Raras
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Amiloidose AA
ORPHA:85445CID-10 · E85.3CID-11 · 5D00.1DOENÇA RARA

A amiloidose secundária é uma forma de amiloidose que complica doenças inflamatórias crônicas (principalmente artrite reumatóide) e é caracterizada pela agregação e deposição de fibrilas amilóides compostas de proteína amiloide A sérica, um reagente de fase aguda. Embora o baço, a glândula suprarrenal, o fígado e o intestino sejam locais frequentes de deposição de amiloide, o quadro clínico é dominado pelo envolvimento renal.

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Introdução

O que você precisa saber de cara

📋

A amiloidose secundária é uma forma de amiloidose que complica doenças inflamatórias crônicas (principalmente artrite reumatóide) e é caracterizada pela agregação e deposição de fibrilas amilóides compostas de proteína amiloide A sérica, um reagente de fase aguda. Embora o baço, a glândula suprarrenal, o fígado e o intestino sejam locais frequentes de deposição de amiloide, o quadro clínico é dominado pelo envolvimento renal.

Pesquisas ativas
1 ensaio
7 total registrados no ClinicalTrials.gov
Publicações científicas
1.445 artigos
Último publicado: 2026 Apr 14

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Adolescent
+ adult, childhood
🏥
SUS: Cobertura mínimaScore: 20%
1 medicamentos CEAFCID-10: E85.3
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🫘
Rins
9 sintomas
🫃
Digestivo
7 sintomas
🧠
Neurológico
2 sintomas
❤️
Coração
2 sintomas
🩸
Sangue
1 sintomas
📏
Crescimento
1 sintomas

+ 10 sintomas em outras categorias

Características mais comuns

90%prev.
Nefropatia
Muito frequente (99-80%)
90%prev.
Amiloidose renal
Muito frequente (99-80%)
90%prev.
Hipotensão
Muito frequente (99-80%)
90%prev.
Amiloidose
Muito frequente (99-80%)
90%prev.
Proteinúria
Muito frequente (99-80%)
55%prev.
Morfologia anormal da mucosa oral
Frequente (79-30%)
32sintomas
Muito frequente (5)
Frequente (13)
Ocasional (10)
Muito raro (4)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 32 características clínicas mais associadas, ordenadas por frequência.

NefropatiaNephropathy
Muito frequente (99-80%)90%
Amiloidose renalRenal amyloidosis
Muito frequente (99-80%)90%
HipotensãoHypotension
Muito frequente (99-80%)90%
AmiloidoseAmyloidosis
Muito frequente (99-80%)90%
ProteinúriaProteinuria
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico1.445PubMed
Últimos 10 anos200publicações
Pico202570 papers
Linha do tempo
2026Hoje · 2026🧪 2001Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

1 gene identificado com associação a esta condição. Padrão de herança: Not applicable.

SAA1Serum amyloid A-1 proteinMajor susceptibility factor inModerado
FUNÇÃO

Major acute phase protein

LOCALIZAÇÃO

Secreted

VIAS BIOLÓGICAS (1)
Amyloid fiber formation
EXPRESSÃO TECIDUAL(Ubíquo)
Fígado
8842.0 TPM
Glândula salivar
1622.0 TPM
Adipose Visceral Omentum
1116.1 TPM
Mama
933.0 TPM
Tecido adiposo
229.3 TPM
OUTRAS DOENÇAS (1)
AA amyloidosis
HGNC:10513UniProt:P0DJI8

Variantes genéticas (ClinVar)

16 variantes patogênicas registradas no ClinVar.

🧬 SAA1: NM_199161.5(SAA1):c.230C>G (p.Thr77Ser) ()
🧬 SAA1: GRCh37/hg19 11p15.1-12(chr11:17120358-41424289)x1 ()
🧬 SAA1: GRCh37/hg19 11p15.5-14.2(chr11:230615-26881146)x3 ()
🧬 SAA1: Single allele ()
🧬 SAA1: GRCh37/hg19 11p15.2-14.1(chr11:13970757-27565888)x3 ()
Ver todas no ClinVar

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
3Fase 32
2Fase 21
1Fase 11
·Pré-clínico2
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 6 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Amiloidose AA

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

1 pesquisa recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

7 ensaios clínicos encontrados, 1 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

🥉Melhor nível de evidência: Relato de caso
Timeline de publicações
624 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 624

#1

Canakinumab treatment in patients with colchicine-resistant familial mediterranean fever: a multicenter observational study.

