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AA amyloidosis in inflammatory joint diseases: A systematic review

Journal: Seminars in Arthritis and Rheumatism

First author: Binta Savadogo

Country of study: France — international systematic literature review

CEREMAIA Tenon author: Sophie Georgin-Lavialle

Reference: Savadogo B, Fahed H, Sellam J, Georgin-Lavialle S, Fautrel B, Mitrovic S. AA amyloidosis in inflammatory joint diseases: A systematic review. Seminars in Arthritis and Rheumatism. 2025;74:152762.


AA amyloidosis in inflammatory joint diseases: A systematic review

5 key points

  1. AA amyloidosis can occur when inflammation remains high for a long time in some inflammatory joint diseases.

  2. The kidneys are often the main organs affected, which may cause protein loss in the urine and reduced kidney function.

  3. The review suggests that AA amyloidosis appears to have become less frequent in some diseases since the introduction of more effective treatments.

  4. Treatments that control inflammation well, especially biologic therapies, may improve markers such as serum amyloid A and kidney function.

  5. The authors highlight the lack of recent data and the need to better measure the current burden of this complication.


Introduction

AA amyloidosis is a complication of chronic inflammation. It occurs when an abnormal protein called amyloid builds up in organs, especially the kidneys, but sometimes also the digestive tract, liver or heart. It may complicate inflammatory joint diseases such as rheumatoid arthritis, spondyloarthritis, psoriatic arthritis or juvenile idiopathic arthritis. Because modern anti-inflammatory treatments have greatly improved, the authors wanted to know whether this complication has become less common.


Methods

The authors performed a systematic literature review. They searched several medical databases up to October 2024 for studies describing patients with biopsy-confirmed AA amyloidosis related to inflammatory joint disease. Included studies had to report at least 10 patients and provide information on frequency, mortality or evolution under treatment.


Results

In total, 33 studies were included, representing almost 14,000 patients with inflammatory joint diseases. The data were very heterogeneous, and most studies were old, published before 2010. In rheumatoid arthritis, reported AA amyloidosis prevalence ranged from 16.7% to 25.2% before 2010 and appeared to decrease to 0.7% after 2010. In ankylosing spondylitis, reported prevalence ranged from 6.1% to 8.5% before 2010 and from 1.1% to 1.3% after 2010. Immunomodulating treatments, especially biologic therapies, seemed to improve some markers of AA amyloidosis.


Discussion

These findings suggest that better control of inflammation may reduce the risk of AA amyloidosis or improve its course. However, the authors remain cautious because available studies are old, very different from one another and do not allow firm conclusions. The article also emphasizes the importance of monitoring chronic inflammation and kidney function in patients with inflammatory joint diseases.


Conclusion

AA amyloidosis now appears to be less frequent than in the past in some inflammatory joint diseases, probably thanks to more effective treatments. Recent, larger and better standardized studies are needed to better understand the current risk and improve prevention.


 
 
 

French protocol for the diagnosis and management of recurrent pericarditis Journal: La Revue de médecine interne

Updated: 2 days ago

First author: Yvan Jamilloux

Country of study: France

CEREMAIA Tenon author: Sophie Georgin-Lavialle

Reference: Jamilloux Y, André M, Maillard H, Baudet M, Beauvais F, Boursier G, Buschiazzo A, Donal E, Flecher E, Georgin-Lavialle S, Gerfaud-Valentin M, Kone-Paut I, Labombarda F, Piriou N, Saadoun D, Aouba A, and collaborators. French protocol for the diagnosis and management of recurrent pericarditis / Protocole national de diagnostic et de soins – Péricardites récidivantes. La Revue de médecine interne. 2026;47:127–146.

DOI: https://doi.org/10.1016/j.revmed.2026.02.002


French protocol for the diagnosis and management of recurrent pericarditis Journal: La Revue de médecine interne

5 key points

  • Recurrent pericarditis means repeated episodes of inflammation of the pericardium, separated by a symptom-free period of at least 4 to 6 weeks.

  • Diagnosis is based on symptoms, clinical examination, electrocardiogram, echocardiography, sometimes cardiac MRI, and inflammation markers such as CRP.

  • Colchicine is the cornerstone treatment to reduce the risk of recurrence and is often combined with anti-inflammatory drugs during flares.

  • Corticosteroids should be avoided as much as possible, except in specific situations, because they may promote treatment dependence and recurrences.

  • In severe or resistant forms, treatments targeting interleukin-1, such as anakinra, may be discussed with expert centres.


