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Yiming Luo

Department of Medicine, Mount Sinai St Luke’s and Mount Sinai West, Icahn School of Medicine at Mount Sinai, New York

Title: Macrophage Activation Syndrome, Glomerulonephritis, Pericarditis and Retinal Vasculitis as Initial Presentation of Systemic Lupus Erythematosus

Biography

Biography: Yiming Luo

Abstract

Background: Systemic lupus erythematosus (SLE) is a systemic autoimmune disorder which can involve almost all vital organs. The clinical heterogeneity of the disease presents a consistent challenge to clinicians. Macrophage activation syndrome (MAS), also known as hemophagocytic lymphohistiocytosis (HLH), is a rare and potentially fatal complication of several autoimmune diseases. The incidence of MAS associated with SLE is about 0.9–4.6%, while MAS as initial presentation of SLE is rarer with less than 30 cases reported in English literature. We report a rare case of a combination of MAS, glomerulonephritis, pericarditis and retinal vasculitis as the initial presentation of systemic lupus erythematosus.

Case Report: 37-year-old Caucasian male with no past medical history was referred for inpatient admission after being found to have pancytopenia. He endorsed fever with maximal temperature 101.2 F, generalized weakness, progressive right eye blurry vision and chest tightness for the past few weeks. There was no skin rash, joint pain, hair loss, heartburn, Raynaud’s phenomenon. Physical exam was only remarkable cervical nontender lymphadenopathy. Laboratory exam was notable for pancytopenia and elevated creatinine. Urine studies showed elevated leukocytes, dysmorphic erythrocytes and nephrotic range roteinuria. Subsequent work up revealed elevated ferritin 6542 ng/mL, high triglycerides 327 mg/dL, marked decreased complement levels (C3 14 mg/dL, C4 3 mg/dL), elevated ESR 137 mm/hr, strong positive for antinuclear antibody (ANA, 1:640) and anti double-stranded DNA antibody (anti-dsDNA, >1:1280). Transthoracic echocardiogram showed normal ventricular function but moderate circumferential pericardial effusion. Abdominal ultrasonography demonstrated hepatosplenomegaly. Funduscopic exam with fluorescein angiogram showed cotton wool spots with retinal hemorrhage consistent with retinal vasculitis. Based on the above findings, he was diagnosed with systemic lupus erythematosus, pericarditis, lupus nephritis and retinal vasculitis. Pulse steroid with methylprednisolone 1000 mg daily was started for 3 days, followed by oral prednisone 60 mg daily. His fever and chest pain resolved and his blood cell counts and creatinine improved after the treatment. He subsequently underwent bone marrow biopsy which showed increased histiocytes with focal evidence of hemophagocytic cells consistent with MAS. Renal biopsy was also performed and confirmed diffuse proliferative and membranous lupus nephritis (Class IV/V). His clinical condition continued to improve with systemic steroid and he was subsequently discharged from the hospital.

Conclusion: The diagnosis of MAS in SLE can be challenging, as the presentation can mimic the clinical features of SLE or an infectious complication. Bone marrow biopsy should be considered in cases that the clinical picture is not clear. This case also highlights the importance of multi-specialty collaboration in the management of systemic autoimmune diseases.