Turkish journal of medical sciences2026

Anti-interleukin-1 agents have known beneficial effects in the treatment of colchicine-resistant familial Mediterranean fever (cr-FMF); however, studies to date have tended to have small sample sizes and to be based on pooled data. The present study investigates the efficacy of Canakinumab (CAN) in a homogeneous cohort of cr-FMF cases. The study included patients who underwent treatment in three tertiary rheumatology departments, whose electronic medical records were reviewed retrospectively. The inclusion criteria were presence of colchicine resistant disease activity or persistent proteinuria secondary to AA-amyloidosis, and treatment with CAN for at least 6 months. Clinical and laboratory parameters were assessed before and after CAN treatment. The study included 65 patients with a mean age of 38.2 ± 13.8 years, with a mean disease duration of 23.7 ± 11.4 years and a mean colchicine dosage of 1.5 ± 0.6 mg/day. Of the total, 60% of the patients had an M694V homozygous mutation, and 41.5% were resistant to Anakinra. Furthermore, 25 had FMF-related amyloidosis, and 16 were renal transplant recipients. The mean CAN treatment duration was 31.3 ± 23.1 months, and 80% of patients achieved complete remission, while 20% achieved partial remission. Erythrocyte sedimentation rate, C-reactive protein, frequency of attacks, and patient global assessment decreased significantly after CAN (p < 0.001 for each). The mean serum creatinine level (mg/dl) decreased from 2.1 ± 0.8 to 1.4 ± 0.8 (p < 0.001), and median proteinuria (mg/day) decreased from 1475 to 675 (p < 0.001) in patients with AA-amyloidosis. Only one patient with chronic monoarthritis affecting the wrist discontinued CAN due to insufficient arthritis relief. Canakinumab demonstrates excellent efficacy and favorable safety as a treatment for cr-FMF. Our study is the first to indicate the efficacy of CAN in reducing serum creatinine levels.

#2

A case of AA amyloidosis with biliary cystadenoma and cervical thymoma in a striped skunk (Mephitis mephitis).

The Journal of veterinary medical science2026 Jan 16

A 6-year and 11-months-old male striped skunk, Mephitis mephitis, showed inappetence and died. Necropsy revealed multiple hepatic masses, an enlarged spleen, wedge-shaped lesions in the kidneys, and a cervical cyst with white nodules. Histopathologically, the hepatic masses were diagnosed as biliary cystadenomas accompanied by chronic inflammation, the cervical cyst was a thymoma, and the splenic and renal lesions were diagnosed as infarctions. Amorphous eosinophilic material was observed in the liver, spleen, and kidney. These proteinaceous deposits were positive for Congo red staining with yellowish to green birefringence under polarized light. These deposits were immunohistochemically positive for amyloid A (AA). This report describes the pathological features of AA amyloidosis, biliary cystadenoma and cervical thymoma in a striped skunk.

#3

AA amyloidosis with clinically overt cardiac involvement confirmed by amyloid A deposition in aortic valve fibroelastoma.

European heart journal2026 Feb 23
#4

AA amyloidosis in inflammatory joint diseases in the era of biological therapies: prevalence, manifestations, management and evolution.

Joint bone spine2026 Mar 19

AA amyloidosis (AAA) is a complication of chronic inflammation whose burden is expected to decrease in the era of biological therapies. We assessed the prevalence, clinical and laboratory manifestations, management and evolution of AAA in patients with inflammatory joint diseases (IJDs). We retrospectively assessed adults followed from 2008 to 2025 in 2 French tertiary university hospitals. AAA had to be histologically confirmed and related to one of the following IJDs: rheumatoid arthritis, spondylarthritis, juvenile idiopathic arthritis, undifferentiated arthritis or gout. The prevalence of rheumatoid arthritis- and spondylarthritis-related AAA was low, estimated at 0.6‰ and 0.5‰, respectively. We identified 15 patients with IJD-related AAA: 5 rheumatoid arthritis, 4 spondylarthritis, 2 juvenile idiopathic arthritis, 3 undifferentiated arthritis and 1 gout. Ten (66.7%) patients were from developing countries. All patients experienced delays in diagnosis and treatment. At AAA diagnosis, most patients (80%) were not receiving any treatment for IJD. AAA clinical manifestations were mainly renal and digestive; the median (interquartile range) C-reactive protein level was 41.5 (16.8-59.5) mg/L, serum amyloid A level 31 (13-44) mg/L and proteinuria/creatinuria ratio 3.5 (1.3-5.2) g/mmol. Biological disease-modifying anti-rheumatic drugs were started in 14 patients (1 patient lost to follow-up) and resulted in clinically inactive disease in 57.2%; normal C-reactive protein and serum amyloid A and proteinuria negativity in 71.4%. In the last 2 decades, AAA occurred in only patients with IJDs who experienced long diagnostic and therapeutic delays, mainly related to country of origin and difficulties in access to care.

#5

Case Report: Anti-TNF-α therapy-associated destructive thyroiditis and unmasking of latent amyloid A amyloidosis in rheumatoid arthritis.

Frontiers in immunology2026

Tumor necrosis factor (TNF)-α inhibitors are widely used for rheumatoid arthritis (RA), but paradoxical immune reactions, including autoimmune thyroid disease, have been reported. We describe a 71-year-old man with a 16-year history of RA who developed destructive thyroiditis after initiation of certolizumab pegol. Despite symptom resolution, he subsequently developed acute renal failure and diarrhea. Biochemical and histological analyses revealed elevated serum amyloid A (AA) and amyloid deposition in the kidney and duodenum, confirming AA amyloidosis. We considered that the latent amyloidosis became clinically apparent following immune modulation by the anti-TNF-α biologic. Treatment with the IL-6 receptor antibody tocilizumab rapidly normalized inflammatory markers and improved both renal function and gastrointestinal symptoms. This case highlights that TNF-α inhibition may paradoxically unmask underlying amyloidosis and induce autoimmune thyroiditis. Clinicians should monitor thyroid and systemic amyloid markers when introducing biologic therapy for long-standing RA.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC610 artigos no totalmostrando 196

2026

AA amyloidosis in inflammatory joint diseases in the era of biological therapies: prevalence, manifestations, management and evolution.

