THERAPEUTIC CHALLENGES IN POLYAUTOIMMUNITY: ANIFROLUMAB FOR LUPUS ERYTHEMATODES IN OVERLAPPING AUTOIMMUNE SYNDROMES
Notice bibliographique
Résumé
PV303 / #378 Case Report Poster Topic: AS24 - SLE-Treatment Introduction Polyautoimmunity is defined as the presence of 2 autoimmune diseases in a single individual, while Multiple Autoimmune Syndrome (MAS) involves 3 or more coexisting autoimmune conditions.[1] Diagnosing specific diseases in patients with multiple autoimmune conditions can be challenging and treatment regimens are often complex. Here we present 2 patients with polyautoimmunity and refractory lupus erythematosus who showed significant symptom improvement after adding anifrolumab, a monoclonal antibody targeting the interferon alpha receptor 1 (IFNAR1), to their treatment regimen. Case Presentation With Investigation Case 1: A 57-year-old woman with a history of multiple sclerosis (MS), treated with glatiramer acetate, presented with recurrent facial and periungual erythema, fatigue, weight loss, fever episodes, and finger pain. Diagnostic workup, including elevated ANA titer of 1:320, positive anti-dsDNA antibodies (42 U/mL), strongly positive anti-MDA5 and anti-RO-52 antibodies, led to an overlap syndrome of amyopathic dermatomyositis and lupus erythematosus. To address her full spectrum of autoimmune diseases, her MS treatment was switched to azathioprine and prednisolone, resulting in improved general condition and reduced facial erythema. However, painful periungual lesions with fissures persisted, greatly impairing her quality of life. Due to fine motor skill loss and significantly elevated Siglec-1 expression (16,347 antigens/monocyte), anifrolumab was added to her treatment regimen. This led to a rapid and marked improvement in the previously resistant periungual lesions, allowing corticosteroid tapering while maintaining MS stability. Case 2: A 60-year-old male patient with a history of psoriasis vulgaris and previous thromboembolic events presented with painful, scarring lesions on the fingers, nose, and scalp. Histology confirmed discoid lupus, and laboratory tests revealed a high ANA titer (1:1280), anti-dsDNA antibodies, and positive antiphospholipid antibodies, resulting in diagnoses of systemic lupus erythematosus (SLE) and antiphospholipid syndrome. Hydroxychloroquine was avoided due to concerns about exacerbating psoriasis. Alternative therapies with methotrexate and mycophenolate mofetil provided limited benefit. Although apremilast effectively controlled psoriasis, lupus lesions continued to progress. The initiation of anifrolumab therapy led to a rapid improvement in cutaneous lupus symptoms within weeks, while psoriasis remained stable and was well-controlled with topical treatments. Phenprocoumon was added for thromboembolic prevention, with no further thromboembolic events reported. Literature Review Anifrolumab is an effective treatment for refractory SLE, particularly cutaneous manifestations. Evidence shows that blocking type I interferon pathways, central in lupus pathology, can effectively reduce inflammatory responses. Clinical trials (eg, TULIP-1 and TULIP-2) have demonstrated anifrolumab’s efficacy in decreasing both systemic and cutaneous disease activity.[2] While the use of anifrolumab in polyautoimmunity and MAS requires further research, its targeted mechanism of action and favorable safety profile already make it a promising option for patients with overlapping connective tissue diseases.[3] Discussion Both cases illustrate the potential of anifrolumab as an effective add-on therapy for targeting refractory cutaneous symptoms in patients with connective tissue diseases. Although anifrolumab is currently approved only for SLE, its positive effects may extend to other connective tissue diseases characterized by elevated interferon alpha activity. The successful and safe use in patients with polyautoimmunity underscores anifrolumab’s suitability for patients with overlapping autoimmune diseases. References: [1.] Matusiewicz A. Int J Rheum Dis 2019;22:386-91. [2.] Morand EF. Lancet Rheumatol 2022;4:282-92. [3.] Shaw KS. J Am Acad Dermatol 2024;91(6):1217-9.
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Comment cette classification a été obtenuedéplier
Prédiction machine sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Le volet Gemma est une étiquette directe du modèle pour chaque travail de la base, lue sur la notice réduite au titre. Le volet Codex est un classifieur appris des 10 348 étiquettes directes de Codex et calibré sur les taux pondérés de l'échantillon; les champs sans appui suffisant ne portent aucun appel Codex. Le mode candidate est l'union des deux volets; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont pas des étiquettes humaines.
Scores du classifieur distillé par catégorie (deux têtes)
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,001 |
| Méta-épidémiologie (sens strict) | 0,001 | 0,000 |
| Méta-épidémiologie (sens large) | 0,001 | 0,000 |
| Bibliométrie | 0,001 | 0,000 |
| Études des sciences et des technologies | 0,001 | 0,001 |
| Communication savante | 0,001 | 0,001 |
| Science ouverte | 0,000 | 0,001 |
| Intégrité de la recherche | 0,002 | 0,002 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,002 | 0,001 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule source (Gemma direct ou Codex distillé), pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».