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Abstract Background Increased malignancy frequency is well documented in adult-systemic lupus erythematosus (SLE), but with limited reports in childhood-onset SLE (cSLE) series. We explored the frequency of malignancy associated with cSLE, describing clinical and demographic characteristics, disease activity and cumulative damage, by the time of malignancy diagnosis. Method A retrospective case-notes review, in a nationwide cohort from 27 Pediatric Rheumatology centres, with descriptive biopsy-proven malignancy, disease activity/damage accrual, and immunosuppressive treatment were compiled in each participating centre, using a standard protocol. Results Of the 1757 cSLE cases in the updated cohort, 12 (0.7%) developed malignancy with median time 10 years after cSLE diagnosis. There were 91% females, median age at cSLE diagnosis 12 years, median age at malignancy diagnosis 23 years. Of all diagnosed malignancies, 11 were single-site, and a single case with concomitant multiple sites; four had haematological (0.22%) and 8 solid malignancy (0.45%). Median (min–max) SLEDAI-2 K scores were 9 (0–38), median (min–max) SLICC/ACR-DI (SDI) score were 1 (1–5) Histopathology defined 1 Hodgkin's lymphoma, 2 non-Hodgkin's lymphoma, 1 acute lymphoblastic leukaemia; 4 gastrointestinal carcinoma, 1 squamous cell carcinoma of the tongue and 1 anal carcinoma; 1 had sigmoid adenocarcinoma and 1 stomach carcinoid; 3 had genital malignancy, being 1 vulvae, 1 cervix and 1 vulvae and cervix carcinomas; 1 had central nervous system oligodendroglioma; and 1 testicle germ cell teratoma. Conclusion Estimated malignancy frequency of 0.7% was reported during cSLE follow up in a multicentric series. Median disease activity and cumulative damage scores, by the time of malignancy diagnoses, were high; considering that reported in adult series. adultsystemic systemic SLE, , (SLE) childhoodonset childhood onset (cSLE series characteristics casenotes notes review centres biopsyproven biopsy proven activitydamage accrual centre protocol 175 07 0 7 (0.7% 91 females malignancies singlesite, singlesite site, site single-site sites 0.22% 022 22 (0.22% 0.45%. 045 0.45% . 45 (0.45%) min–max minmax min max (min–max SLEDAI2 SLEDAI SLEDAI- 0–38, 038 0–38 38 (0–38) SLICC/ACRDI SLICCACRDI SLICC/ACR DI SLICC ACR SDI (SDI 1–5 15 5 (1–5 Hodgkins Hodgkin s lymphoma nonHodgkins non leukaemia carcinoid carcinomas oligodendroglioma teratoma 0.7 diagnoses high (SLE 17 (0.7 0.22 02 (0.22 04 0.45 (0.45% 03 0–3 (0–38 ACRDI SLICCACR 1– (1– 0. (0. 0.2 (0.2 0.4 (0.45 0– (0–3 (1 (0 (0.4 (0– (