IgG4-related disease and other fibro-inflammatory conditions - Nature Reviews Rheumatology IgG4-related disease is a fibro-inflammatory disorder with a complex pathogenesis. Herein the authors review the latest developments in IgG4-related disease clinical phenotyping, pathophysiology and m...

IgG4-related disease and other fibro-inflammatory conditions

www.nature.com/articles/s41...

Mechanisms of prothrombotic cell activation by anti-β2GPI IgG. The diagram highlights the receptors most commonly linked to cell activation by β2GPI/anti-β2GPI IgG surface complexes, as well as some effectors known to be downstream of that activation. β2GPI: beta-2 glycoprotein I, LRP: low-density lipoprotein receptor-related protein, ROS: reactive oxygen species, TF: tissue factor, TLR: Toll-like receptor, and VWF, von Willebrand factor

Mechanisms of prothrombotic cell activation by anti-β2GPI IgG. The diagram highlights the receptors most commonly linked to cell activation by β2GPI/anti-β2GPI IgG surface complexes, as well as some effectors known to be downstream of that activation

#Rheumsky #Immunosky

Non-Criteria Obstetric Antiphospholipid Syndrome: Myth or Reality?

NC-OAPS: heterogeneous condition and ambiguously described in literature

#APS #OAPS #Pregnancy #autoimmune

Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis - Nature Reviews Rheumatology This article presents the first Evidence-Based Guideline dedicated specifically to the diagnosis and management of eosinophilic granulomatosis with polyangiitis. The 16 statements and five overarching...

Today is International Vasculitis Day!

Take a look at some of our articles on vasculitis:

Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis bit.ly/4kjASRj

The new look of classification criteria for systemic vasculitis bit.ly/3GXJLlc

Behçet syndrome

🩸systemic #vasculitis, affects vessels of all calibers
🩺 venous #thrombosis with arterial #aneurysms, inflammatory parenchymal brain involvement, posterior #uveitis with retinal vasculitis, bipolar #ulcers and erythema nodosum
⁉️ controversy: pathergy test or HLA-B*51 in diagnosis

🧬Monogenic vasculitis: DADA2, HA20, SAVI, COPA, LAVLI, VEXAS—rare but real
🧠DADA2: Livedo racemosa, strokes, skin necrosis
🧠 HA20: Behçet-like—ulcers, uveitis, venous thrombosis.
🧠SAVI: Skin ulcers, nasal perforation
🧠COPA: Skin/intestinal vasculitis, ILD
🧠VEXAS

Arterial and venous thrombosis in systemic and monogenic vasculitis - Nature Reviews Rheumatology Systemic vasculitis, including monogenic forms, is associated with an increased risk of arterial and venous thromboembolic events (AVTEs). This Review highlights the latest advances in the understandi...

Arterial and venous thrombosis in systemic and monogenic vasculitis

⚠️ Be aware for early diagnosis‼️

🧬 Genetic testing vital—variable penetrance! Early strokes hint at diagnosis.

#vasculitis #thrombosis #monogenic #stroke #skin

www.nature.com/articles/s41...

🚨2024 American College of Rheumatology (ACR)
Guideline for the Screening, Treatment, and
Management of Lupus Nephritis

#Lupus #Nephritis #Guideline #Treatment #SLE

assets.contentstack.io/v3/assets/bl...

Consensus nomenclature and abbreviation for anti-synthetase syndrome: an IMACS project ABSTRACTObjectives. The lack of uniform terminology and abbreviations for anti-synthetase syndrome has led to significant challenges in research and clinic

Consensus nomenclature and abbreviation for anti-synthetase syndrome: an IMACS project

🚩lack of uniform terminology and abbreviations = challenges in research and clinical practice
🏳️anti-synthetase syndrome and ASyS should be adopted as the standard nomenclature
academic.oup.com/rheumatology...