Introduction

Pericarditis is inflammation of the pericardium, the thin sac surrounding the heart. When it comes back several times after a first episode, it is called recurrent pericarditis. This condition can cause significant chest pain, anxiety, emergency visits and difficulties in family, social or professional life.


Methods

This article is a French national protocol for diagnosis and care. It brings together expert recommendations to help physicians diagnose, treat and follow people with recurrent pericarditis, including adults, children and specific situations such as pregnancy.


Results

Diagnosis relies on several elements: typical chest pain, a pericardial rub heard during examination, electrocardiogram changes, fluid around the heart on echocardiography, and blood signs of inflammation, especially CRP. Cardiac MRI can help in difficult cases. Most cases are called idiopathic, often presumed to follow a viral infection, but some may be linked to autoimmune, autoinflammatory or infectious diseases, or to inflammation after cardiac surgery or procedures. Serious complications, such as cardiac tamponade or constrictive pericarditis, are rare but must be recognized quickly.


Discussion

Treatment aims to control inflammation, relieve pain and, above all, prevent recurrences. Colchicine is central and often needs to be continued for several months. During flares, anti-inflammatory drugs or aspirin may be used together with colchicine. Corticosteroids are no longer recommended except in specific cases. In severe, resistant or corticosteroid-dependent forms, interleukin-1 inhibitors, particularly anakinra, can improve symptoms and quality of life. Follow-up should also include rest, gradual return to physical activity, patient education and attention to psychological impact.


Conclusion

Recurrent pericarditis requires coordinated care involving cardiologists, internal medicine specialists, general practitioners and expert centres. Accurate diagnosis, appropriate treatment and very gradual treatment withdrawal can reduce relapses and improve quality of life.

 
 
 

VEXAS syndrome

Updated: 2 days ago

Journal: Nature Reviews Disease Primers

First author: David B. Beck

Country of study: international article — United States, France, Japan and Italy

CEREMAIA Tenon author: Sophie Georgin-Lavialle

Reference: Beck DB, Georgin-Lavialle S, Kirino Y, Patel BA, Ferrari S. VEXAS syndrome. Nature Reviews Disease Primers. 2026;12:19.

DOI: https://doi.org/10.1038/s41572-026-00695-w


VEXAS syndrome

5 key points:

  • VEXAS syndrome is a rare adult-onset inflammatory disease, discovered in 2020, caused by an acquired mutation in the UBA1 gene in blood cells.

  • It mainly affects men over the age of 50, but it can also occur in women, especially in the context of X chromosome abnormalities.

  • Common signs include corticosteroid-dependent inflammation, skin lesions, cartilage and lung involvement, blood abnormalities and sometimes myelodysplastic features.

  • Diagnosis is based on the combination of symptoms, laboratory abnormalities — especially macrocytic anaemia and inflammation — and confirmation by genetic testing for a UBA1 mutation.

  • Treatments aim to control inflammation and/or the abnormal blood cell clone, but management remains complex and must be tailored to each patient.


Introduction:

VEXAS syndrome is a recently identified autoinflammatory disease. Its name stands for vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic. It is caused by an acquired mutation in the UBA1 gene in certain blood stem cells. This mutation disrupts immune cell function and leads to significant chronic inflammation.


Methods:

This article is a general review, called a Primer, that brings together recent knowledge about VEXAS syndrome, including its frequency, biological mechanisms, clinical signs, diagnosis, complications, treatments and impact on quality of life.


Results:

VEXAS mainly affects men over the age of 50. Symptoms may include fever, fatigue, weight loss, skin involvement, cartilage inflammation — for example of the ears — lung involvement, joint pain, blood abnormalities and an increased risk of blood clots. Many patients have anaemia with enlarged red blood cells, sometimes low platelet counts, and bone marrow abnormalities. Diagnosis is confirmed by genetic testing for a UBA1 mutation.


Discussion:

The disease is often difficult to recognize because it can resemble other inflammatory, dermatological, rheumatological or haematological diseases. Corticosteroids often improve symptoms rapidly, but long-term corticosteroid dependence is common. Other treatments may be used, including JAK inhibitors, therapies targeting specific cytokines, azacitidine or, in selected cases, stem cell transplantation.


Conclusion:

VEXAS has changed the understanding of some adult inflammatory diseases by showing the link between inflammation, acquired genetics and blood disorders. Earlier diagnosis and prospective studies are needed to improve patient care and quality of life.


 
 
 
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