Adult Still's disease associated with cytomegalovirus infection

doi:10.1007/s10156-007-0503-1

Journal of Infection and Chemotherapy

Volume 13, Issue 2, 2007, Pages 114-117

https://doi.org/10.1007/s10156-007-0503-1 Get rights and content

Abstract

A 77-year-old woman was admitted to our hospital complaining of high fever. The physical examination on admission indicated no abnormality. Although several antibiotics were administered, the spiking high fever was not alleviated. Two weeks after admission a macular rash appeared, and a high concentration of serum ferritin was observed. At this point, Still's disease was suspected, and the patient was referred to Nagasaki University Hospital. During the prescribed course of prednisolone, hepatic enzymes gradually increased to high titers accompanied by a positive test for cytomegalovirus (CMV) antigen. The CMV antigen disappeared after ganciclovir administration, and the concentration of serum ferritin decreased after steroid administration. In this report, we attempt to portray the relation between the pathogenesis of adult-onset Still's disease and the presence of CMV antigen.

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Significant association of past parvovirus B19 infection with cytopenia in both adult-onset Still's disease and systemic lupus erythematosus patients
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Citation Excerpt :
Some clinical features of AOSD, including fever, sore throat, rash, arthritis, lymphadenopathy and self-limited or remittent course, are similar to those seen in viral infections, suggesting viral infection may be implicated in the pathogenesis of this disease. Viral infections that have been reported as a possible cause or trigger of AOSD include rubella, Epstein–Barr virus (EBV), cytomegalovirus (CMV), and B19 virus [21–26]. Case reports and small series have indicated an association between B19 infection and AOSD, and suggested B19 infection as one of potential viral infectious triggers of AOSD [23–26].
Human parvovirus B19 (B19) infection has been reported as a possible cause of autoimmune diseases. The association of B19 infection with adult-onset Still's diseases (AOSD) remains unclear.
IgM and IgG against B19-VP1/2 were determined using ELISA in 86 patients with AOSD, 58 patients with systemic lupus erythematosus (SLE) and 64 healthy controls. Anti-B19-VP1-unique region (VP1u) and anti-B19-nonstructural protein-1(NS1) antibodies were assessed by Western blot. B19 DNA was detected by nested PCR.
Forty-three (50.0%) of 86 AOSD patients, 27 (46.6%) of 58 SLE patients and 30 (46.9%) of 64 controls had positivity for anti-B19-VP1/2-IgG in the absence of anti-B19-VP1/2-IgM, indicating past infection. None of enrolled subjects had detectable circulating B19 DNA. Significantly higher positivity rates for anti-B19-VP1u and anti-B19-NS1 IgG were observed in AOSD patients (39.5% and 46.5% respectively) and SLE patients (34.5% and 36.2% respectively) than in healthy controls (14.1%, p < 0.005 for AOSD and p < 0.05 for SLE, and 15.6%, p < 0.001 for AOSD and p < 0.05 for SLE; respectively). AOSD patients and SLE patients with seropositive results for anti-B19-VP1/2 IgG, anti-B19-VP1u or anti-B19-NS1 antibodies had a higher frequency of leucopenia and thrombocytopenia when compared to those with seronegative results. AOSD patients with anti-B19-VP1u or anti-B19-NS1 antibodies were more likely to have arthritis in comparison with those without these antibodies.
Higher seroreactivity for anti-B19-VP1u and anti-B19-NS1 IgG were associated with cytopenia in both AOSD and SLE patients with unique correlation to arthritis in the AOSD. Further studies are necessary to determine if these responses correlate with a common genetic risk in patients with AOSD and SLE.
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La réactivation des Herpesviridae est bien connue chez les patients greffés mais ce phénomène est peu étudié chez des patients recevant un traitement immunosuppresseur dans le cadre d’une maladie inflammatoire systémique.
L’infection à cytomégalovirus (CMV) semble relativement rare, elle peut être facilement diagnostiquée par la polymerase chain reaction (PCR) en temps réel qui représente un moyen diagnostique rapide et fiable. La maladie à CMV se manifeste le plus souvent sous forme de pneumopathie, d’hépatite ou de colite.
Les traitements les plus à risque sont les bolus de corticoïdes et de cyclophosphamide ainsi que le méthotrexate.
Le traitement prophylactique ne peut pas être recommandé en pratique. Le monitoring de la virémie et l’instauration d’un traitement préemptif à partir d’un seuil de virémie à déterminer sont à évaluer.
Le zona est l’infection virale la plus fréquente au cours des maladies de système. La plupart des traitements immunosuppresseurs à part le méthotrexate semblent favoriser l’apparition d’un zona. L’atteinte est rarement systématisée et l’évolution presque toujours favorable. Un traitement prophylactique n’est pas recommandé.
Reactivation of Herpesviridae is well known among transplant patients, but has not been sufficiently studied in patients who receive immunosuppressive treatment for systemic inflammatory diseases.
CMV infection seems relatively rare; it is easily diagnosed by real-time PCR, a fast and reliable diagnostic tool. CMV disease is most often manifested in the form of lung disease, hepatitis, or colitis.
The highest risks are associated with steroid or cyclophosphamide boluses and methotrexate.
Prophylactic treatment cannot be recommended in clinical practice. The utility of monitoring viremia and of preemptive therapy must be evaluated.
Herpes zoster is the most frequent viral infection in systemic diseases. Most immunosuppressive treatments, except methotrexate, promote its occurrence. Visceral involvement is quite rare, and outcome almost always favorable. Prophylactic treatment cannot be recommended.
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