Joint bone spine
2026

Case Report: Anti-TNF-α therapy-associated destructive thyroiditis and unmasking of latent amyloid A amyloidosis in rheumatoid arthritis.

Frontiers in immunology
2026

Canakinumab treatment in patients with colchicine-resistant familial mediterranean fever: a multicenter observational study.

Turkish journal of medical sciences
2026

Childhood-onset hypocomplementaemic urticarial vasculitis in France: phenotypic and genotypic diversity in 10 children.

Pediatric rheumatology online journal
2026

Association Between Common Variable Immunodeficiency and Pulmonary Amyloidosis: Review.

Journal of clinical medicine
2026

Graft and Recipient Survival Analysis of Renal Transplantation in Rheumatic Diseases: A Retrospective Case-Control Study.

Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation
2026

Stroke characteristics in giant cell arteritis and Takayasu arteritis: A multicenter retrospective cohort study of 108 patients.

Seminars in arthritis and rheumatism
2026

Tocilizumab prevented the progression of AA amyloidosis derived from adult-onset Still's disease.

Clinical nephrology
2025

Thrombosis Despite Sub-Nephrotic Proteinuria.

Cureus
2026

Renal Biopsy as a Diagnostic Salvage Modality in Gouty Nephropathy Coexisting With Amyloid A Amyloidosis.

International journal of surgical pathology
2026

An intriguing case report of coincidental occurrence of Amyloid A amyloidosis with renal cell carcinoma in the ipsilateral kidney and adrenal gland.

Indian journal of pathology &amp; microbiology
2026

In situ, antibody-independent, and multiplexed characterization of amyloid plaques by MALDI MS/MS imaging using iprm-PASEF.

Analytical and bioanalytical chemistry
2025

The Interplay Between Rheumatoid Arthritis and Chronic Kidney Disease: From Mechanisms to Treatment.

Journal of clinical medicine
2026

Assessment of the biochemical basis underlying the resistance against systemic amyloidosis.

Scientific reports
2025

Prognostication: A Young Patient With AA Amyloidosis.

Cureus
2025

Destabilization of Helix III Initiates Early Serum Amyloid A Misfolding by Exposing Its Amyloidogenic Core.

The journal of physical chemistry letters
2025

Endoscopic Diagnosis and Clinical Course of Isolated Upper Gastrointestinal Amyloidosis: A Retrospective Observational Study.

The Korean journal of helicobacter and upper gastrointestinal research
2025

The Inflammatory Role of Serum Amyloid A in the Pathogenesis and Progression of Diabetic Nephropathy.

Journal of clinical medicine
2025

Amyloid A Amyloidosis Secondary to Thymoma.

Diagnostics (Basel, Switzerland)
2025

Amyloid A amyloidosis associated with Streptococcus suis infection in a young commercial pig with wild-type serum amyloid A.

Veterinary research communications
2025

Efficacy and safety of anti-interleukin-6 treatment in familial Mediterranean fever: a systematic literature review.

Reumatologia
2025

Early-onset gastrointestinal amyloid A amyloidosis without renal involvement in a patient with RA-pSpA overlap: A case report.

Medicine
2025

Epigallocatechin-3-Gallate: A potential amyloid Fibril Disaggregator of Serum amyloid A1.

Biochemistry and biophysics reports
2026

AA amyloidosis with clinically overt cardiac involvement confirmed by amyloid A deposition in aortic valve fibroelastoma.

European heart journal
2025

Case Report: A rare case of concurrence of IgG4-related tubulointerstitial nephritis and renal Amyloid A amyloidosis.

Frontiers in immunology
2026

AA amyloidosis as an adverse event of immune checkpoint inhibitor therapy: evidence from the FDA adverse event reporting system and a systematic review.

Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
2025

Renal AA amyloidosis following COVID-19 infection.

Nephrologie &amp; therapeutique
2026

A case of AA amyloidosis with biliary cystadenoma and cervical thymoma in a striped skunk (Mephitis mephitis).

The Journal of veterinary medical science
2025

[An unusual cause for kidney transplantation].

Innere Medizin (Heidelberg, Germany)
2026

Clinical profile and outcome of AA amyloidosis associated kidney disease in India.

Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
2025

Case Report: Expanding the spectrum of renal involvement in adult-onset still's disease: a case of focal proliferative glomerulonephritis and ischemic nephropathy.

Frontiers in medicine
2025

From ulcerative colitis to amyloid storm: a case-based review.

Rheumatology international
2025

Effect of MEFV mutations and HLA-B27 on clinical findings of familial Mediterranean fever and spondyloarthritis.

