IgM on the surface of T cells: a novel biomarker of pediatric-onset systemic lupus erythematosus
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ORIGINAL ARTICLE
IgM on the surface of T cells: a novel biomarker of pediatric-onset systemic lupus erythematosus Manuela Colucci 1 & Barbara Ruggiero 2 & Alessandra Gianviti 2 & Maria Manuela Rosado 3 & Rita Carsetti 3 & Claudia Bracaglia 4 & Fabrizio De Benedetti 4 & Francesco Emma 2 & Marina Vivarelli 2 Received: 15 June 2020 / Revised: 7 August 2020 / Accepted: 3 September 2020 # IPNA 2020
Abstract Background Children with systemic lupus erythematosus (SLE) frequently have kidney involvement. Lupus nephritis sometimes presents alone, without systemic SLE features, representing the so-called full-house nephropathy (FHN). Distinguishing patients with SLE or FHN has therapeutic and prognostic implications. Methods In this retrospective observational study, we determined the presence of IgM on the surface of T cells (T cell IgM) by flow cytometry and characterized its ability in distinguishing SLE and FHN patients in a large pediatric cohort (n = 84). Fifty-seven patients with SLE (≥ 4 SLICC criteria at disease onset or during the follow-up) and 27 patients with FHN (3 or less SLICC criteria) were enrolled. Results Elevated T cell IgM levels were found in 24/25 SLE patients in active phase of disease and in 29/45 SLE patients in remission. In contrast, among FHN patients, only 1/9 presented this characteristic in active phase of disease and 0/20 in remission. Compared with standardized SLICC laboratory parameters, i.e., autoantibody titers and hypocomplementemia, T cell IgM positivity showed an extremely high sensitivity and specificity for the diagnosis of SLE, with the highest area under the curve (0.97, p < 0.001) by receiver operating characteristic analysis, similar to ANA (0.96, p < 0.001) and anti-dsDNA (0.90, p < 0.001) autoantibodies. Conclusions Altogether, our data indicate that T cell IgM intensity may be a useful tool to correctly classify patients with lupus nephritis as SLE or FHN since disease onset. Keywords Systemic lupus erythematosus . Biomarkers . Immunology . Lymphocytes . IgM
Introduction Systemic lupus erythematosus (SLE) is a severe multisystemic disease. About 10–20% of patients are Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00467-020-04761-7) contains supplementary material, which is available to authorized users. * Manuela Colucci [email protected] 1
Renal Diseases Research Unit, Genetics and Rare Diseases Research Area, Bambino Gesù Children’s Hospital, IRCCS, Viale S. Paolo 15, 00146 Rome, Italy
2
Division of Nephrology, Department of Pediatric Subspecialties, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy
3
B Cell Pathophysiology Unit, Immunology Research Area, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy
4
Division of Rheumatology, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy
diagnosed under 16 years of age [1]. Four SLICC criteria, including one clinical and one immunologic criterion, are required for the diagnosis of SLE both in adults and in children [2, 3]. Different from adults, children
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