Epidemic typhus is the most severe form of rickettsial infections. This louse-borne typhus is an acute febrile illness that features a headache, rash, myalgia, vasculitis, and is complicated by severe central nervous system (CNS) manifestations, gangrene, and high rates of death in untreated individuals. It is diagnosed by a thorough history and evaluation of risk factors, clinical exam, and laboratory studies.
Presentation
Epidemic typhus (ET) is caused by Rickettsia prowazekii, which is transmitted by human body louse [1] [2]. As a part of the typhus group, it is described as an acute febrile illness. Significant outbreaks have occurred during the Napoleonic Wars and World War I and II, resulting in the death of millions [3] [4]. More recent epidemics emerged in Algeria, Burundi, and Russia [5] [6] [7]. Regions that have been affected include the parts of Africa, China, the Himalayas, and Central and South America. Risk factors encompass war, natural disaster, overcrowding, poor hygiene, and cold weather. Additionally, military personnel and medical relief workers are considered vulnerable populations.
The clinical presentation is characterized by nonspecific symptoms such as a high fever, intense headache, myalgia, and a rash [8]. The latter erupts approximately 5 to 7 days after the onset of the disease [8]. Additionally, ET may cause pneumonia, multisystemic vasculitis, and CNS manifestations such as mental status changes, encephalitis or coma. Other features are malaise, rigors, and sudden prostration.
Complications
ET can give rise to gangrene infection and the resultant loss of digits, extremities and other body parts. Also, patients may develop hypotension with vascular collapse, renal failure, and other severe sequelae. If left untreated, epidemic typhus leads to death in up to 30% of cases [4] [8] [9]. Note that nearly 15% of victims develop the Brill-Zinsser disease, which is a recrudescent form that may recur years later.
Physical exam
Findings are notable for a high fever that reaches 39-41ºC and remains elevated if untreated. Also remarkable is the rash, which appears as maculopapular or petechial. It manifests centrally and spreads to the extremities while sparing the face, palms, and soles. Other signs may comprise of splenomegaly and bruises.
Entire Body System
- Fever
From Wikidata Jump to navigation Jump to search Human disease epidemic louse-borne typhus louse-borne typhus jail fever hospital fever ship fever famine fever putrid fever camp fever petechial fever exanthematic Typhus fever NOS sylvatic typhus Exanthematous [wikidata.org]
One of the first of these was an epidemic of typhus and relapsing fever which started in Serbia at the end of 1914 and killed upwards of 150,000 people in a population of around four and a half million. [blogs.bl.uk]
Hearing loss as a frequent complication of louse-borne epidemic typhus fever has been well documented in the reports of ENT specialists serving in both the Allied and the German armies in the last war. [ncbi.nlm.nih.gov]
Typhus fever was also a significant killer during the American Civil War, although typhoid fever was the more prevalent cause of US Civil War "camp fever." Typhoid is a completely different disease from typhus. [en.wikipedia.org]
- Chills
All had been counselors at the camp and had experienced febrile illness with myalgia, chills, and sweats; 2 had been hospitalized. [ncbi.nlm.nih.gov]
Symptoms include chills, headache and fever, lasting about 12 days. Rash and other manifestations are similar to epidemic typhus. [thirdworldtraveler.com]
Nature of the disease The onset is variable but often sudden, with headache, chills, high fever, prostration, coughing and severe muscular pain. [who.int]
Malaise or general weakness occurs 1 to 3 days before the onset of sudden high fever, headache, chills, cough, muscle pain, sensitivity to light, and lack of energy. [iamat.org]
Fuji p re sents to the emergency clinic with "the worst headache of his life," accompanied by fevers, chills, malaise, photophobia, and a macular/papular rash spread across his trunk, face, and upper extremities. [medbullets.com]
- Pain
Symptoms are chills, fever, dry cough, and pain in the affected side (a stitch). [sedgleymanor.com]
Symptoms of epidemic typhus may include chills, cough, high fever (104 degrees Fahrenheit), delirium or stupor, joint pain, sensitivity to light, severe headache, severe muscle pain, low blood pressure, and a rash that begins on the chest and spreads [epi.publichealth.nc.gov]
[…] and diarrhoea joint and muscle pain – backache is common a cough In addition, the person with typhus is often mentally dazed or delirious – the word "typhus" comes from the Greek word meaning "a cloud". [beaconmedicalgroup.nhs.uk]
There is often a large painful sore at the site of the bite and nearby lymph nodes are swollen and painful. Epidemic typhus (European, Classic, Louse-borne) Epidemic typhus is prevalent worldwide. [thirdworldtraveler.com]
- High Fever
Physical exam Findings are notable for a high fever that reaches 39-41ºC and remains elevated if untreated. Also remarkable is the rash, which appears as maculopapular or petechial. [symptoma.com]
Nature of the disease The onset is variable but often sudden, with headache, chills, high fever, prostration, coughing and severe muscular pain. [who.int]
