AB1048 SYSTEMIC LUPUS ERYTHEMATOUS WITH UNUSUAL PRESENTATION : SINGLE CENTRE EXPERIENCE FROM NORTH INDIA

Abstract

Background Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease.Presentation can range from constellation of non specific constitutional symptoms to definitive signs and symptoms.Systemic Lupus International Collaborating Clinics(SLICC) criteria help in making a diagnosis of SLE. SLE mimics many clinical entities and diagnosis may become difficult at times,especially if presentation is atypical.We describe children who presented with unusual manifestations and were finally diagnosed to have pediatric SLE. Objectives To highlight unusual presentation of pediatric SLE. Methods Retrospective case review of all children diagnosed as SLE from July 2017–November 2018 at a single tertiary care hospital in north India was done. We here describe 5 children who presented with unusual manifestations. CASE 1: 8 years,girl, presented with fever for 1 month and bilateral pleural effusion. She had had 2 hospitalizations in the past. Once with fever, epistaxis and haemorrhagic shock and subsequently with fever, hepatosplenomegaly and bicytopenia and was managed as tropical infections at both times.She was evaluated this time and was diagnosed to have SLE and managed for the same. CASE 2: 11 years,boy, presented with arthritis in multiple small and large joints of body.No other features of SLE were seen. On evaluation ANA and anti dsDNA was found to be positive and he fulfilled criteria for SLE. He was started on subcutaneous, weekly methotrexate and responded to treatment. However, he developed lupus nephritis during disease course after 6 months of diagnosis. Renal biopsy revealed ClassIII+V lupus nephritis. Intravenous Cyclophosphamide pulses were started for induction along with tapering doses of corticosteroids. CASE 3: 8 years,girl, presented with early morning periorbital puffiness and abdominal distension. She also had a history of photosensitivity and Raynaud phenomenon. Urine routine examination revealed nephrotic range proteinuria with no hematuria. Clinical possibility of nephrotic syndrome secondary to SLE was kept.Renal biopsy revealed Class V Lupus nephritis. She was started on oral prednisolone and enalapril. CASE 4: 15 years old, girl, presented with non-specific generalized body pains with n joint pains associated with low grade fever.There were no other complaints. Examination was unremarkable. Investigations, however, were suggestive of SLE. CASE 5: 17 years old,girl,presented with high grade fever and pain in bilateral knee joints and malar rash. Investigation were suggestive of SLE with MAS(macrophage activation syndrome). Child was started on iv antibiotics and iv immunoglobulins but died during the disease process. ANA positivity, hypocomplimentimia and Anti ds DNA positivity were seen in all the 5 cases. Results: out of 11 children diagnosed to have SLE, 5 presented with unusual manifestations as described above.All patients were initiated on hydroxychloroquine and photoprotection other drugs used were prednisolone, naproxen, subcutaneous weekly methotrexate, cyclophosphamide and Azathioprine. Conclusion In this report we present unusual presentations as the predominant manifestation of SLE and emphasize the fact that early recognition and awareness of unusual presentation of SLE help institute timely initiation of appropriate treatment.