548 results match your criteria Amyloidosis AA Inflammatory


Renal Amyloidosis: Epidemiological, Clinical, and Laboratory Profile in Adults from One Nephrology Center.

Int J Nephrol 2022 18;2022:8493479. Epub 2022 Jul 18.

Department of Medicine A, Charles Nicolle Hospital, Tunis, Tunisia.

Background: Renal amyloidosis is one of the main differential diagnoses of nephrotic proteinuria in adults and the elderly. The aim of this study with the most important series in our country is to contribute to the epidemiological, clinical, and etiological study of the renal amyloidosis.

Methods: In a retrospective study carried out between 1975 and 2019, 310 cases of histologically proven and typed renal amyloidosis were selected for this study. Read More

View Article and Full-Text PDF

Serum Amyloid A Protein-Associated Kidney Disease: Presentation, Diagnosis, and Management.

Kidney Med 2022 Aug 26;4(8):100504. Epub 2022 Jun 26.

Department of Medicine, Dalhousie University and Nova Scotia Health.

Serum amyloid A protein (AA) amyloidosis, also known as secondary amyloidosis, is a known consequence of chronic inflammation and results from several conditions including inflammatory arthritis, periodic fever syndromes, and chronic infection. AA amyloidosis can lead to multiorgan dysfunction, including changes in glomerular filtration rate and proteinuria. Definitive diagnosis requires tissue biopsy, and management of AA amyloid kidney disease is primarily focused on treating the underlying inflammatory condition to stabilize glomerular filtration rate, reduce proteinuria, and slow potential progression to kidney failure. Read More

View Article and Full-Text PDF

Gain-of-function mutations in cause an NF-κB-mediated autoinflammatory disease: functional assessment, clinical phenotyping and disease course of patients with ROSAH syndrome.

Ann Rheum Dis 2022 Jul 22. Epub 2022 Jul 22.

Inflammatory Disease Section, National Human Genome Research Institute, Bethesda, Maryland, USA.

Objectives: To test the hypothesis that ROSAH (retinal dystrophy, optic nerve oedema, splenomegaly, anhidrosis and headache) syndrome, caused by dominant mutation in , is an autoinflammatory disease.

Methods: This cohort study systematically evaluated 27 patients with ROSAH syndrome for inflammatory features and investigated the effect of mutations on immune signalling. Clinical, immunologic and radiographical examinations were performed, and 10 patients were empirically initiated on anticytokine therapy and monitored. Read More

View Article and Full-Text PDF

AA Amyloidosis associated with cancers.

Nephrol Dial Transplant 2022 Jul 22. Epub 2022 Jul 22.

Department of Nephrology, Peupliers Private Hospital, Ramsay Générale de Santé, Paris, France.

Systemic AA amyloidosis is associated with systemic inflammatory processes such as autoimmune disorders or chronic infections. In addition, AA amyloidosis can develop in a localized or systemic form in patients with malignant neoplastic disorders, and usually involves kidneys impacting renal function. Among solid tumors, renal cell carcinoma (RCC) appears to be responsible for one-quarter to half of all cancers associated with amyloidosis. Read More

View Article and Full-Text PDF

[Nephrotic Syndrome Caused by AA Amyloidosis Secondary to Unicentric Castleman's Disease:Report of One Case].

Zhongguo Yi Xue Ke Xue Yuan Xue Bao 2022 Jun;44(3):540-544

Department of Nephrology,PUMC Hospital,CAMS and PUMC,Beijing 100730,China.

AA amyloidosis is a rare systemic complication caused commonly by chronic inflammatory arthritis,periodic fever disease,vasculitis,tumors,etc.Castleman's disease is an uncommon cause of AA amyloidosis.Here,we reported a case of unicentric Castleman's disease-induced AA amyloidosis with nephrotic syndrome as the main manifestation. Read More

View Article and Full-Text PDF

Anti-Inflammatory, Antioxidant, and Anti-Atherosclerotic Effects of Natural Supplements on Patients with FMF-Related AA Amyloidosis: A Non-Randomized 24-Week Open-Label Interventional Study.