Epidemic typhus is characterized clinically by sudden onset, sustained high fever of about 2 weeks duration, a maculopapular rash, and altered mental state. There is effective antimicrobial therapy for the typhus fevers. [asmscience.org]
Malaise or general weakness occurs 1 to 3 days before the onset of sudden high fever, headache, chills, cough, muscle pain, sensitivity to light, and lack of energy. [iamat.org]
Read: Everything you need to know about head lice - Symptoms of this disease include chills, confusion, a cough, high fever, joint pain, sensitive eyes, a rash and a severe headache. - Blood tests for typhus may show a high level of typhus antibodies, [health24.com]
- Malaise
Other features are malaise, rigors, and sudden prostration. Complications ET can give rise to gangrene infection and the resultant loss of digits, extremities and other body parts. [symptoma.com]
Signs and symptoms include sudden headache, generalized muscle pain, malaise, and macular skin lesions. The infection may affect the central nervous system causing encephalitis. [fpnotebook.com]
Malaise or general weakness occurs 1 to 3 days before the onset of sudden high fever, headache, chills, cough, muscle pain, sensitivity to light, and lack of energy. [iamat.org]
Fever, malaise, head and muscle ache, cough, and general weakness. Blotchy rash that spreads from the abdomen to chest to rest of body, but rarely to hands and feet. Severe disease includes prostration, delirium, very low blood pressure, and coma. [extension.entm.purdue.edu]
Fuji p re sents to the emergency clinic with "the worst headache of his life," accompanied by fevers, chills, malaise, photophobia, and a macular/papular rash spread across his trunk, face, and upper extremities. [medbullets.com]
Respiratoric
- Dry Cough
[…] arthalgia myalgia photophobia delirium murine or endemic typhus abdominal pain diarrhea backache headache extremely high fever (105-106 F) may last for up to two weeks dull, red rash begins on the trunk and spreads peripherally hacking, dry cough arthralgia [medbullets.com]
Symptoms are chills, fever, dry cough, and pain in the affected side (a stitch). [sedgleymanor.com]
Gastrointestinal
- Diarrhea
Other symptoms may include nausea, diarrhea, and constipation. If untreated, the infection can lead to gangrene and necrosis (death of body tissue). [iamat.org]
[…] including sudden-onset fever (104 F) chills severe headache maculopapular rash appears 5-9 days after onset of symptoms spreads peripherally from trunk to extremities cough arthalgia myalgia photophobia delirium murine or endemic typhus abdominal pain diarrhea [medbullets.com]
- Loss of Appetite
About 10 days after being bitten, an infected person experiences headache, loss of appetite, malaise, and a rapid rise in temperature with fever, chills, marked prostration, and nausea. [britannica.com]
Cardiovascular
- Hypotension
Also, patients may develop hypotension with vascular collapse, renal failure, and other severe sequelae. If left untreated, epidemic typhus leads to death in up to 30% of cases. [symptoma.com]
Hypotension occurs in most seriously ill patients. Vascular collapse, renal insufficiency, encephalitic signs, ecchymosis with gangrene, and pneumonia are poor prognostic signs. [msdmanuals.com]
The symptoms of Epidemic Typhus or Brill-Zinsser Disease include severe headache, persistent high fever, severe cough, rashes, excruciating muscle pain, chills, hypotension, photosensitivity, delirium and ultimately death. [epainassist.com]
- Tachycardia
[…] local arteries with a consecutive softening of the brain), extrapyramidal syndrome (disappears with fever decrease and is due to vascular disorders of the basal nucleus), cerebellar syndrome, bulbo-protuberantial syndrome with bulbar phenomena (dyspnea, tachycardia [ncbi.nlm.nih.gov]
During the serious course of the epidemic typhus fever expressive intoxication, hypotonia, tachycardia ( till 140 in minute) are observed. The tones of the heart are deaf. There is acrocyanosis. [intranet.tdmu.edu.ua]
Musculoskeletal
- Myalgia
The clinical presentation is characterized by nonspecific symptoms such as a high fever, intense headache, myalgia, and a rash. The latter erupts approximately 5 to 7 days after the onset of the disease. [symptoma.com]
Headaches, fever, myalgias, and exanthems were among the presenting complaints. The disease seemed milder than classic louse-born epidemic typhus, but in some instances, it was life-threatening. [ncbi.nlm.nih.gov]
Clinically, patients have presented with fever (100%), headache (81%), skin rash (66%), confusion (44%), and myalgia (42%). The skin rash has been characterized as maculopapular, usually involving the trunk and spreading to the extremities. [cdc.gov]
Skin
- Skin Rash
Clinically, patients have presented with fever (100%), headache (81%), skin rash (66%), confusion (44%), and myalgia (42%). The skin rash has been characterized as maculopapular, usually involving the trunk and spreading to the extremities. [cdc.gov]
They are responsible for a variety of insect-borne human diseases characterized by acute onset, fever, delirium and skin rash. Rickettsia prowazekii, the causative agent of louse-borne epidemic typhus, is named after H. T. Ricketts and S. J. M. [nature.com]
Four days after the onset of symptoms she had developed a generalized maculopapular skin rash. [jcm.asm.org]
- Purpura