Medicine
2025

Amyloid and Light Chain Deposition Disorders.

Clinics in chest medicine
2025

The decline of AA amyloidosis in familial Mediterranean fever is significant, but the story is not over.

Clinical and experimental rheumatology
2026

Renal AA Amyloidosis Associated With Systemic Lupus Erythematosus Treated With Anakinra.

The Journal of rheumatology
2025

Comparative Histopathological Characteristics of Duodenal Involvement in Different Types of Amyloidosis.

Biomedicines
2025

Cryo-EM Observation of AA Amyloid Fibrils in Mouse Model of Systemic AApoAII Amyloidosis.

Journal of molecular biology
2025

Acquired Renal Amyloidosis in a Patient With X-Linked Hyper-IgM Immunodeficiency With Novel Hemizygotic Pathogenic Variant in CD40LG Gene.

Case reports in nephrology
2025

Carpal tunnel syndrome with both AA amyloidosis and elderly-onset Still disease: A case report.

Medicine
2025

Secondary amyloidosis as a cause of nephrotic syndrome in Takayasu arteritis.

BMJ case reports
2025

Idiopathic AA amyloidosis presenting with initial abdominal pain: a case report and literature review.

Frontiers in medicine
2025

AA-Amyloidosis in the Eurasian stone-curlew (Burhinus oedicnemus).

PloS one
2025

Serum Amyloid A3 Expression Is Enhanced by Gram-Negative Bacterial Stimuli in Bovine Endometrial Epithelial Cells.

Pathogens (Basel, Switzerland)
2025

Systemic AA Amyloidosis Secondary to Chronic Bronchiectasis and Primary Immunodeficiency: A Case Report.

Cureus
2025

Acute Renal Failure Due to Amyloidosis Associated With Intravenous Heroin Use.

Cureus
2025

Pre-existing oncohematological disease in kidney transplant recipients: impact on graft survival, acute rejection, and long-term clinical outcomes.

Frontiers in immunology
2025

Unmasking Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS): Diagnostic pitfalls of de novo mutations.

The journal of allergy and clinical immunology. In practice
2025

A Rare Case of Cerebral Amyloidoma Mimicking Thalamic Glioma in a Rheumatoid Arthritis Patient.

Pathophysiology : the official journal of the International Society for Pathophysiology
2025

R202Q homozygosity in the MEFV gene may not be classified as classical FMF.

European journal of internal medicine
2025

A Rare Cause of Childhood Nephrotic Syndrome: AA Amyloidosis in Epidermolysis Bullosa.

Cureus
2025

Polymorphisms in SAA alter intrarenal amyloid distribution of AA amyloidosis in cats.

Scientific reports
2025

Transient elastography measurements of the liver and transplanted kidney in patients with AA amyloidosis: a cross-sectional comparative study.

Rheumatology international
2025

AA amyloidosis with multiorgan involvement secondary to familial Mediterranean fever (FMF): dreadful consequences of delayed diagnosis and management.

BMJ case reports
2025

Efficacy and safety of treatments in familial Mediterranean fever and its complications: a systematic review informing the EULAR/PReS recommendations for familial Mediterranean fever.

Annals of the rheumatic diseases
2025

[Frequency and clinical and laboratory features of ankylosing spondylitis with secondary AA-amyloidosis].

Terapevticheskii arkhiv
2025

A comprehensive evaluation of cardiac amyloidosis epidemiology and diagnostics in French Guiana.

PloS one
2025

AA amyloidosis in inflammatory joint diseases: A systematic review.

Seminars in arthritis and rheumatism
2025

Surveillance of amyloid A deposits in commercial beef liver and assessment of food hygiene risks.

The Journal of veterinary medical science
2025

Epidemiology and clinical presentation of kidney amyloidosis have changed over the past three decades: a nationwide population-based study.

BMC nephrology
2025

Amyloidosis in Human Inborn Errors of Immunity Predicts Poor Prognosis.

Journal of clinical immunology
2025

EULAR/PReS endorsed recommendations for the management of familial Mediterranean fever (FMF): 2024 update.

Annals of the rheumatic diseases
2025

Mannheimia granulomatis-Infected Pyogranulomatous Oral Lesions with Concurrent Amyloidosis in Roe Deer (Capreolus capreolus) in France.

Journal of wildlife diseases
2025

Use of Biologic Therapy in AA Amyloidosis Patients Undergoing Dialysis-A Systematic Literature Review.

Hemodialysis international. International Symposium on Home Hemodialysis
2025

Digital clubbing, an unfamiliar feature of cryopyrin-associated periodic syndrome: A cohort study.

Journal of the American Academy of Dermatology
2025

Systemic amyloid A amyloidosis secondary to chronic enteritis in a captive little owl (Athenenoctua).

Journal of comparative pathology
2025

Concurrent extramedullary hematopoiesis and AA amyloidosis in the kidney.

Nefrologia
2025

Canakinumab Treatment in Familial Mediterranean Fever Patients: With/Without Colchicine.

International journal of rheumatic diseases
2025

The evolving spectrum of kidney amyloidosis: advances in diagnosis, typing and treatment.

Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
2025

Systemic AA amyloidosis with amyloid deposition in the peritoneum at the time of initiating peritoneal dialysis.

CEN case reports
2025

Serum Amyloid A Binding to Glycosaminoglycans is Synergistic with Amyloid Formation: Therapeutic Targeting in the Inflammation-linked Amyloidosis.

Journal of molecular biology
2025

Waldenström's Macroglobulinemia/Lymphoplasmacytic Lymphoma Developing Renal AA Amyloidosis: A Case Report and Literature Review.

Internal medicine (Tokyo, Japan)
2025

Renal outcomes and survival in amyloidosis associated with familial Mediterranean fever: A longitudinal study.

Seminars in arthritis and rheumatism
2025

New trends in AA amyloidosis with renal involvement: a single-center experience.

Jornal brasileiro de nefrologia
2025

Hepatic Amyloidosis Manifesting as Budd-Chiari Syndrome: An Unusual Presentation.

Cureus
2025

Characteristics and outcomes of adult patients with familial Mediterranean fever: Comparison of patients with one versus two pathogenic exon 10 MEFV mutations.

Joint bone spine
2025

The question of strains in AA amyloidosis.

Scientific reports
2025

Identification and characterization of spontaneous AA amyloidosis in CD-1 mice used in toxicity studies: implications of SAA1 and SAA2 copy number variations.

Journal of toxicologic pathology
2025

Liver Disease Complicating Familial Mediterranean Fever: A Study on 66 Patients Out of 533 Adult From the JIR Cohort.

Liver international : official journal of the International Association for the Study of the Liver
2025

Performance of serum IL-18 levels for the follow-up of patients with familial Mediterranean fever.

The journal of allergy and clinical immunology. In practice
2024

Amyloid light chain and amyloid A protein, two types of gastrointestinal amyloidosis.

Revista espanola de enfermedades digestivas
2024

Inflammatory biomarker analysis confirms reduced disease severity in heterozygous patients with familial Mediterranean fever.

RMD open
2024

Secondary Amyloidosis Treated with Tocilizumab as a Complication of Temporal Arteritis.

European journal of case reports in internal medicine
2025

Co-occurrence of Primary Tracheal Diffuse Large B-Cell Lymphoma and Pulmonary AA Amyloidosis: A Unique Case Report.

Internal medicine (Tokyo, Japan)
2024

Renal colocalisation of Rosai-Dorfman-Destombes disease and secondary AA amyloidosis successfully treated with lenalidomide and dexamethasone.

The Lancet. Haematology
2024

Real-Time 3D Imaging and Inhibition Analysis of Human Serum Amyloid A Aggregations Using Quantum Dots.

International journal of molecular sciences
2025

AA amyloidosis in vertebrates: epidemiology, pathology and molecular aspects.

Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
2024

[Clinical aspects of systemic amyloidosis in 2024].

Annales de pathologie
2024

Histopathological and Immunohistochemical Characteristics of Different Types of Cardiac Amyloidosis.

International journal of molecular sciences
2024

Urinary Chemokines CXCL9 and CXCL10 Are Non-Invasive Biomarkers of Kidney Transplant Rejection.

Annals of transplantation
2024

Cases report: Unexpected arterial thromboembolic events without cardiac involvement in two AA amyloidosis patients.

International journal of rheumatic diseases
2024

Systemic amyloidosis: an aggressive evolution in a patient with relapsing polychondritis and monoclonal gammopathy of undetermined significance (MGUS) undergoing peritoneal dialysis.

Autopsy &amp; case reports
2024

Systemic juvenile idiopathic arthritis and adult-onset Still's disease are the same disease: evidence from systematic reviews and meta-analyses informing the 2023 EULAR/PReS recommendations for the diagnosis and management of Still's disease.

Annals of the rheumatic diseases
2024

Diagnostic delays in familial Mediterranean fever: a Juvenile Inflammatory Rheumatism (JIR) cohort study.

Rheumatology international
2024

Uncovering the knowledge about systemic amyloidosis relevant to the rheumatologists.

Advances in rheumatology (London, England)
2024

HGA Triggers SAA Aggregation and Accelerates Fibril Formation in the C20/A4 Alkaptonuria Cell Model.

Cells
2024

Pregnancy occurring in AA amyloidosis: a series of 27 patients including 3 new French cases.

Journal of nephrology
2024

Case 28-2024: A 75-Year-Old Woman with Edema, Arthritis, and Proteinuria.

The New England journal of medicine
2024

Heterozygous MEFV Mutation Leading to Renal Failure: A Case Study.

Global pediatric health
2024

AA amyloidosis: An uncommon case presenting with a polyneuropathy.

Journal of the neurological sciences
2024

Implication of the LRR Domain in the Regulation and Activation of the NLRP3 Inflammasome.

Cells
2025

Successful treatment of aggressive AA amyloidosis with tocilizumab in a patient with polymyalgia rheumatica.

Modern rheumatology case reports
2024

Key charged residues influence the amyloidogenic propensity of the helix-1 region of serum amyloid A.