Remnant cholesterol predicts risk
of recurrent thrombosis beyond LDL‑cholesterol
in patients with antiphospholipid syndrome

🟠RC ➡️ increased risk of trombosis
🟠RC marker for thrombotic risk❓
🟠potential target
for therapeutic intervention.

bmcmedicine.biomedcentral.com/counter/pdf/...

EULAR recommendations for use of antirheumatic
drugs in reproduction, pregnancy, and lactation: 2024
update

🤰🏽👩🏽‍🍼

ard.eular.org/action/showP...

Consensus Recommendations for the Management of Neurosarcoidosis | Neurology Clinical Practice Background and ObjectivesNeurosarcoidosis poses a diagnostic and management challenge due to its rarity, phenotypic variability, and lack of randomized controlled studies to guide treatment selection....

Consensus Recommendations for the Management of Neurosarcoidosis

www.neurology.org/doi/10.1212/...

Recommendations for the use of DMARDs in pregnancy and reproductive health for patients with rheumatic disease: A scoping review Objective Autoimmune rheumatic diseases commonly affect individuals of childbearing age, with historically increased adverse pregnancy outcomes in this group. The advent of disease-modifying anti-rh...

Recommendations for the use of DMARDs in pregnancy and reproductive health for patients with rheumatic disease: A scoping review

acrjournals.onlinelibrary.wiley.com/doi/10.1002/...

To B or Not to B — the Role of B-Cell Depletion in Lupus Nephritis | NEJM B cells play a key role in the pathogenesis of lupus nephritis, yet in the Lupus Nephritis Assessment with Rituximab (LUNAR) trial, rituximab therapy did not improve clinical outcomes after 1 year ...

To B or Not to B — the Role of B-Cell Depletion in Lupus Nephritis

www.nejm.org/doi/full/10....

IL-10 in Systemic Lupus Erythematosus: Balancing Immunoregulation and Autoimmunity

🔑involved in inflammatory and immune processes in SLE.
🔹IL-10 did not correlate with disease activity, organ in-
volvement, or treatment response.

www.mdpi.com/1422-0067/26...

A proposed clinical tool to identify high-risk patients for monogenic lupus: a pilot study Paediatric Rheumatology; Authors: S.M. Al-Mayouf, D. Hadef, N. Aljaberi, N. Movahedi, A. Aleed, A. Almutairi, A. Asiri, S. Volpi, M. Gattorno, C.-Y. Wu, H. Tamim, A. Al-Saleem

A proposed clinical tool to identify high-risk patients for #monogenic #lupus: a pilot study

💡<5 years
💡cosanguinity
💡Family #SLE
💡elevated ANA
💡pancytopenia
💡cutaneus lesions
💡Low C1q
💡Recurrent infections
💡Failure to thrive

www.clinexprheumatol.org/abstract.asp...

Therapeutic approaches for
#SAPHO syndrome from the
perspective of pathogenesis:
a review of the literature

🔸Autoinflammatory
🔸cutaneous & osteoarticular
🔹ATB, tonsillectomy
🔹DMARDs
🔹Bisphosphonates

www.frontiersin.org/journals/imm...

British Association of Dermatologists and British Society for Rheumatology living guideline for managing people with Behçets 2024 The overall objective of the guideline is to provide up-to-date, evidence-based recommendations for the management of Behçets. The document aims to offer a

British Association of Dermatologists and British Society for Rheumatology living guideline for managing people with Behçets 2024

👇🏼👀

academic.oup.com/bjd/article/...

Prognostic insights from symptom clustering analysis in systemic lupus erythematosus Objective Systemic lupus erythematosus (SLE), a systemic autoimmune disease with heterogeneous severity, poses a challenge in classifying patients due to its dynamic phenotype. We therefore aimed to i...

Prognostic insights from symptom clustering analysis in systemic lupus erythematosus

1️⃣ Articular/mucocutaneous ➡️better survival
2️⃣Severe phenotype with renal involvement ➡️ poor prognosis
3️⃣Mixed connective tissue disease-like features with anti-U1-RNP antibodies

rmdopen.bmj.com/content/11/2...

