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.