Life (Basel) 2022 Jun 15;12(6). Epub 2022 Jun 15.

Department of Paediatrics, Division of Paediatric Rheumatology, Schulich School of Medicine & Dentistry, University of Western Ontario, London, ON N6A 5W9, Canada.

We aimed to evaluate the effect of a combination of natural products on parameters related to inflammation, endothelial dysfunction, and oxidative stress in a cohort of familial Mediterranean fever (FMF) patients with Serum Amyloid A amyloidosis, in a non-randomized, 24-week open-label interventional study. (anti-atherosclerotic-AAL), omega-3 (anti-inflammatory-AIC), and extract with Alaskan blueberry (antioxidant-AOL) were given to patients with FMF-related biopsy-proven AA amyloidosis. Patients were >18 years and had proteinuria (>3500 mg/day) but a normal estimated glomerular filtration rate (eGFR). Read More

View Article and Full-Text PDF

Frequency of AA amyloidosis has decreased in Behçet's syndrome: a retrospective study with long-term follow-up and a systematic review.

Rheumatology (Oxford) 2022 Jun 3. Epub 2022 Jun 3.

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

Objective: A decline in the frequency of AA amyloidosis secondary to RA and infectious diseases has been reported. We aimed to determine the change in the frequency of AA amyloidosis in our Behçet's syndrome (BS) patients and to summarize the clinical characteristics of and outcomes for our patients, and also those identified by a systematic review.

Methods: We identified patients with amyloidosis in our BS cohort (as well as their clinical and laboratory features, treatment, and outcome) through a chart review. Read More

View Article and Full-Text PDF

COVID-19 infection among patients with autoinflammatory diseases: a study on 117 French patients compared with 1545 from the French RMD COVID-19 cohort: COVIMAI - the French cohort study of SARS-CoV-2 infection in patient with systemic autoinflammatory diseases.

RMD Open 2022 05;8(1)

Sorbonne University, AP-HP, Tenon Hospital, Internal Medicine Department, 4 rue de la Chine, 75020, Paris, France; national Reference center for autoinflammatory diseases and AA amyloidosis (CEREMAIA), Tenon Hospital, Paris, France

Objective: There is little known about SARS-CoV-2 infection in patients with systemic autoinflammatory disease (SAID). This study aimed to describe epidemiological features associated with severe disease form and death. Mortality between patients with and without SAID hospitalised for SARS-CoV-2 infection was compared. Read More

View Article and Full-Text PDF

Down-Regulated Th17 Cells in a Patient With Familial Mediterranean Fever Associated with AA Amyloidosis in the Treatment of Canakinumab.

Mod Rheumatol Case Rep 2022 Mar 29. Epub 2022 Mar 29.

Section of Rheumatology, Sakurajyuji Hospital, Kumamoto, Japan.

Autoinflammatory diseases are innate immune-mediated inflammatory disorders, unlike autoimmune diseases, which are characterised by abnormalities in adoptive immunity, although autoimmune and autoinflammatory diseases have certain similar clinical features. Familial Mediterranean fever (FMF), the most common monogenic autoinflammatory disease, is associated with mutations in the MEFV gene that encodes pyrin, which results in inflammasome activation and uncontrolled production of interleukin (IL)-1β. Regular use of colchicine, the primary drug for FMF treatment, prevents febrile attacks and reduces long-term risk of subsequent complications of amyloid A (AA) amyloidosis. Read More

View Article and Full-Text PDF

Interventions for reducing inflammation in familial Mediterranean fever.

Cochrane Database Syst Rev 2022 03 29;3:CD010893. Epub 2022 Mar 29.

Department of Pharmacy, West China Hospital, Sichuan University, Chengdu, China.

Background: Familial Mediterranean fever (FMF), a hereditary auto-inflammatory disease, mainly affects ethnic groups living in the Mediterranean region. Early studies reported colchicine may potentially prevent FMF attacks. For people who are colchicine-resistant or intolerant, drugs such as anakinra, rilonacept, canakinumab, etanercept, infliximab or adalimumab might be beneficial. Read More

View Article and Full-Text PDF

A patient with urticarial lesions, recurrent fever, and IgM-type monoclonal gammopathy.

