BackgroundHemophagocytic lymphohistiocytosis is an aggressive life-threatening syndrome of excessive immune activation. Hemophagocytic lymphohistiocytosis due to systemic lupus erythematosus is described as acute lupus hemophagocytic syndrome. Acute lupus hemophagocytic syndrome presenting with negative antinuclear antibody is uncommon.Case presentationA 57-year-old Sri Lankan woman presented with intermittent fever, weight loss, episodic confusion, and alopecia for 3 months. Investigations showed pancytopenia. Her erythrocyte sedimentation rate was 76 mm/hour and C-reactive protein was 2 mg/l. Septic screen was negative except for a positive sputum culture for Gram-negative coliforms. Chest X-ray was normal. Direct antiglobulin test was positive. Fever persisted with clinical worsening despite treatment with intravenous antibiotics. Antinuclear antibodies and double-stranded-deoxyribonucleic acid antibodies were negative. Bone marrow aspiration revealed features compatible with hemophagocytosis. Her serum ferritin and triglycerides were elevated. Diagnosis of hemophagocytic lymphohistiocytosis due to an evolving autoimmune disorder was made and she was treated with steroids. She showed a dramatic improvement and was discharged on oral steroids.After 6 months, while the steroids were being tapered she experienced oral ulcers, frothy urine, and ankle swelling and she was rehospitalized. Urine analysis revealed proteinuria with active sediment. Antinuclear antibodies and double-stranded-deoxyribonucleic acid antibodies were positive. Complement C3 and C4 were reduced. A renal biopsy revealed class IV-G lupus nephritis with immunofluorescence pattern consistent with systemic lupus erythematosus. Steroid dose was increased and mycophenolate mofetil was commenced. She improved.ConclusionsThis case showcases an uncommon presentation of acute lupus hemophagocytic syndrome with initial negative antinuclear antibody probably due to its cytokine-mediated pathogenesis. This is the first such reported case in South Asia to the best of our knowledge. According to the American College of Rheumatology criteria, our patient did not fulfill the criteria for systemic lupus erythematosus diagnosis for the initial hospitalization. But, according to the 2012 Systemic Lupus International Collaborating Clinics criteria, she did fulfill the criteria for systemic lupus erythematosus even in the first hospitalization which was subsequently proven with renal biopsy findings. This case confirms the increased sensitivity of Systemic Lupus International Collaborating Clinics criteria over American College of Rheumatology criteria in diagnosis of systemic lupus erythematosus.
BackgroundHaemophagocytic Lymphohistiocytosis (HLH) is an aggressive, life-threatening syndrome of increased immune activation.1 HLH secondary to underlying systemic lupus erythematosus (SLE) is described as acute lupus haemophagocytic syndrome (ALHS).2 ALHS presenting with negative ANA is uncommon. We describe a case of ALHS with diagnostic difficulty, presenting with negative ANA.Materials and methodsWe present an unusual case of a 57 year old female hospitalised with on and off fever, episodes of confusion and alopecia for 03 months.ResultsInvestigations showed a pancytopenia (haemoglobin 6.0 g/dl, WBC 1,690/mm3, platelets 102,000/mm3). ESR was 76 mm/hour. CRP was 02 mg/L. Septic screen was negative except for a positive sputum culture for coliforms. Chest x-ray was normal. Direct Coombs test was positive. The fever persisted despite treatment with IV antibiotics. ANA and Ds-DNA were negative. Bone marrow revealed features of primary or secondary haemophagocytosis. Serum ferritin (>1650 ng/dl) and triglycerides were elevated. Diagnosis of HLH probably due to an evolving autoimmune disorder was made and the patient was treated with steroids. The patient dramatically improved and was discharged on oral steroids.After 06 months, she experienced frothy urine, ankle swelling and readmitted. Evaluation revealed proteinuria with active sediment in urine. ANA and Ds DNA were positive. Complement C3 and C4 were reduced. Renal biopsy revealed Class IV G lupus nephritis with immunofluorescence pattern consistent with SLE. The dose of steroids were increased and she was started on mycophenolate mofetil. The patient improved.ConclusionThis case showcases an uncommon presentation of ALHS with initial negative ANA probably due to its cytokine mediated pathogenesis. This is the first such reported case in South Asia to our knowledge. Interestingly, according to the ACR criteria and its 1997 update, this patient does not fulfil the criteria for SLE diagnosis for the initial hospitalisation. But according to the 2012 SLICC revised ACR SLE criteria, the patient fulfils the criteria for SLE even in the first hospitalisation which was subsequently proven without doubt with renal biopsy findings. This case confirms the increased sensitivity of SLICC criteria over ACR criteria in diagnosis of SLE.References1. Rahal AK, Fernandez J, Dakhil C. Undiagnosed Systemic Lupus Erythematosus Presenting as Hemophagocytic Lymphohistiocytosis. Case Rep Rheumatol. 2015;2015:748713.2. Wong KF, Hui PK, Chan JK, Chan YW, Ha SY. The acute lupus hemophagocytic syndrome. Ann Intern Med. 1991;114(5):387–90.
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