Distinct clinical correlates of immune thrombocytopenic purpura at diagnosis of childhood-onset and adult SLE

Abstract

Objectives: To compare clinical and laboratorial features between childhood-onset systemic lupus erythematosus (cSLE) and adult SLE (aSLE) at concomitant diagnosis of immune thrombocytopenic purpura (ITP).

Methods: This study evaluated 56 cSLE and 73 aSLE patients regularly followed at Pediatric and Rheumatology Divisions of the same University hospital with ITP (platelets count <100,000/mm³ in the absence of other causes) at lupus onset.

Results: Median current age was 11.6 and 27.3 years in cSLE and aSLE, respectively. cSLE had a higher frequency of ITP compared to aSLE (17% vs. 4%, p < .0001) and the former group had more hemorrhagic manifestations (36% vs. 16%, p = .0143). Constitutional symptoms and reticuloendothelial manifestations (p < .05), as well as pericarditis (25% vs. 10%, p = .029) and central nervous system (CNS) involvement (30% vs. 14%, p = .029) were more common in cSLE. Conversely, in aSLE, ITP was solely associated with cutaneous and articular involvements (p < .05). Concerning treatment, intravenous methylprednisolone, intravenous immunoglobulin, blood transfusion and platelets transfusion were more frequently used in the cSLE population (p < .05).

Conclusion: ITP at cSLE has distinct features compared to aSLE with a more severe presentation characterized by concomitant constitutional/reticuloendothelial manifestations, CNS involvement and hemorrhagic manifestation. These findings reinforce the need for a more aggressive treatment in this age group.

Keywords:

• Adult systemic lupus erythematosus
• childhood-onset systemic lupus erythematosus
• hematologic manifestation
• immune thrombocytopenic purpura

Additional information

Funding

This study was supported by grants from Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq 301805/2013-0 to RMRP, 303422/2015-7 to CAS and 305068/2014-8 to EB), Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP #2015/20056-6 to GCXE and #2015/03756-4 to EB and Federico Foundation (to RMRP, CAS and EB).