Buerger’s Disease May be a Chronic Rickettsial Infection with Superimposed Thrombosis: Literature Review and Efficacy of Doxycycline in Three Patients

ABSTRACT

Buerger’s disease (BD) is a chronic inflammatory vasculitis of unknown etiology. The infectious etiology of BD was proposed by Buerger in 1914. Furthermore, there are scattered reports insisting that BD may be related to rickettsial infection, first asserted by Goodman since 1916, followed by Giroud and other French investigators from the 1940s through the 1960s, Nicolau in the 1960s, Bartolo (1980s), and Fazeli (2010s). However, their causal relationship has hardly been accepted because rickettsial infections are known to be acute febrile, vector-borne illnesses, whereas BD is a chronic afebrile illness. In this article we review the relevant literature on the chronic nature of Rickettsia and Orientia infections and on the rickettsial etiology of BD. Excellent initial responses to doxycycline in three patients with BD are briefly described. Based on these findings, we hypothesize that BD patients acquired a rickettsial infection far before the onset of BD. Over years, the infected area expands to become a segment of the infected vessel. Subsequently, thrombus develops on the luminal surface of the infected endothelial cells, which produces the vascular obstructive manifestations of BD. Collectively, it is postulated that BD is a chronic infection with a member of the family Rickettsiaceae with superimposed thrombosis.