Pediatric Lupus: Varied Haematological Picture and Presentation

Abstract

Systemic lupus erythematosus (SLE) is a chronic, multisystem, autoimmune disease characterized by periods of increased disease activity caused by inflammation of blood vessels and connective tissue. Pediatric patients with SLE have a more severe clinical course when compared with adults. Patients commonly present with rash, fever, and arthritis, although the presentation may be unpredictable. Hematological findings are more predominant in children than adults. Thirty-nine percent of children with SLE will develop hematological abnormalities, one of the American Rheumatic Association criteria for classifying the disease. In our case series we found varied hematological picture and presentation. We present here four case reports of SLE cases with interesting hematological features. Our first case is a 13 month old female child who was initially diagnosed as Evans syndrome and 2 years later diagnosed as SLE. Second case is a 3 year old male child who had SLE with warm antibody AIHA. Third case is a 6 year old female child who presented with AIHA and was diagnosed with SLE 6 years later. Fourth case is a 6 year old female child diagnosed as SLE with aplastic anemia. Hematological findings should be carefully assessed and treated in order to decrease disease related morbidity.

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

“…Despite the fact that isolated AIHA or isolated ITP are common lupus manifestations, ES has been rarely studied in cSLE and adult SLE populations . The occurrence of ES during lupus follow‐up was reported in 2.7–10% of adult SLE and 8.4–10% of cSLE patients .…”

“…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.…”

Síndrome de Evans em pacientes com lúpus eritematoso sistêmico juvenil

“…, púrpura trombocitopênica imune (PTI) 13,14 , anemia hemolítica autoimune (AHAI) 9,11,12,14 , púrpura trombocitopênica trombótica 11, 14-16 e síndrome do anticorpo antifosfolípide 11,14,17,18 .…”

“…Essa síndrome foi raramente descrita em pacientes com LESJ, sempre limitada a pequenas populações e em relato de casos 12,14,25,26 . No entanto, a prevalência dessa manifestação na população de LESJ bem como seus possíveis fatores associados, ainda não foi estudada.…”