Distinct Clinical Correlates of Immune Thrombocytopenic Purpura at Diagnosis of Childhood-Onset and Adult SLE

Gladys Esteves¹, Natali W. Gormezano², Oriany Pereira¹, David Kern¹, Katia T. Kozu², Rosa M R Pereira³, Clovis A Silva⁴, Eloisa Bonfa⁵ and Nadia E Aikawa⁶, ¹University of São Paulo, São Paulo, Brazil, ²Pediatric Rheumatology Unit, University of São Paulo, São Paulo, Brazil, ³Rheumatology, University of São Paulo, São Paulo, Brazil, ⁴Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil, ⁵Rheumatology Divison, Hospital das Clinicas, Faculdade de Medicina, University of São Paulo, São Paulo, Brazil, ⁶Rheumatology Division, University of São Paulo, São Paulo, Brazil

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: Immune, juvenile SLE and thrombocytopenia, SLE

Session Information

Date: Monday, November 14, 2016

Title: Pediatric Rheumatology – Clinical and Therapeutic Aspects - Poster II: Myositis, Systemic Lupus Erythematosus, Sjögren's Syndrome

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: Hematologic abnormalities are common manifestations in systemic lupus erythematosus (SLE) patients. Several studies have demonstrated a higher frequency of immune thrombocytopenic purpura (ITP) in pediatric compared to adult lupus but none evaluated ITP at lupus diagnosis in both age groups. Simultaneous diagnosis of these two conditions is challenging and analysis of clinical correlates of ITP in pediatric and adults at lupus presentation may provide a more appropriate time frame from diagnosis to treatment in these patients. Therefore, the objectives of this study were to compare clinical and laboratorial features between pediatric and adult patients at concomitant diagnosis of ITP and SLE.

Methods: This was a retrospective study evaluating 336 cSLE and 1,830 aSLE patients from 1983 to 2014 regularly followed at the Pediatric Rheumatology Unit and the Rheumatology Division of the same University hospital. ITP was defined as platelets count <100,000/mm³ in the absence of other causes often with the characteristic purpuric rash. Data were obtained from medical charts assessed at ITP and SLE diagnosis, including demographic characteristics, clinical and laboratorial findings of SLE, disease activity and initial treatment.

Results: The median current age was 11.6 years and 27.3 years, respectively. The frequencies of female gender and Caucasian race were similar in both groups (p > 0.05). cSLE had a higher frequency of ITP compared to aSLE (17% vs. 4%, p<0.0001) and the former group had more hemorrhagic manifestations (59% vs. 34%, p=0.007). Simultaneous severe lupus manifestations such as pericarditis (25% vs. 10%, p=0.029) and central nervous system involvement (30% vs. 14%, p=0.029) were more common in cSLE. Concomitant constitutional symptoms (fever and weight loss) and reticuloendothelial manifestations (adenomegaly, hepatomegaly and splenomegaly) were also more frequent in this age group (p<0.05), whereas cutaneous and articular involvements were less often observed in cSLE patients (p<0.05). A trend for a higher SLEDAI score was observed in pediatric patients (18 vs. 13, p=0.072). Analysis of autoantibodies showed a lower frequency of anti-Ro (11% vs. 27%, p=0.026) in cSLE patients compared to adults. Concerning treatment, intravenous methylprednisolone, intravenous immunoglobulin, blood transfusion and platelets transfusion were more frequently used in the pediatric population (p < 0.05).

Conclusion: ITP at cSLE presentation is more severe than in aSLE with distinct features characterized by major organ involvements and constitutional/reticuloendothelial manifestations. These findings reinforce the need for a more aggressive treatment in this age group.

Disclosure: G. Esteves, None; N. W. Gormezano, None; O. Pereira, None; D. Kern, None; K. T. Kozu, None; R. M. R. Pereira, None; C. A. Silva, CNPq, 2; E. Bonfa, None; N. E. Aikawa, None.

To cite this abstract in AMA style:

Esteves G, Gormezano NW, Pereira O, Kern D, Kozu KT, Pereira RMR, Silva CA, Bonfa E, Aikawa NE. Distinct Clinical Correlates of Immune Thrombocytopenic Purpura at Diagnosis of Childhood-Onset and Adult SLE [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/distinct-clinical-correlates-of-immune-thrombocytopenic-purpura-at-diagnosis-of-childhood-onset-and-adult-sle/. Accessed .

« Back to 2016 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/distinct-clinical-correlates-of-immune-thrombocytopenic-purpura-at-diagnosis-of-childhood-onset-and-adult-sle/