Fatal colitis associated with active systemic lupus erythematosus complicated by cytomegalovirus superinfection.

Gravito-Soares E, et al. BMJ Case Rep 2017. doi:10.1136/bcr-2017-223458 Description A 47-year-old woman was diagnosed with systemic lupus erythematosus (SLE) at age 27 based on articular involvement (Jaccoud’s arthropathy), lymphopaenia, thrombocytopaenia, pleuritis and positive antinuclear/anti-dsDNA antibodies. She was under azathioprine 75 mg and mycophenolate mofetil 3 g daily. Renal involvement (diffuse proliferative glomerulonephritis) was diagnosed 2 months ago. She was admitted with a 2-week history of diarrhoea, abdominal pain and intermittent fever. Physical examination revealed lower abdominal discomfort and mild bilateral leg oedema. Laboratory analysis showed severe hypoalbuminaemia, pancytopaenia, elevated C reactive protein (25.8; n<0.5 mg/dL) and normal coagulation tests. She had active disease Systemic lupus erythematosus disease activity index of 23 (SLEDAI-23), starting prednisolone 1 mg/kg/day and 5-day course of intravenous immunoglobulin 2 g/kg/day; mycophenolate mofetil dose was reduced (2.5 mg/ day). Infectious disease screening was negative, including cytomegalovirus (CMV) (IgM−/IgG+, negative serum CMV-DNA). Ileocolonoscopy showed a colorectal congestive/granular mucosa with erosions, friability and subepithelial haemorrhagic suffusions (figure 1A–C). Biopsies revealed normal ileal mucosa and SLE-related pancolitis (figure 2A,B). However, she worsened with febrile neutropaenia, increasing inflammatory parameters and chronic renal disease without exacerbation or oligoanuria. Infectious disease rescreening, including blood and urine cultures, was negative. Chest and plain abdominal X-rays were normal. Broad-spectrum antibiotics, 3-day course of intravenous methylprednisolone 500 mg, ciclosporin 150 mg/day and filgrastim were added. Two weeks after admission, despite improvement in inflammatory parameters, she developed intermittent haematochezia with haemodynamic instability requiring daily blood components transfusion. Oesophagogastroduodenoscopy was normal. Colonoscopy was repeated showing vasculitis and several ulcers throughout the colon (figure 1D), the biopsies of which revealed CMV-positive cells (figure 2C,D). CMV serology was repeated with negative IgM, but elevated CMV-DNA (5 693 532 copies/mL), starting intravenous ganciclovir 5 mg/kg/day. She continually worsened with severe anaemia (haemoglobin: 4–5 g/dL). Additional coagulopathies study was negative without evidence of disseminated intravascular coagulation. CT angiography showed mild diffuse colorectal wall thickening but no evidence of focal active bleeding, small bowel Fatal colitis associated with active systemic lupus erythematosus complicated by cytomegalovirus superinfection Elisa Gravito-Soares, Marta Gravito-Soares, Maria Augusta Cipriano, Pedro Amaro Images in...