Adult Onset Still's Disease and Rocky Mountain Spotted Fever

Abstract

Adult Still's Disease was first described in 1971 by Bywaters in fourteen adult female patients who presented with symptoms indistinguishable from that of classic childhood Still's Disease (Bywaters, 1971). George Still in 1896 first recognized this triad of quotidian (daily) fevers, evanescent rash, and arthritis in children with what later became known as juvenile inflammatory arthritis (Still, 1990). Adult Onset Still's Disease (AOSD) is an inflammatory condition of unknown etiology characterized by an evanescent rash, quotidian fevers, and arthralgias. Numerous infectious agents have been associated with its presentation. This case is to our knowledge the first presentation of AOSD in the setting of Rocky Mountain Spotted Fever. Although numerous infectious agents have been suggested, the etiology of this disorder remains elusive. Nevertheless, infection may in fact play a role in triggering the onset of symptoms in those with this disorder. Our case presentation is, to our knowledge, the first case of Adult Onset Still's Disease associated with Rocky Mountain spotted fever (RMSF).

Bartonella henselae infection presenting with a picture of adult-onset Still's disease

“…Numerous infectious agents have been proposed as potential inciting factors for AOSD so far, but not Bartonella. [18][19][20] This is the first report suggesting the possibility that B. henselae may in part be responsible for the development of AOSD by means of either a direct inflammatory process or 'molecular mimicry' that triggers the host's autoimmune response. Further research is necessary to determine the role of Bartonella in the pathogenesis of AOSD.…”

Interstitial Lung Disease: A Rare Association with Adult-Onset Still’s Disease

“…6 One case review also reported AOSD with Rocky Mountain spotted fever. 7 AOSD can be divided into two distinctive phenotypes, i.e., a systemic pattern and articular pattern based on cytokine profile, clinical features, and laboratory investigation. 8 Mechanisms for autoimmunity, such as activation of macrophage, inflammatory cytokines (e.g., IL-1, IL-6, IL-18, IFNδ, and TNFα), and genetic factors, along with infection play a role in the pathogenesis of AOSD as indicated in literature.…”