Systemic Lupus Erythematosus mimicking lymphoproliferative malignancy in a child

Abstract

Systemic Lupus Erythematosus (SLE) is a chromic autoimmune disease with multisystemic involvement. The estimated prevalence of SLE among children is 1-6/100000. Here we report a child who presented with generalized lymphadeopathy as the first manifestation of SLE. A 13 year old previously healthy girl presented with cervical lymphadenopathy and low grade remitting fever for 1 month duration. On examination she was found to have generalized lymphadenopathy and unremarkable rest of the clinical examination. Her basic haematological and biochemical parameters were normal. ESR was 110mm/hr and CRP was 6mg/dL. Ultrasound scan and Contrast CT of the abdomen and pelvis revealed extensive mediastinal, mesenteric, para-aortic, splenic hilar, bilateral axillary and inguinal lymphadenopathy suggestive of lymphoproliferative malignancy. However, her bone marrow and lymph node biopsies revealed reactive pattern. Meantime she developed a malar rash, oral ulcers and 2 episodes of generalized tonic-clonic convulsions. Her urinalysis revealed microscopic haematuria and sub nephrotic range proteinuria. Her ANA and dsDNA levels were positive. Her subsequent full blood count revealed severe bicytopenia, predominetly lymphopenia.The diagnosis of SLE was made and she was started on Hydroxychloroquine, high dose prednisolone and cyclophosphamide pulse therapy. The most common presenting symptoms of SLE in children include, constituitional symptoms, joint symptoms and haematological abnormalities. It is estimated 23-34% children with SLE are having lymphadenopathy. Though, generalized lymphadenopathy as the presenting symptom of SLE is uncommon, it should not be forgotten in order to avoid diagnostic delay.