Acta Dermatovenerol Alp Pannonica Adriat 2022 03;31(Suppl):S27-S29

Department of Dermatovenereology, Ljubljana University Medical Centre, Ljubljana, Slovenia.

Schnitzler syndrome is a rare acquired autoinflammatory syndrome. It presents with an urticarial rash and a monoclonal gammopathy, usually of the IgM kappa type. In addition, patients can present with bone and/or joint pain, recurrent fever, asthenia, weight loss, myalgia, headache, lymphadenopathy, hepatomegaly, or splenomegaly. Read More

View Article and Full-Text PDF

Rare cause of thyroid enlargement: Localized AA amyloid goiter - A case report.

Int J Surg Case Rep 2022 Mar 25;92:106876. Epub 2022 Feb 25.

Kasturba Medical College, Manipal, Manipal Academy of Higher Education, India.

Introduction: Amyloidosis is extracellular deposition of fibrillary amyloid proteins in various organs. Amyloid infiltration in thyroid is common; however, the occurrence of clinically enlarged thyroid, subsequently leading to goiter, is a rare phenomenon.

Case Presentation: 36 years old female presented to our OPD with multinodular goiter. Read More

View Article and Full-Text PDF

AA amyloidosis without systemic inflammation: when clinical evidence validates predictions of experimental medicine.

Kidney Int 2022 02;101(2):219-221

Department of Molecular Medicine, University of Pavia, Pavia, Italy; Wolfson Drug Discovery Unit, National Amyloidosis Centre, Division of Medicine, University College London, London, UK.

Amyloid A (AA) amyloidosis is a well-known consequence of chronic inflammatory diseases in which elevated plasma concentrations of serum amyloid A result in amyloid aggregation and organ damage. In this issue, Sikora et al. report, for the first time, an inherited form of AA amyloidosis occurring in the absence of systemic inflammation. Read More

View Article and Full-Text PDF
February 2022

Plasminogen gene polymorphisms [c.924C>T and IVS 8+14 G>A] in periodontitis and familial Mediterranean fever: A case-control study.

J Periodontal Res 2022 Apr 22;57(2):371-380. Epub 2021 Dec 22.

Biostatistics and Medical Informatics Department, Faculty of Medicine, University of Suleyman Demirel, Isparta, Turkey.

Background And Objective: The plasminogen (PLG) activation system plays an essential role in severe inflammation based diseases such as periodontitis, destructive membranous periodontal disease (ligneous periodontitis), familial Mediterranean fever (FMF), and amyloidosis. We have aimed to evaluate variations in PLG and the associations between PLG and MEFV genotypes in patients with FMF/ FMF-related secondary amyloidosis and periodontitis.

Material And Methods: A total of 247 individuals who were either diagnosed with FMF or systemically healthy were recruited to this human observational study with a cross-sectional design. Read More

View Article and Full-Text PDF

AA-amyloidosis in captive northern tree shrews ().

Vet Pathol 2022 03 21;59(2):340-347. Epub 2021 Dec 21.

University of Veterinary Medicine Hannover, Foundation, Hannover, Germany.

A high prevalence of AA-amyloidosis was identified in a breeding colony of northern tree shrews () in a retrospective analysis, with amyloid deposits in different organs being found in 26/36 individuals (72%). Amyloid deposits, confirmed by Congo red staining, were detected in kidneys, intestines, skin, and lymph nodes, characteristic of systemic amyloidosis. Immunohistochemically, the deposited amyloid was intensely positive with anti-AA-antibody (clone mc4), suggesting AA-amyloidosis. Read More

View Article and Full-Text PDF

Identification of the minimal active soluble TREM2 sequence for modulating microglial phenotypes and amyloid pathology.

J Neuroinflammation 2021 Dec 10;18(1):286. Epub 2021 Dec 10.