Biochimica et biophysica acta. General subjects
2024

Non-Immunoglobulin Amyloidosis-Mediated Kidney Disease: Emerging Understanding of Underdiagnosed Entities.

Advances in kidney disease and health
2024

"Cruising together"-ASC specks and SAA, a perfect match in chronic inflammation.

EMBO molecular medicine
2024

The ASC inflammasome adapter governs SAA-derived protein aggregation in inflammatory amyloidosis.

EMBO molecular medicine
2024

Authentic hSAA related with AA amyloidosis: New method of purification, folding and amyloid polymorphism.

Biophysical chemistry
2024

Detecting Amyloid Goiter With 99mTc-PYP SPECT/CT in the Setting of Ankylosing Spondylitis: An Uncommon Involvement of AA Amyloidosis.

Clinical nuclear medicine
2024

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

Biomedical physics &amp; engineering express
2025

Could tocilizumab be used in familial Mediterranean fever? A systematic review.

Rheumatology (Oxford, England)
2024

Kidney Amyloidosis: Updates on Pathogenesis and Therapeutic Frontiers.

American journal of nephrology
2024

Renal alterations in cats (Felis catus) housed in shelters and affected by systemic AA-amyloidosis: Clinicopathological data, histopathology, and ultrastructural features.

Veterinary pathology
2024

Thromboembolic and bleeding risk in cardiac amyloidosis.

Journal of thrombosis and haemostasis : JTH
2025

Late initiation of anakinra can induce complete renal response in renal AA amyloidosis secondary to Familial Mediterranean Fever.

Journal of nephrology
2024

Deactivation of the Unfolded Protein Response Aggravated Renal AA Amyloidosis in HSF1 Deficiency Mice.

Molecular and cellular biology
2024

Advancing Care for AA Amyloidosis with Biomarker-Based Staging.

Journal of the American Society of Nephrology : JASN
2024

Coronavirus Disease 2019 Outcomes in Amyloid A Protein Amyloidosis Secondary to Rheumatic Conditions and Signs of Post- Coronavirus Disease 2019 Proteinuria Progression.

European journal of rheumatology
2024

Cardiac Amyloidosis: A Contemporary Review of Medical and Surgical Therapy.

Current cardiology reviews
2024

bDMARD can prevent the progression of AA amyloidosis to end-stage renal disease.

Annals of the rheumatic diseases
2024

Renal manifestations in adult-onset Still's disease: a systematic review.

Rheumatology international
2024

Historic characteristics and mortality of patients in the Swiss Amyloidosis Registry.

Swiss medical weekly
2024

Preclinical evaluation of Tc-99m p5+14 peptide for SPECT detection of cardiac amyloidosis.

PloS one
2024

AA Amyloidosis: A Contemporary View.

Current rheumatology reports
2024

Systemic Amyloid A Protein Amyloidosis With Gastrointestinal Involvement.

ACG case reports journal
2024

A rare clinical case of systemic AA amyloidosis with cardiac involvement complicating ankylosing spondylitis: a case report.

The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of Cardiology
2024

Functional diversity of NLRP3 gain-of-function mutants associated with CAPS autoinflammation.

The Journal of experimental medicine
2024

A case of AA amyloidosis complicated by proliferating pilomatricoma and a review of the literature.

The Journal of dermatology
2024

Diagnosis traps for patients with acquired NLRP3 mutation.

European journal of internal medicine
2024

Development and Validation of Staging Systems for AA Amyloidosis.

Journal of the American Society of Nephrology : JASN
2024

Waldenstrom macroglobulinaemia with AA amyloidosis reveals a B-cell-restricted NLRP2 variant.

British journal of haematology
2024

Predicting genetic risk factors for AA amyloidosis in Algerian patients with familial Mediterranean fever.

Molecular genetics and genomics : MGG
2024

Elevated expression of interleukin-6 (IL-6) and serum amyloid A (SAA) in the skin and the serum of recessive dystrophic epidermolysis bullosa: Skin as a possible source of IL-6 through Toll-like receptor ligands and SAA.

Experimental dermatology
2024

Isolated AL Amyloidosis of the Colon: A Rare Presentation.

ACG case reports journal
2024

AA amyloidosis With Ig-Dominant Staining and Diagnostically Unusual Features.

Kidney international reports
2024

Tocilizumab (anti-IL-6) treatment for AA renal amyloidosis in a patient with advanced chronic kidney disease, a case report.

Journal of nephrology
2023

Acute Tubulointerstitial Nephritis and Secondary Renal Amyloidosis: A Rare Complication of Atezolizumab.

Cureus
2024

A comparative urinary proteomic and metabolomic analysis between renal aa amyloidosis and membranous nephropathy with clinicopathologic correlations.

Journal of proteomics
2023

Single Nucleotide Polymorphisms Associated with AA-Amyloidosis in Siamese and Oriental Shorthair Cats.

Genes
2024

Symptomatic SARS-CoV2 infection associated with high mortality in AA amyloidosis.

Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
2023

[Renal AA amyloidosis revealing extramedullary plasmocytoma].

