A 37-year-old Caucasian male with a past medical history of alcohol abuse was referred for inpatient admission after being found to have pancytopenia. He had subjective fever and productive cough with yellow sputum a few weeks ago, which resolved after a few days without treatment. He visited his primary care physician and laboratory exam showed pancytopenia, thus leading to the referral. He also endorsed 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, and Raynaud's phenomenon. Physical exam was only remarkable for cervical nontender lymphadenopathy.
Upon admission, he was febrile with a maximum temperature of 101.2 F. Laboratory exam was notable for white blood cell 1,900 counts/muL, hemoglobin 7.2 g/dL, hematocrit 21.2%, platelet 19,000 counts/muL, sodium 126 mmol/L, and creatinine 1.44 mg/dL. Urinalysis showed elevated white blood cells, dysmorphic red blood cells, and proteinuria. Further work up showed that he had elevated ferritin 6542 ng/mL, high triglycerides 327 mg/dL, marked decreased complement levels (C3 14 mg/dL and C4 3 mg/dL), elevated ESR 137 mm/hr, strongly positive for antinuclear antibody (ANA, 1 : 640), and anti-double-stranded DNA antibody (anti-dsDNA, >1 : 1280). Anticardiolipin antibody (IgM and IgG) and beta-2 glycoprotein I antibody (IgG) were also positive. Direct antiglobulin test (Coomb's) was positive. Urine studies showed microalbumin to creatinine ratio of 1958 mg/g and protein creatinine ratio of 7.04 mg/mg consistent with nephrotic-range proteinuria. Transthoracic echocardiogram showed normal ventricular function but moderate circumferential pericardial effusion. Abdominal ultrasonography showed hepatosplenomegaly with evidence of cirrhosis. Based on the above findings, he was diagnosed with systemic lupus erythematosus, pericarditis, and lupus nephritis. 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 macrophage activation syndrome considering his clinical presentation. Renal biopsy was also performed and confirmed diffuse proliferative and membranous lupus nephritis (Class IV/V) along with focal segmental glomerulosclerosis NOS type. He also underwent ophthalmology evaluation, and funduscopic exam with fluorescein angiogram showed cotton-wool spots with retinal hemorrhage consistent with retinal vasculitis. His laboratory examination continued to improve, and he was subsequently discharged from hospital one week after admission. Mycophenolate mofetil was started in addition to steroid upon discharge. Repeat transthoracic echocardiogram three weeks after treatment showed marked decrease in pericardial effusions. His disease remained stable while on mycophenolate mofetil with prednisone gradually tapered from 60 mg to 10 mg twelfth week after discharge. His vision acuity remained stable, and repeat funduscopic examination showed improvement of his retinal lesions. Detailed laboratory examination in the hospital and on follow-up visit is presented in Table 1.