Systemic sclerosis and prevalence of monoclonal immunoglobulin |
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Authors: | Salim Trad Audrey Nosbaum Lucile Musset Pascale Ghillani-Dalbin David Launay Nathalie Costedoat-Chalumeau David Saadoun Jean Cabane Eric Hachulla Thomas Hanslik Camille Frances |
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Institution: | 1. AP-HP, Internal Medicine Department 2, Groupe Hospitalier Pitié Salpêtrière, 75013 Paris, France;2. AP-HP, Internal Medicine Department, Hôpital Saint-Antoine, 75012 Paris, France;3. AP-HP, Immunochemistry Laboratory, Groupe Hospitalier Pitié Salpêtrière, 75013 Paris, France;4. Internal Medicine Department, Hôpital Claude Huriez, CHRU, 59000 Lille, France;5. University Hospital, Lille, 75013 Paris, France;6. Université Pierre et Marie Curie, Paris 6, 75013 Paris, France;g Versailles-Saint-Quentin-en-Yvelines University, 78000 Versailles, France;h AP-HP, Service de Dermatologie-Allergologie, Hôpital Tenon, 75020 Paris, France |
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Abstract: | IntroductionThe purpose of this study was to estimate the prevalence of monoclonal immunoglobulin (MIg) among patients with systemic sclerosis (SSc) according to the capillary electrophoresis or immunofixation method of detection and to search for any related clinical correlations.Patients and methodsRetrospective multicenter comparison of capillary electrophoresis and immunofixation results in SSc patients and of the characteristics of patients with and without MIg.ResultsThe study included 244 SSc patients (216 women and 28 men, mean age: 55 ± 14 years). Median time since SSc diagnosis was 51 months 0–320]; disease was diffuse in 48% of cases. Ten percent of patients had cancer, including Waldenström macroglobulinemia (n = 1) and multiple myeloma (n = 3).Capillary electrophoresis showed a γ-globulin anomaly in 41% of cases, and immunofixation in 18%: MIg (13.5%) and restriction of heterogeneity (4.5%). Capillary electrophoresis failed to detect 60% of the 33 MIg patients. Measurable MIg concentrations were obtained from 7 patients.MIg patients were significantly older at SSc diagnosis than those without MIg (p = 0.002), had a lower diffusing capacity (p = 0.002), a higher prevalence of pulmonary hypertension and cancer (p = 0.002) and were more frequently positive for anti-mitochondrial and anti-beta2-glycoprotein-I antibodies (p = 0.03 and p = 0.02, respectively). Multivariate analyses showed that only age at test hazard ratio 1.03 (95% CI, 1.00–1.07, p = 0.04)] and presence of cancer hazard ratio 4.46 (95% CI, 1.6–12.4, p = 0.004)] were associated with MIg.ConclusionImmunofixation detected a high prevalence of MIg among SSc patients especially in patients aged 50-years or older. MIg was not detected by the standard capillary electrophoresis in 60% of cases and was significantly associated with cancer. |
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Keywords: | ACA anti-centromere antibodies ANA anti-nuclear antibodies CI confidence interval DLCO diffusing capacity for carbon monoxide dSSc diffuse systemic sclerosis FVC forced vital capacity Ig immunoglobulin ILD interstitial lung disease lSSc limited systemic sclerosis MGUS monoclonal gammapathy of unknown significance MIg monoclonal immunoglobulin PAH pulmonary arterial hypertension PBC primary biliary cirrhosis PFT pulmonary function test SD standard deviation SLE systemic lupus erythematosus SSc systemic sclerosis TLC total lung capacity anti-β2GP1 anti-beta2-glycoprotein I |
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