Nephrologie &amp; therapeutique
2024

A rare cause of AA amyloidosis: Glomus tumor and treatment with anakinra-Case report and literature review.

International journal of rheumatic diseases
2024

Renal amyloid-A amyloidosis in cats: Characterization of proteinuria and biomarker discovery, and associations with kidney histology.

Journal of veterinary internal medicine
2023

Systemic AA Amyloidosis Secondary to Metastatic Renal Cell Carcinoma in a Hemodialysis Patient with Intractable Diarrhea.

Case reports in gastroenterology
2023

Histological evaluation of the distribution of systemic AA-amyloidosis in nine domestic shorthair cats.

PloS one
2023

French protocol for the diagnosis and management of familial Mediterranean fever.

La Revue de medecine interne
2023

Kidney Biopsy Corner: Amyloidosis.

Glomerular diseases
2023

Predictive Clinical and Biological Criteria for Gene Panel Positivity in Suspected Inherited Autoinflammatory Diseases: Insights from a Case-Control Study.

Genes
2023

Pathomorphological Features of the Novel Coronavirus Disease in Patients with Systemic Amyloidosis.

Biomedicines
2024

Unicentric Castleman's disease presenting as amyloid A cardiac amyloidosis: a case report.

Annals of hematology
2023

Behçet's syndrome: one year in review 2023.

Clinical and experimental rheumatology
2023

Pathological Characterization of Spontaneous AA Amyloidosis in Microminipigs.

Toxicologic pathology
2024

AA amyloidosis in a father and daughter as complication of PSTPIP1-associated myeloid-related proteinemia inflammatory (PAMI) syndrome.

Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
2024

A rare case of hydatid cyst-associated AA amyloidosis and literature review of hydatid cyst-associated nephropathy.

Infectious diseases (London, England)
2023

Development and characterization of a prototypic pan-amyloid clearing agent - a novel murine peptide-immunoglobulin fusion.

Frontiers in immunology
2023

Hyperspectral Raman Imaging for Automated Recognition of Human Renal Amyloid.

The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society
2023

Nephrotic Syndrome in a Retroviral Disease Due to AA Amyloidosis: A Rare Presentation.

Cureus
2023

Investigation of serum amyloid a within animal species focusing on the 1-25 amino acid region.

The veterinary quarterly
2024

Successful Pregnancy Outcome in a Patient with Juvenile Idiopathic Arthritis in Adulthood, Amyloid A Amyloidosis, and Chronic Kidney Disease Using Tocilizumab with Strict Blood Pressure Control.

Internal medicine (Tokyo, Japan)
2024

Clinical presentation and genetic variants in patients with autoinflammatory diseases: results from the German GARROD registry.

Rheumatology international
2024

Kidney Transplantation in Patients With AA Amyloidosis: Outcomes in a French Multicenter Cohort.

American journal of kidney diseases : the official journal of the National Kidney Foundation
2024

Characteristics and course of patients with AA amyloidosis: single centre experience with 174 patients from Turkey.

Rheumatology (Oxford, England)
2023

Therapeutic effects of biological treatments on AA amyloidosis associated with inflammatory bowel disease: a case report and literature review.

European journal of gastroenterology &amp; hepatology
2023

The spectrum of pulmonary amyloidosis.

Respiratory medicine
2023

[Autoinflammatory syndromes].

Zeitschrift fur Rheumatologie
2023

Intestinal Reactive Amyloid A (AA) Amyloidosis in a Patient With Multiple Myeloma: A Case Report and Literature Review.

Cureus
2023

Stress-mediated aggregation of disease-associated proteins in amyloid bodies.

Scientific reports
2023

Nakajo-Nishimura Syndrome: The First African Case.

Mediterranean journal of rheumatology
2023

Vector autoregression: Useful in rare diseases?-Predicting organ response patterns in a rare case of secondary AA amyloidosis.

PloS one
2023

Chemical design of radioiodinated probes with a metabolizable linkage for target-selective imaging of systemic amyloidosis.

Bioorganic &amp; medicinal chemistry
2023

Kidney dysfunction due to AA amyloidosis in a morbidly obese female.

Clinical nephrology. Case studies
2023

Secondary amyloidosis with concomitant gastric GIST and gastric adenocarcinoma - A case report.

Indian journal of pathology &amp; microbiology
2023

COVID-19 Infection and Vaccination and Its Relation to Amyloidosis: What Do We Know Currently?

Vaccines
2023

Pathogenic variants in the NLRP3 LRR domain at position 861 are responsible for a boost-dependent atypical CAPS phenotype.

The Journal of allergy and clinical immunology
2023

Kidney Involvement in Autoinflammatory Diseases.

Kidney diseases (Basel, Switzerland)
2023

AA-type amyloidosis associated with lymphoma: a study of 19 cases including 5 new French cases and a systematic literature review.

Leukemia &amp; lymphoma
2023

Gastrointestinal AA Amyloidosis following Recurrent SARS-CoV-2 Infection: A Case Report.

Yonsei medical journal
2023

TNFRSF1A-pR92Q variant identifies a subset of patients more similar to systemic undifferentiated recurrent fever than TNF receptor-associated periodic syndrome.

