| Title: |
Lupuslike Manifestations in Myelodysplastic Syndromes and Chronic Myelomonocytic Leukemia |
| Authors: |
Chauffier, Jeanne; Jachiet, Vincent; Battistella, Maxime; Romero, Pierre; Fenaux, Pierre; Zakine, Eve; Zhao, Lin Pierre; Mahévas, Thibault; Bouaziz, Jean-David; Hadjadj, Jerome; Amoura, Zahir; Mathian, Alexis; Breillat, Paul; Hirsch, Pierre; Bourguiba, Rim; De Voeght, Adrien; Grobost, Vincent; Begon, Edouard; Jandus, Peter; Brenaut, Emilie; Laumondais, Victoire; Fain, Olivier; Moguelet, Philippe; Mekinian, Arsene; Chasset, François; EMSED Group; MINHEMON Group |
| Source: |
ISSN: 2168-6068 ; JAMA dermatology, vol. 162, no. 1 (2026) p. 31-40. |
| Publication Year: |
2026 |
| Collection: |
Université de Genève: Archive ouverte UNIGE |
| Subject Terms: |
info:eu-repo/classification/ddc/616; Adult; Aged; 80 and over; Case-Control Studies; Female; Humans; Leukemia; Myelomonocytic; Chronic / complications; Chronic / immunology; Lupus Erythematosus; Cutaneous / diagnosis; Cutaneous / etiology; Cutaneous / immunology; Cutaneous / pathology; Systemic / diagnosis; Systemic / etiology; Systemic / immunology; Systemic / pathology; Male; Middle Aged; Myelodysplastic Syndromes / complications; Myelodysplastic Syndromes / immunology; Retrospective Studies; Skin / pathology |
| Description: |
Importance: Immune-mediated inflammatory diseases are rare but increasingly reported among patients with myelodysplastic syndromes (MDS) or chronic myelomonocytic leukemia (CMML). Systemic lupus erythematosus (LE) and cutaneous LE associated with MDS/CMML have been rarely described, with atypical features and refractory disease. Objective: To provide a comprehensive description of the phenotype and therapeutic responses of LE associated with MDS/CMML and to compare them with idiopathic LE. Design, setting, and participants: This retrospective case-control study included nationwide, multicenter data from January 1975 to January 2023. Patients with MDS/CMML who either fulfilled classification criteria for systemic LE or had skin lesions diagnosed as cutaneous LE were included. For MDS/CMML systemic LE, a 2:1 case-control study was conducted with idiopathic systemic LE. Clinical features, centralized skin histopathology, and targeted next-generation sequencing were analyzed. Data were analyzed from May 2022 to June 2025. Main outcomes and measures: The clinical, pathological, and molecular features of LE occurring in the setting of MDS or CMML compared with idiopathic LE. Results: Of 24 included patients, 9 (38%) were female, 15 (63%) were male, and the median (range) age at diagnosis was 65 (32-85) years. A total of 19 were diagnosed with systemic LE and 5 with cutaneous LE. The median (range) follow-up was 4.5 (1-31) years. Cutaneous involvement was the most common manifestation of LE (17 [71%]). Chilblain lupus was the predominant subtype (6 [35%]). Compared with idiopathic systemic LE, patients with MDS/CMML-associated LE were older (median [range] age, 65 [32-85] years vs 23 [11-55] years; P < .001), more frequently male (10 [53%] vs 3 [8%]; P = .008), had less kidney involvement (2 [10%] vs 27 [71%]; P < .001), had less articular involvement (7 [36%] vs 37 [97%]; P < .001), and had reduced anti-double-stranded DNA positivity (6 [32%] vs 29 [76%]; P = .001). The underlying hematologic diseases ... |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| Relation: |
info:eu-repo/semantics/altIdentifier/pmid/41259063; unige:191054 |
| Availability: |
https://archive-ouverte.unige.ch/unige:191054 |
| Rights: |
info:eu-repo/semantics/openAccess |
| Accession Number: |
edsbas.59610D1 |
| Database: |
BASE |