Vessel Wall Inflammatory Activity as Determined by F-18 Fluorodeoxyglucose PET in Large Vessel Vasculitis Is Attenuated by Immunomodulatory Drugs.

F-18 fluorodeoxyglucose (F-18 FDG) PET/CT plays an increasing role in the diagnostic workup of large vessel vasculitis (LVV); however, information on the relationship between immunosuppressive drugs and vessel wall uptake is limited. In 94 patients with a confirmed diagnosis of LVV, the vessel wall-to-liver ratio (VLR) was assessed in eight vessel segments. Patients were grouped according to intake of immunomodulatory drugs (Group 1, prednisone; Group 2, prednisone + methotrexate; and Group 3, prednisone + others) and compared to treatment-naïve individuals. A total of 54/94 (57.4%) were treated with immunomodulatory drugs (Group 1, 29/49 (53.7%); Group 2, 9/54 (16.7%); Group 3, 11/54 (20.4%); and Group 4, 5/54 (9.3%)), whereas the remainder received no therapy (40/94 (42.6%)). The mean VLR of the arterial segments correlated significantly with the hematopoietic organs (r ≥ 0.22, p ≤ 0.05), c-reactive protein (r ≥ 0.25, p ≤ 0.05), and prednisone dosage (r ≥ -0.4, p ≤ 0.05). Relative to treatment-naïve patients, a significantly lower VLR was recorded in 5/8 (62.5%) of the investigated vessel segments in Group 1 (p ≤ 0.02), in 6/8 of the vessel segments in Group 2 (75.0%, p ≤ 0.006), and in 7/8 of the segments in Group 3 (87.5%, p ≤ 0.05). In LVV, the F-18 FDG uptake in vessel wall as a marker of inflammatory activity was attenuated by immunomodulatory drugs, which provides a foundation for future serial monitoring of treatment efficacy.

Severe Progressive Multifocal Leukoencephalopathy (PML) and Spontaneous Immune Reconstitution Inflammatory Syndrome (IRIS) in an Immunocompetent Patient.

Background: Progressive multifocal leukoencephalopathy (PML) is an opportunistic infection with JC-virus (JCV), a papova-virus, affecting mostly oligodendrocytes and the white matter of the central nervous system. Progressive Multifocal Leukoencephalopathy (PML) almost exclusively occurs in immunocompromised patients based on different underlying conditions of severe cellular immunodeficiency such as HIV/AIDS, secondary to neoplastic and autoimmune diseases, or during immunosuppressive therapy. Case presentation: We present the case of an otherwise healthy and immunocompetent patient without immunosuppressive therapy who was admitted with hemianopsia to the right side, sensory aphasia and changes of behavior. Magnet resonance imaging (MRI) and laboratory testing confirmed the diagnosis of PML, although functional tests did not show any evidence for cellular immunodeficiency. Extensive immunological tests did not reveal an apparent immunodeficiency. During symptomatic therapy the patient developed seizures which were assumed to be caused by a spontaneous immune reconstitution inflammatory syndrome (IRIS) demonstrated by MRI. We added a high dose of intravenous corticosteroids to the antiepileptic treatment and seizures ended shortly thereafter. However, the impairments of vision, behavior and language persisted. Conclusions: Our case report highlights that an apparently immunocompetent patient can develop PML and IRIS spontaneously. Therefore, MRI should be applied immediately whenever a rapid progression of PML symptoms occurs as treatment of IRIS with corticosteroids can result in a marked clinical improvement.