Urine Protein Tests in Systemic Lupus Erythematosus:
What Do They Mean?

#SLE #Lupus #LN

www.jrheum.org/content/jrhe...

Autoinflammatory Diseases: A Review Autoinflammatory disease (AID) is a vast spectrum of disorders characterized by recurrent attacks of sterile inflammation. Since the first cloning of the familial Mediterranean fever gene in 1997, the...

Autoinflammatory Diseases: A Revision

www.jrheum.org/content/51/9...

Antiphospholipid syndrome in pregnancy: a comprehensive literature review - BMC Pregnancy and Childbirth Background Antiphospholipid syndrome (APS) is an autoimmune disorder associated with thrombotic events and adverse obstetric outcomes, particularly in its obstetric form (OAPS). Affecting approximatel...

Antiphospholipid syndrome in pregnancy: a comprehensive literature review

🤍

bmcpregnancychildbirth.biomedcentral.com/articles/10....

A Phase 3 Trial of Upadacitinib for Giant-Cell Arteritis | NEJM Giant-cell arteritis is a systemic vasculitis with limited treatment options. The efficacy and safety of upadacitinib — a selective Janus kinase (JAK) inhibitor that blocks the signaling of several...

A Phase 3 Trial of Upadacitinib for Giant-Cell Arteritis

📌In patients with giant-cell arteritis, upadacitinib at a dose of 15 mg — but not 7.5 mg — with a 26-week glucocorticoid taper showed efficacy superior to that of placebo with a 52-week glucocorticoid taper

www.nejm.org/doi/full/10....

Comparative Efficacy of Immunosuppressive Therapies in the Treatment of Diffuse Cutaneous Systemic Sclerosis Objectives The RESOLVE-1 trial of lenabasum in diffuse cutaneous systemic sclerosis (dcSSc) allowed background immunosuppressive therapy (IST) at the discretion of individual investigators, and no s...

Comparative Efficacy of Immunosuppressive Therapies in the Treatment of Diffuse Cutaneous Systemic Sclerosis

🔸MMF in dcSSc and show benefit especially in early-stage disease. 🔸High-risk ab for lung fibrosis ➡️ suitable for MMF treatment.

acrjournals.onlinelibrary.wiley.com/doi/full/10....

Thoracic Manifestations of ANCA-associated Vasculitis: Review of the 2022 American College of Rheumatology–European Alliance of Associations of Rheumatology Classification Criteria

#ANCA #Vasculitis

pubs.rsna.org/doi/10.1148/...

2024 American College of Rheumatology (ACR) Guideline for the Screening, Treatment, and Management of Lupus Nephritis The accepted version of this article was posted prematurely on March 24, 2025. The final version of record will be made fully available at a later date.

2024 American College of Rheumatology (ACR) Guideline for the Screening, Treatment, and Management of Lupus Nephritis

acrjournals.onlinelibrary.wiley.com/doi/10.1002/...

Familial Mediterranean Fever; Recent Advances, Future Prospectives

🤩 Wonderful review of #FMF, it’s a must!

www.mdpi.com/2075-4418/15...

Systemic lupus erythematosus: one year in review 2025

🔸New insights into immune dysregulation
🔸Emerging treatments: Promising therapies
🔸Real-world impact: Anifrolumab and belimumab

www.clinexprheumatol.org/article.asp?...

Pentose phosphate pathway metabolite restores T cell balance in SLE - Nature Reviews Rheumatology Gluconolactone promotes Treg cell function and might have potential for treating skin lesions in systemic lupus erythematosus.

Pentose phosphate pathway metabolite restores T cell balance in SLE

#SLE #LUPUS

www.nature.com/articles/s41...

Low aCL levels are associated with:

📍IgG aCL: higher incidence of pregnancy-related complications
❓Should Screening for aCL in the first trimester be implemented into routine clinical practice?