RelapsingA case report

E. ROSENTHAL

fever •In Cape

SA MEDICAL JOURNAL 22 MAY 1982 801

Town

Summary

A 19-year-old male member of the South AfricanDefence Force presented in Cape Town with feversoon after returning from service in northern SouthWest Africa. The typical clinical features ofrelapsing fever were confirmed by the finding ofspirochaetes on stained blood smears. A casereport and short review of a disease which has notbeen reported preViously in Cape Town ispresented.

S. Air. med. J.. 61, 801 (1982).

Relapsing fever is caused by spirochaetes of the genus Borrelia.Various strains and species have been identified but theclassification is not exact because strain and species differencesare not constant. The 0,2 - 0,5 J,lm wide and 8 - 40 J,lm longspiralled organisms I are carried by human lice and by ticks of thegenus Ornirhodorus (soft-bodied ticks).

Louse-borne disease (Borrelia recurremis and related strains)commonly occurs in epidemics and requires a human reservoir.The disease is transmined to man via the infected haemolymphof crushed hce on abraded skin or mucous membranes and notvia the bites of living lice. The disease is presently endemic innorth-east Africa, Asia and South America. 2 Epidemics occurredafter both World Wars with a death rate of 5 - 10%, althoughdeath rates of up to 40% have been reported.3.~

Tick-borne disease (B. durconi and related strains) is endemicand almost world-wide in distribution (including tropicalAfrica). The vector ticks are carried by various rodents andtransmit the organisms in their saliva when they feed. The ticksfeed at night for short periods and have a painless bite, whichaccounts for the fact that patients rarely recall having beenbmen. Transovarial spread of the spirochaetes from generationto generation of ticks obviates the need for a human or rodentreservoir. The ticks can survive for years without food, often inold uninhabited buildings, and have been known to harbourborreliae for many years. 1

•5 The case-fatality rate in tick-borne

disease is around 2%.5Tick-borne relapsing fever was first reported in South Africa

in 1912 in Zululand,6 and sporadic cases and outbreaks werenoted thereafter. By the 19405 the use oflong-acting insecticidesto eliminate ticks from human dwellings and improvement inhousing conditions7.8 resulted in fewer than 100 cases on avera"ebeing notified annually (most]y in Transvaal Blacks). By theearly 19505 these measures had virtually eradicated the disease;only 2 further cases have been notified since then, both in theearly 19705.9

Department of Medicine, 2 Military Hospital, Wynberg, epE. ROSENTHAL, .\1.8. CH. B., MR.CP

Date received: 28 August 1981.

Case report

A 19-year-old male member of the South African Defence Forcewho had been on service in northern South West Africa (SWA)returned to Cape Town and was admitted I week later with fever.He had experienced a sudden onset of fever with rigors andsweatmg, severe headache, general weakness and loss ofappetite3 days before admission and was still having the same symptoms.Malaria prophylaxis had been adequate and he could recall nobites or skin rashes. He admitted to having swum in rivers inSWA.

On admission he was flushed, with conjunctival injection anda temperature of 40,3°C. There were no other abnormalphysical findings. Laboratory tests revealed the following:haemo?lobin concentration 14,6 g/dl; white blood cell count 5,7X 10'/J,l1 (60% polymorphonuclear leucocytes, 35%Iymphocytes, 3% monocytes and 2% eosinophils); an ESR of 60mm/h; a negative malaria smear; and normal urea and electrolytelevels and hver function. Hi chest radiograph was normal.There was no growth on blood culture; the midstream urinesample showed a moderate amount of red cells but no organisms,casts or growth; and the VDRL, Paul Bunnell, Australia antigen,hepatitis A IgM, Widal, ~ eil-Felix, Brucella and Leprospiraagglutination and cercarial complement fixation tests were allnegative.

Fever, symptoms and haematuria persisted for 36 hours afteradmission and then subsided spontaneously. He was discharged3 days later without a firm diagnosis to return for follow-up Iweek later.

The patient was readmitted 6 days later complaining of thesame symptoms as well as a non-productive cough which hadbeen present for 2 days. Physical examination was as before buton this occasion he was more apathetic and had markedphotophobia as well as right upper quadrant tenderness. Urin­alysis again revealed a trace of haematuria. Blood smears wereexamined for the presence of spirochaetes and these were foundon both thick and thin smears (Fig. I).

Treatment with procaine penicillin 1,2 X 106 Uintramuscularly was followed by a drop in temperature within 2hours and he remained apyrexial thereafter. Treatment was

Fig. 1. Peripheral blood smear showing a spirochaete (Wright's stain X2800).

802 SA MEDIESE TYDSKRIF 22 MEI 1982

continued with oral penicillin for 5 days. The day after initiationof treatment (when he was already apyrexial) he developed a red,painful eye with miosis; the diagnosis of anterior uveitis wasconfirmed on slit-lamp examination. This responded well totopical steroid and mydriatic therapy.

