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Article

Long-Term Clinical Outcome in Systemic Lupus Erythematosus Patients Followed for More Than 20 Years: The Milan Systemic Lupus Erythematosus Consortium (SMiLE) Cohort

by
Maria Gerosa
1,2,*,
Lorenzo Beretta
3,
Giuseppe Alvise Ramirez
4,
Enrica Bozzolo
4,
Martina Cornalba
2,
Chiara Bellocchi
3,
Lorenza Maria Argolini
2,
Luca Moroni
4,
Nicola Farina
4,
Giulia Segatto
3,
Lorenzo Dagna
4 and
Roberto Caporali
1,2
1
Research Centre for Adult and Pediatric Rheumatic Diseases, Department of Clinical Sciences and Community Health, University of Milan, 20129 Milan, Italy
2
ASST Pini CTO, Lupus Clinic, Division of Clinical Rheumatology, 20122 Milan, Italy
3
Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Referral Centre for Systemic Autoimmune Diseases, 20122 Milan, Italy
4
IRCCS Ospedale San Raffaele, Unit of Immunology, Rheumatology, Allergy and Rare Diseases, 20132 Milan, Italy
*
Author to whom correspondence should be addressed.
J. Clin. Med. 2022, 11(13), 3587; https://doi.org/10.3390/jcm11133587
Submission received: 25 April 2022 / Revised: 31 May 2022 / Accepted: 16 June 2022 / Published: 22 June 2022
(This article belongs to the Special Issue Clinical Features and Long-Term Outcomes of Systemic Lupus Erythematosus)
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Abstract

Tackling active disease to prevent damage accrual constitutes a major goal in the management of patients with systemic lupus erythematosus (SLE). Patients with early onset disease or in the early phase of the disease course are at increased risk of developing severe manifestations and subsequent damage accrual, while less is known about the course of the disease in the long term. To address this issue, we performed a multicentre retrospective observational study focused on patients living with SLE for at least 20 years and determined their disease status at 15 and 20 years after onset and at their last clinical evaluation. Disease activity was measured through the British Isles Lupus Assessment Group (BILAG) tool and late flares were defined as worsening in one or more BILAG domains after 20 years of disease. Remission was classified according to attainment of lupus low-disease-activity state (LLDAS) criteria or the Definitions Of Remission In SLE (DORIS) parameters. Damage was quantitated through the Systemic Lupus Erythematosus International Collaborating Clinics/American College of Rheumatology damage index (SLICC/ACR-DI). LLAS/DORIS remission prevalence steadily increased over time. In total, 84 patients had a late flare and 88 had late damage accrual. Lack of LLDAS/DORIS remission status at the 20 year timepoint (p = 0.0026 and p = 0.0337, respectively), prednisone dose ≥ 7.5 mg (p = 9.17 × 10−5) or active serology (either dsDNA binding, low complement or both; p = 0.001) were all associated with increased late flare risk. Late flares, in turn, heralded the development of late damage (p = 2.7 × 10−5). These data suggest that patients with longstanding SLE are frequently in remission but still at risk of disease flares and eventual damage accrual, suggesting the need for tailored monitoring and therapeutic approaches aiming at effective immunomodulation besides immunosuppression, at least by means of steroids.
Keywords: lupus; flare; damage; long disease duration; trajectories; remission; low disease activity lupus; flare; damage; long disease duration; trajectories; remission; low disease activity

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MDPI and ACS Style

Gerosa, M.; Beretta, L.; Ramirez, G.A.; Bozzolo, E.; Cornalba, M.; Bellocchi, C.; Argolini, L.M.; Moroni, L.; Farina, N.; Segatto, G.; et al. Long-Term Clinical Outcome in Systemic Lupus Erythematosus Patients Followed for More Than 20 Years: The Milan Systemic Lupus Erythematosus Consortium (SMiLE) Cohort. J. Clin. Med. 2022, 11, 3587. https://doi.org/10.3390/jcm11133587

AMA Style

Gerosa M, Beretta L, Ramirez GA, Bozzolo E, Cornalba M, Bellocchi C, Argolini LM, Moroni L, Farina N, Segatto G, et al. Long-Term Clinical Outcome in Systemic Lupus Erythematosus Patients Followed for More Than 20 Years: The Milan Systemic Lupus Erythematosus Consortium (SMiLE) Cohort. Journal of Clinical Medicine. 2022; 11(13):3587. https://doi.org/10.3390/jcm11133587

Chicago/Turabian Style

Gerosa, Maria, Lorenzo Beretta, Giuseppe Alvise Ramirez, Enrica Bozzolo, Martina Cornalba, Chiara Bellocchi, Lorenza Maria Argolini, Luca Moroni, Nicola Farina, Giulia Segatto, and et al. 2022. "Long-Term Clinical Outcome in Systemic Lupus Erythematosus Patients Followed for More Than 20 Years: The Milan Systemic Lupus Erythematosus Consortium (SMiLE) Cohort" Journal of Clinical Medicine 11, no. 13: 3587. https://doi.org/10.3390/jcm11133587

APA Style

Gerosa, M., Beretta, L., Ramirez, G. A., Bozzolo, E., Cornalba, M., Bellocchi, C., Argolini, L. M., Moroni, L., Farina, N., Segatto, G., Dagna, L., & Caporali, R. (2022). Long-Term Clinical Outcome in Systemic Lupus Erythematosus Patients Followed for More Than 20 Years: The Milan Systemic Lupus Erythematosus Consortium (SMiLE) Cohort. Journal of Clinical Medicine, 11(13), 3587. https://doi.org/10.3390/jcm11133587

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