Abstract

Louse-borne relapsing fever (LBRF) is a classical epidemic disease, which in the past was associated with war, famine, poverty, forced migration, and crowding under poor hygienic conditions around the world. The disease’s causative pathogen, the spirochete bacterium Borrelia recurrentis, is confined to humans and transmitted by a single vector, the human body louse Pediculus humanus corporis. Since the disease was at its peak before the days of modern medicine, many of its aspects have never been formally studied and to date remain incompletely understood. In order to shed light on some of these aspects, we have systematically reviewed the accessible literature on LBRF since the recognition of its mode of transmission in 1907, and summarized the existing data on mortality, Jarisch–Herxheimer reaction (JHR), and impact on pregnancy.Publications were identified by using a predefined search strategy of electronic databases and a subsequent review of the reference lists of the obtained publications. All publications reporting patients with a confirmed diagnosis of LBRF published in English, French, German, and Spanish since 1907 were included. Data extraction followed a predefined protocol and included a grading system to judge the certainty of the diagnosis of reported cases.The high mortality rates often found in literature are confined to extreme scenarios. The case fatality rate (CFR) of untreated cases is on average significantly lower than frequently assumed. In recent years, a rise in the overall CFRs is documented, for which reasons remain unknown.Lacking standardized criteria, a clear diagnostic threshold defining antibiotic treatment-induced JHR does not exist. This explains the wide range of occurrence rates found in literature. Pre-antibiotic era data suggest the existence of a JHR-like reaction also in cases treated with arsenicals and even in untreated cases.LBRF-related adverse outcomes are observed in 3 out of 4 pregnancies.

Highlights

  • Relapsing fevers are potentially fatal ectoparasite-borne diseases which are caused by spirochetes of the genus Borrelia and characterized by recurring episodes of fever [1].Relapsing fevers are classified according to their transmitting vector which include tickborne relapsing fever (TBRF) and louse-borne relapsing fever (LBRF)

  • In order to shed light on some of these aspects, we have systematically reviewed the accessible literature on LBRF since the recognition of its mode of transmission in 1907, and summarized the existing data on mortality, Jarisch–Herxheimer reaction (JHR), and impact on pregnancy

  • The disease has fallen into oblivion, and reports of the disease are largely confined to Ethiopia

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Summary

Introduction

Relapsing fevers are potentially fatal ectoparasite-borne diseases which are caused by spirochetes of the genus Borrelia and characterized by recurring episodes of fever [1].Relapsing fevers are classified according to their transmitting vector which include tickborne relapsing fever (TBRF) and louse-borne relapsing fever (LBRF). The incubation period of LBRF ranges from 4 to 18 (average 7) days. Untreated attacks resolve by crisis after 4 to 10 (average 5) days. This is followed by an afebrile remission of 5 to 9 days and succeeded by up to 5 relapses of diminishing severity, during which there may be epistaxis, but no petechial rashes [3]. The crisis which abruptly terminates untreated attacks is a consequence of specific bactericidal antibodies lysing spirochetes in the blood. Once antibodies have been generated against a specific vmp variant, a new vmp variant is expressed by the Borrelia. After the removal of the majority population through antibodies, the minority variant population expands until antibodies are generated against the new vmp variant. Linked to vmp is the phenomenon of Jarisch–Herxheimer reaction (JHR) [15]

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