Similarities and differences between pediatric and adult patients with systemic lupus erythematosus

Abstract

Systemic lupus erythematosus (SLE) is a multifactorial autoimmune disease with highest prevalence among women of childbearing age. However, children younger than 16 years also can develop SLE (childhood-onset lupus/juvenile-type SLE). The aim of our study was to compare the clinical course of adult and pediatric-onset SLE. Data from 342 adult patients followed at the University of Debrecen, Hungary, and 79 children documented in the Hungarian National Pediatric SLE registry were analyzed using hospital medical records. Organ manifestations, laboratory parameters, and immunoserological characteristics were reviewed and the results were evaluated using SPSS for Windows software.Gender distribution was not significantly different between groups with disease starting in childhood vs adulthood. The prevalence of the following manifestations was significantly higher for pediatric than for adult-onset disease including: lupus nephritis (43% pediatric vs 26.4% for adult-onset), hematological disorders (57% vs 36.4%), photosensitivity (20% vs 9%), butterfly rash (61% vs 35.5%) and mucosal ulceration (11.4% vs 4%). For adult-onset SLE, neurological symptoms (30% vs 6%) and polyarthritis (86% vs 68%) occurred significantly more frequently than in children. Anti-SSA, anti-SSB and antiphospholipid antibodies were detected at significantly higher levels in adult-onset patients compared to those in pediatrics. Children were more commonly given high-dose intravenous immunoglobulin treatment (6.3% vs 0.6%) and mycophenolate mofetil (15.2% vs 5.3%) than adults.These results suggest that pediatric and adult-onset SLE differ in multiple aspects, and it is important to recognize these differences for optimal treatment and prognosis of these patients.

Prospective validation of a novel renal activity index of lupus nephritis

“…This is especially important for the use of the RAIL in adults with LN as they frequently are found to have not only features of LN activity, but also histological changes compatible with chronic LN damage on kidney biopsy (12,13). …”

Evans Syndrome at Childhood‐Onset Systemic Lupus Erythematosus Diagnosis: A Large Multicenter Study

“…The median of current prednisone dose was similar between groups (1.1 [0.76-1.5] vs. 1.0 mg/kg/day [0-30], P = 0.195). The median duration of cytopenia in ES cSLE patients was 4 months (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12). None of ES patients had ITP/AIHA recurrence or died.…”

“…Childhood‐onset systemic lupus erythematosus (cSLE) is a rare systemic inflammatory autoimmune disease characterized by the involvement of various organs and systems, including hematologic manifestations . Hematologic features have been reported from 34% to 86% in pediatric lupus populations and the most frequently reported are anemia of chronic disease, immune thrombocytopenia (ITP), autoimmune hemolytic anemia (AIHA), thrombotic thrombocytopenic purpura, and antiphospholipid syndrome …”

“…Childhood-onset systemic lupus erythematosus (cSLE) is a rare systemic inflammatory autoimmune disease characterized by the involvement of various organs and systems, including hematologic manifestations. [1][2][3] Hematologic features have been reported from 34% to 86% in pediatric lupus populations [2][3][4][5][6][7] and the most frequently reported are anemia of chronic disease, [6] immune thrombocytopenia (ITP), [8,9] autoimmune hemolytic anemia (AIHA), [4,6,7,9] thrombotic thrombocytopenic purpura, [6,[9][10][11] and antiphospholipid syndrome. [6,9,12,13] Evans syndrome (ES) is an uncommon manifestation characterized by autoimmune destruction of red cells and platelets and concomitant or sequential appearance of ITP and AIHA.…”

Organ involvement other than lupus nephritis in childhood-onset systemic lupus erythematosus

“…4 There is considerable debate concerning the relative frequency, with some studies reporting a higher incidence in adults, 5 and others similar proportions. 6 The types of pulmonary manifestations reported are diverse, and may involve any portion of the pulmonary organ system including the pleura, diaphragm, parenchyma, and vasculature. The wide range of prevalence estimates found in the literature may be due to known racial and ethnic phenotypic variability, as well as different approaches taken to determine the presence of pulmonary involvement with cSLE.…”