Clinical and experimental rheumatology
2023

Human lysozyme inhibits the fibrillation of serum amyloid a protein from systemic AA amyloidosis.

Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
2024

Systemic Amyloid A Amyloidosis Secondary to Xanthogranulomatous Pyelonephritis.

Internal medicine (Tokyo, Japan)
2024

Gut microbiota alterations are associated with phenotype and genotype in familial Mediterranean fever.

Rheumatology (Oxford, England)
2023

Comparing COVID-19 disease severity in patients with rheumatic and inflammatory diseases between the first and the subsequent waves.

Joint bone spine
2023

Gout With Associated Cutaneous AA Amyloidosis: A Case Report and Review of the Literature.

British journal of biomedical science
2023

AA amyloidosis of unknown aetiology: response to treatment with interleukin-1 inhibitors.

Clinical kidney journal
2023

Renal involvement in monogenic autoinflammatory diseases: A narrative review.

Nephrology (Carlton, Vic.)
2023

Gastroparesis: an under-recognised manifestation of systemic amyloidosis.

BMJ case reports
2023

Influences of amino-terminal modifications on amyloid fibril formation of human serum amyloid A.

Archives of biochemistry and biophysics
2023

Renal Amyloid-Associated (AA) Amyloidosis in a Sickle Cell Patient: A Case Report and Literature Review.

Cureus
2023

Post COVID-19 AA amyloidosis of the kidneys with rapidly progressive renal failure.

Prion
2023

Case report: Rapid development of amyloid A amyloidosis in temporal arteritis with SAA1.3 allele; An unusual case of intestinal amyloidosis secondary to temporal arteritis.

Frontiers in immunology
2023

AA-amyloidosis in cats (Felis catus) housed in shelters.

PloS one
2023

[A case of systemic amyloidosis presenting with gastric emphysema].

Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterology
2023

Renal involvement, presence of amyloidosis, and genotype-phenotype relationship in pediatric patients with Familial Mediterranean fever: a single center study.

European journal of pediatrics
2023

French practical guidelines for the diagnosis and management of AA amyloidosis.

La Revue de medecine interne
2023

A novel TNFRSF1A mutation associated with TNF-receptor-associated periodic syndrome and its metabolic signature.

Rheumatology (Oxford, England)
2023

Renal involvement in familial Mediterranean fever in an Algerian population.

Clinical nephrology
2023

A Comprehensive Multidisciplinary Diagnostic Algorithm for the Early and Efficient Detection of Amyloidosis.

Clinical lymphoma, myeloma &amp; leukemia
Ver todos os 610 no EuropePMC

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Canakinumab treatment in patients with colchicine-resistant familial mediterranean fever: a multicenter observational study.
    Turkish journal of medical sciences· 2026· PMID 41816744mais citado
  2. A case of AA amyloidosis with biliary cystadenoma and cervical thymoma in a striped skunk (Mephitis mephitis).
    The Journal of veterinary medical science· 2026· PMID 41260700mais citado
  3. AA amyloidosis with clinically overt cardiac involvement confirmed by amyloid A deposition in aortic valve fibroelastoma.
    European heart journal· 2026· PMID 41312856mais citado
  4. AA amyloidosis in inflammatory joint diseases in the era of biological therapies: prevalence, manifestations, management and evolution.
    Joint bone spine· 2026· PMID 41864492mais citado
  5. Case Report: Anti-TNF-&#x3b1; therapy-associated destructive thyroiditis and unmasking of latent amyloid A amyloidosis in rheumatoid arthritis.
    Frontiers in immunology· 2026· PMID 41822475mais citado
  6. Oral AA amyloidosis with granulomatous cheilitis: a case report and literature review.
    Clin Rheumatol· 2026· PMID 41979812recente
  7. Dual Diagnosis of Thyroid Amyloidosis and Lipomatosis: An Uncommon Concurrence.
    Cureus· 2026· PMID 41913884recente
  8. Chronic Brucella infection associated with amyloid light chain cardiac amyloidosis: a case report.
    Front Cardiovasc Med· 2026· PMID 41908037recente
  9. Protective Effect of Paeoniae Radix Alba Carbonisata on Hepatic Amyloidosis by Regulating Calcium Homeostasis.
    Int J Mol Sci· 2026· PMID 41898444recente
  10. A missense mutation in the SAA1 protein causing hereditary amyloid A amyloidosis.
    Kidney Int· 2026· PMID 41887599recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:85445(Orphanet)
  2. MONDO:0019439(MONDO)
  3. GARD:10560(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Q2844591(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Amiloidose AA
Compêndio · Raras BR

Amiloidose AA

ORPHA:85445 · MONDO:0019439
🇧🇷 Brasil SUS
CEAF
1ATafamidis
Geral
Prevalência
Unknown
Herança
Not applicable
CID-10
E85.3 · Amiloidose sistêmica secundária
CID-11
Ensaios
1 ativos
Início
Adolescent, Adult, Childhood
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0221014
EuropePMC
Wikidata
Papers 10a
Evidência
🥉 Relato de caso
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