Tiredness and weakness persisted for a few days but this soonresolved and when seen again 2 weeks later he was completelywell and had suffered no further relapses. Once he becameafebrile the haematuria subsided and an intravenous pyelogramwas normal.

Discussion

The symptoms of relapsing fever are nonspecific and includefever of sudden onset with rigors and sweating, headache, eyepain (often with photophobia), myalgia and lethargy. Othersymptoms which may be present are coughing and dyspnoea,abdominal pain, nausea, vomiting, diarrhoea and epistaxis.

Physical findings are also nonspecific, fever, apathy, flushedfacies and iniected conjunctivae being the commonest.Splenomegaly (often with tenderness), hepatomegaly, and lessoften jaundice and even lymphadenopathy, may also be present.A rash (usually erythematous macules but sometimes evenpetechial or purpuric3) occurs in about 25% of patients, usuallyduring or after the first febrile period, and lasts 24 - 48 hours. 5

Microscopic haematuria is a fairly common finding. 2

The only specific clinical feature is the panern of the fever.After an incubation periof of 4-18 days there is a sudden onset offever (mean temperature now being 39-40°C), which lasts anaverage of 3 - 5 days, running a reminent course. Thisterminates, often owing to a crisis, with a sudden drop intemperature, chills, and sweating and leaves the patient feelingweak. After an afebrile interval of 4-10 days there is a suddenrelapse with recurrence of fever and symptoms. Louse-bornedisease is usually associated with only up to 3 or 4 relapseswhereas in tick-borne disease there may be as many as 13relapses, usually with milder crises.5

Febrile episodes are initiated by proliferation of the organismsand are terminated by the host developing antibodies which helpin destroying most of the organisms. The organisms that survivechange their antigenic structure during the afebrile period and inthe absence of host immunity can then proliferate, resulting in arelapse with rerum offever and symptoms. Eventually immunityis developed to all antigenic forms and full recovery ensues. 1

Complications are usually related to the severity ofthe diseaseand occur commonly in louse-borne disease, especially in thesening of lowered host immunity. Neurological complications(which may occur in up to 30% of cases of louse-borne diseaseand 9% of cases of tick-borne disease) include meningism,meningitis and transient focal defects. 5 Bronchitis, broncho­pneumonia and lobar pneumonia may occur, and evencardiac failure and endocarditis, 10 although these occur only veryrarely. Haemorrhagic manifestations varying in severity may bepresent, and are related to a lowered prothrombin index due tohepatic involvement. 10 Iritis or iridocyclitis occurs in up to 15%of cases of tick-borne disease, usually after the second or thirdpyrexial episode.5 The outcome appears to be unrelated tofurther relapses, leading to visual deterioration in severe cases.Diagnosis should be considered when a febrile illness, especiallyone involving relapses occurs in a traveller who has visted an

..endemic area. During the fever the spirochaetes will show up onstained peripheral blood smears in 70% ofpatients. 5Borreliae arethe only spirochaetes stained by aniline dyes and are found mosteasily on thick smears. Inoculation of blood into rodents willincrease the diagnostic yield by about 15%. A further method isto examine suspected ticks for the organism I1 - this, however, isusually limited to epidemiological ~ork.The spirochaetes mayalso be found (rarely) m cerebrospmal flUId and urine.

Agglutinins to Proreus OXK occur in 90% of cases of louse­borne disease and in 30% ofcases of tick-borne disease, and false­positive serological tests for syphilis are present in about 5%.5Routine haematological and biochemical investigations showvariable and nonspecific changes. Specific serological tests(including a complement fixation test l2) exist but are not widelyavailable.

Antibiotics used in treatment have included penicillin,streptomycin, tetracycline, chloramphenicol and erythromycin,varying from single-dose therapy to 5-1O-day courses (berhorally and parenterally). Initiation of therapy may precipitate aJarisch-Herxheimer reaction - an exaggeration of the naturalcrisis related to rapid destruction of the organisms - which mayresult in severe circulatory collapse and even death. This severereaction has usually occurred in louse-borne disease but may alsooccur in tick-borne disease. 13 Therapy is aimed at effecting agradual clearance ofspirochaetes from the blood without causinga severe Jarisch-Herxheimer reaction and preventing furtherrelapses.

It has recently been shown that single doses oftetracycline 500mg, erythromycin 500 mg or doxycycline 100 mg orally, orprocaine penicillin 6 X l05 U intramuscularly are adequate toclear the blood of spirochaetes. 13

,14 Oral tetracyclines anderythromycin are often followed by a severe Jarisch-Herxheimerreaction whereas penicillin has a more gradual onset of actionwith a less severe reaction, although there is a slightly higherrelapse rate. It is therefore suggested that therapy should beginwith single dose of intramuscular penicillin and be followed by a5-day course of tetracycline (or erythromycin in pregnancy) toprevent relapses. 15

In conclusion, this is an uncommon disease but one in whichawareness may well help avoid a diagnostic dilemma and obviatethe fairly considerable morbidity which may occur in this easilytreatable condition.

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