosis. Read More

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December 2020

Gastric AA amyloidosis secondary to chronic infection presenting with hematemesis: a case report.

Clin J Gastroenterol 2020 Dec 27;13(6):1070-1073. Epub 2020 Aug 27.

University of Maryland School of Medicine, Baltimore, 21201, USA.

AA amyloidosis, previously known as secondary amyloidosis, has been associated with multiple chronic inflammatory conditions, including various autoimmune diseases and rarely chronic infection. Hereby, we present a case of AA amyloidosis secondary to chronic infection which initially presented with nausea and hematemesis. Endoscopic biopsies revealed diffuse AA amyloid deposition in the stomach, but not the esophagus. Read More

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December 2020