Fujian Provincial Key Laboratory of Neurodegenerative Disease and Aging Research, Institute of Neuroscience, School of Medicine, Xiamen University, Xiamen, 361102, China.

Background: TREM2 is a microglial receptor genetically linked to the risk for Alzheimer's disease (AD). The cerebrospinal fluid (CSF) levels of soluble TREM2 (sTREM2) have emerged as a valuable biomarker for the disease progression in AD and higher CSF levels of sTREM2 are linked to slower cognitive decline. Increasing sTREM2 in mouse models of amyloidosis reduces amyloid-related pathology through modulating microglial functions, suggesting a beneficial role of sTREM2 in microglia biology and AD pathology. Read More

View Article and Full-Text PDF
December 2021

AA renal amyloidosis: Clinical observations over 20 years.

Clin Nephrol 2022 Mar;97(3):167-172

Objective: AA renal amyloidosis is present in Algeria, often secondary to chronic infections, the most frequent being tuberculosis. We studied the evolution of the epidemiology of AA amyloidosis over a period of 20 years.

Materials And Methods: We conducted a retrospective study of all adult and pediatric patients diagnosed with renal symptomatology of AA amyloidosis from 1994 to 2014 inclusive. Read More

View Article and Full-Text PDF

Azacitidine for patients with Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic syndrome (VEXAS) and myelodysplastic syndrome: data from the French VEXAS registry.

Br J Haematol 2022 02 14;196(4):969-974. Epub 2021 Oct 14.

Department of Internal Medicine, Assistance Publique Hôpitaux de Paris (AP-HP), Hôpital Saint-Antoine, Sorbonne University, Paris, France.

Azacitidine can be effective in myelodysplastic syndromes (MDS) associated with inflammatory/autoimmune diseases. Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic syndrome (VEXAS) is a new monogenic autoinflammatory syndrome caused by somatic ubiquitin-like modifier-activating enzyme 1 (UBA1) mutation, often associated with MDS, whose treatment is difficult and not yet codified. Based on a French nationwide registry of 116 patients with VEXAS, we report the efficacy and safety of azacitidine treatment in 11 patients with VEXAS with MDS. Read More

View Article and Full-Text PDF
February 2022

2021 update of the EULAR points to consider on the use of immunomodulatory therapies in COVID-19.

Ann Rheum Dis 2022 01 7;81(1):34-40. Epub 2021 Oct 7.

Department of Rheumatology, INSERM UMR1184, Le Kremlin Bicêtre, France

Objectives: To update the EULAR points to consider (PtCs) on the use of immunomodulatory therapies in COVID-19.

Methods: According to the EULAR standardised operating procedures, a systematic literature review up to 14 July 2021 was conducted and followed by a consensus meeting of an international multidisciplinary task force. The new statements were consolidated by formal voting. Read More

View Article and Full-Text PDF
January 2022

A mutation in the SAA1 promoter causes hereditary amyloid A amyloidosis.

Kidney Int 2022 02 21;101(2):349-359. Epub 2021 Sep 21.

Research Unit for Rare Diseases, Department of Paediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General Teaching Hospital in Prague, Prague, Czech Republic; Section on Nephrology, Wake Forest School of Medicine, Winston-Salem, North Carolina, USA. Electronic address:

Amyloid A amyloidosis is a serious clinical condition resulting from the systemic deposition of amyloid A originating from serum amyloid A proteins with the kidneys being the most commonly and earliest affected organ. Previously described amyloid A amyloidosis is linked to increased production and deposition of serum amyloid A proteins secondary to inflammatory conditions arising from infectious, metabolic, or genetic causes. Here we describe a family with primary amyloid A amyloidosis due to a chr11:18287683 T>C (human genome version19) mutation in the SAA1 promoter linked to the amyloidogenic SAA1. Read More

View Article and Full-Text PDF
February 2022

Mevalonate Kinase Deficiency: A Cause of Severe Very-Early-Onset Inflammatory Bowel Disease.

