Abstract

Abstract Background and Aims Cytomegalovirus (CMV) infection is an opportunistic pathogen in immunocompromised patients and its management is well described after solid organ/bone marrow transplant or in HIV patients. Although less common, since Systemic Lupus Erythemathous (SLE) is a chronic auto immune disease often requiring intense immunosuppression to induce remission of disease exacerbation, SLE patients are also prone to it. Besides complicating the course of the disease, it may be a life-threatening infection. Due to the heterogeneity of SLE manifestations, usually is difficult to distinguish between a SLE flare and CMV active infection, making the diagnose challenging. It has also been described in the literature as an exacerbating SLE factor. We report the case of an Asian, 27-year-old woman, with a recent SLE diagnosis that was admitted with a SLE flare while developing a CMV encephalitis. She was previously admitted due to a nephrotic syndrome. Immunological studies revealed an ANA title of 1:640 and a positive anti dsDNA Ab with diminished C3 and C4 levels, nephrotic range proteinuria and a diagnose of SLE was made. Renal biopsy revealed class IV lupus nephritis. She started high dose intravenous methylprednisolone (3 pulses of 500mg) and mycophenolate mofetil (MMF) at a dose of 2g per day. She was discharged taking 60 mg of oral prednisolone and the same dose of MMF. Two weeks after being discharged, she was readmitted due to worsening anaemia (Hb 6.8 g/dL), thrombocytopenia (Pl 27 000/µL) and deteriorating renal function with a sCr of 5.5 mg/dL with de novo haematuria. It was admitted a severe SLE flare and she was given another 3 pulses of 500mg I.V methylprednisolone and cyclophosphamide (CYC) was started (1 pulse of 500mg I.V). At the same time, she started to complain of myalgias and malaise, generalized hypotonia, developed fever, leukopenia with neutropenia and seizures. Serum CMV viremia was 71 000 copies and CMV polymerase chain reaction was positive in cerebrospinal fluid. She was started on I.V Ganciclovir, CYC was suspended and clinical improvement was observed. Conclusion Studies about the risk of different treatment drugs and other risk factors on the development of CMV disease in SLE are lacking. These studies will be useful for establishing guidelines on the institution of prophylaxis or pre-emptive treatment of CMV infection in SLE patients. Protocols for screening and prevention in this population should be implemented to account for this emerging problem. Given the rising prevalence of CMV infection in the past few years, the authors recommend that patients recently diagnosed with SLE while taking high doses of corticosteroids, which appears to be a risk factor for CMV reactivation, should be routinely tested for CMV viremia. There should be a low threshold for suspicion, hence treatment should be started as soon as possible given the high morbidity and mortality in severe cases.

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