Inflamm Bowel Dis 2021 10;27(11):1853-1857

Department of Paediatric Immunology, Hematology, and Rheumatology, Groupe Hospitalier Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Reference Centre for Rheumatic, Autoimmune and Systemic Diseases in Children, Assistance Publique-Hôpitaux de Paris, and Imagine Foundation, Paris, France.

Mevalonate kinase deficiency should be considered in patients with severe very-early-onset inflammatory bowel disease (IBD), especially in patients with a history of recurrent or chronic fever, peritoneal adhesions, and atypical IBD pathology. Anti-interleukin-1 therapy may be efficacious in these patients with monogenic very-early-onset IBD. Read More

View Article and Full-Text PDF
October 2021

AA amyloidosis complicating monoclonal gammopathies, an unusual feature validating the concept of "monoclonal gammopathy of inflammatory significance"?

Int J Clin Pract 2021 Nov 21;75(11):e14817. Epub 2021 Sep 21.

Department of Internal Medicine, Centre de référence des maladies auto-inflammatoires et des amyloses d'origine inflammatoire (CEREMAIA), Sorbonne University, AP-HP, Tenon Hospital, Paris, France.

Introduction: AL amyloidosis is caused by the proliferation of an immunoglobulin-secreting B cell clone. AA amyloidosis is a rare complication of chronic inflammation. However, some patients present with diseases combining monoclonal immunoglobulin production and chronic inflammation. Read More

View Article and Full-Text PDF
November 2021

Gastrointestinal AA amyloidosis secondary to chronic pyelonephritis presenting with refractory diarrhea and severe hypoalbuminemia.

Clin J Gastroenterol 2021 Dec 1;14(6):1642-1648. Epub 2021 Sep 1.

Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, 23-3 Matsuoka Shimoaizuki, Eiheiji-cho, Yoshida-gun, Fukui, 910-1193, Japan.

Secondary amyloidosis is a rare complication of chronic inflammatory diseases, such as collagen diseases, and is often difficult to treat. In addition, the gastrointestinal tract is frequently involved in amyloid deposition that often results in various disorders and symptoms. A 70-year-old woman was admitted to our hospital with refractory diarrhea and hypoalbuminemia. Read More

View Article and Full-Text PDF
December 2021

Biologic therapy for amyloid A amyloidosis secondary to rheumatoid arthritis treated with interleukin 6 therapy: Case report and review of literature.

Medicine (Baltimore) 2021 Aug;100(32):e26843

Department of Rheumatology, Ajou University School of Medicine, Suwon, Korea.

Introduction: Secondary amyloidosis is a rare complication of rheumatoid arthritis (RA) that is histologically characterized by the deposition of amyloid fibrils in target organs, such as the kidneys and gastrointestinal tract. Controlling the inflammatory response is essential to prevent organ dysfunction in amyloid A (AA) amyloidosis secondary to RA, and no clear treatment strategy exists.

Patient Concerns And Diagnosis: A 66-year-old woman with RA, who had been treated with disease-modifying anti-rheumatic drugs for 1 year, presented with recurrent abdominal pain and prolonged diarrhea. Read More

View Article and Full-Text PDF

Gout and AA-Amyloidosis: A Case-Based Review.

Mediterr J Rheumatol 2021 Mar 15;32(1):74-80. Epub 2021 Feb 15.

Pirogov Russian National Research Medical University (RNRMU), Moscow, Russian Federation.

Background: AA-amyloidosis complicates many chronic infections and inflammatory diseases, including rheumatoid arthritis, ankylosing spondylitis, and psoriatic arthritis, but its relationship to gout is extremely rare. As it is unknown definitely what the pathophysiological connections between gout and amyloidosis are, treatment issues of the diseases are open for discussion.

Aim: To establish a link between gout and AA-amyloidosis, and to improve the quality of treatment in patients suffering from gout and AA-amyloidosis. Read More

View Article and Full-Text PDF

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

View Article and Full-Text PDF

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

View Article and Full-Text PDF
November 2021

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

View Article and Full-Text PDF

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 15;145(6):675-683. 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

View Article and Full-Text PDF