Puking Fever Milk sickness Pulmonary Consumption Probably Tuberculosis Purples/Purpura This is a rash due to spontaneous bleeding in to the skin. There are many causes. [sedgleymanor.com]
The gray matter is smoky gray while the white matter is speckled with punctiform hemorrhages (cerebral purpura). [intranet.tdmu.edu.ua]
- Petechiae
[…] rash begins on the trunk and spreads peripherally hacking, dry cough arthralgia myalgia nausea vomiting Physical exam hypotension fever photophobia early rash light rose color and blanches under pressure late rash dull red that does not fade scattered petechiae [medbullets.com]
Urogenital
- Kidney Failure
If typhus is not diagnosed and treated promptly, there is a risk of developing complications, including: long-term hearing loss or tinnitus low blood pressure organ damage and kidney failure secondary bacterial infection, such as pneumonia seizures confusion [beaconmedicalgroup.nhs.uk]
failure Differential Rocky mountain spotted fever, Lyme disease Treatment Medical management antibiotic therapy indicated in all types of typhus disease doxycycline tetracycline may permanently stain developing teeth usually not prescribed in children [medbullets.com]
Signs of pneumonia or kidney failure are common. Prostration is progressive, and delirium and coma follow; cardiac failure may be the immediate cause of death. [britannica.com]
Neurologic
- Headache
The clinical presentation is characterized by nonspecific symptoms such as a high fever, intense headache, myalgia, and a rash. The latter erupts approximately 5 to 7 days after the onset of the disease. [symptoma.com]
Symptoms are prolonged high fever, intractable headache, and a maculopapular rash. Epidemic typhus is a rickettsial disease. [msdmanuals.com]
Headaches, fever, myalgias, and exanthems were among the presenting complaints. The disease seemed milder than classic louse-born epidemic typhus, but in some instances, it was life-threatening. [ncbi.nlm.nih.gov]
Clinically, patients have presented with fever (100%), headache (81%), skin rash (66%), confusion (44%), and myalgia (42%). The skin rash has been characterized as maculopapular, usually involving the trunk and spreading to the extremities. [cdc.gov]
- Confusion
Read: Everything you need to know about head lice - Symptoms of this disease include chills, confusion, a cough, high fever, joint pain, sensitive eyes, a rash and a severe headache. - Blood tests for typhus may show a high level of typhus antibodies, [health24.com]
Clinically, patients have presented with fever (100%), headache (81%), skin rash (66%), confusion (44%), and myalgia (42%). The skin rash has been characterized as maculopapular, usually involving the trunk and spreading to the extremities. [cdc.gov]
Central nervous system involvement may include confusion, delirium, coma, transverse myelitis, and cranial nerve palsies. Tinnitus and deafness have been reported. Splenomegaly is common. [visualdx.com]
- Stupor
Symptoms of epidemic typhus may include chills, cough, high fever (104 degrees Fahrenheit), delirium or stupor, joint pain, sensitivity to light, severe headache, severe muscle pain, low blood pressure, and a rash that begins on the chest and spreads [epi.publichealth.nc.gov]
The word typhus (from the Greek typhos, meaning smoky or hazy, and used by Hippocratesto describe a “confused state of intellect with a tendency to stupor”) was not applied to typhus fever itself until 1760; moreover, typhus was not clearly distinguished [nature.com]
'Typhus' in Greek literally means 'stuporous' after this classic symptom. After several days of these symptoms, the patient will develop a rash that covers nearly the entire body. [study.com]
- Meningism
[…] s) Bacterial Diseases Animal Diseases Fiche 2, Les abréviations, Anglais Fiche 2, Justifications, Anglais Record number: 2, Textual support number: 1 DEF A febrile disease typically characterized by a skin eruption, such as typhus, epidemic cerebral meningitis [btb.termiumplus.gc.ca]
During the period of onset of the diseasethe differential diagnosis is performed with grippe, pneumonia, meningitis, hemorrhagic fevers. [intranet.tdmu.edu.ua]
Epidemic typhus meningitis in the southwestern United States. Clin. Infect. Dis. 32 : 979 -982. ↵ McDade, J. E., C. C. Shepard, M. A. Redus, V. F. Newhouse, and J. D. Smith. 1980. Evidence of Rickettsia prowazekii infections in the United States. [jcm.asm.org]
Tâche Cérébrale Red line on the forehead seen in tuberculous meningitis Teething The entire process which results in the eruption of the teeth. [sedgleymanor.com]
Differential diagnosis includes malaria, typhoid fever, viral haemorrhagic fever, leptospirosis, endemic typhus, tick-borne and louse-borne relapsing fevers, non-typhoidal salmonellosis, meningococcal septicaemia and meningitis. [ecdc.europa.eu]
Workup
During the assessment, the clinician should elicit the risk factors and patient history, and perform a careful physical exam and the appropriate tests. Note the administration of antibiotics should be started as soon as the provisional diagnosis for this diseases is established.
Laboratory tests
Generally, laboratory studies such as complete blood count (CBC) and comprehensive metabolic panel (CMP) consisting of liver and renal function tests, electrolyte levels, serum albumin concentration, and others should be obtained. Common findings may include but are not limited to leukopenia, leukocytosis, thrombocytopenia, mild increase in transaminases, hyponatremia, hypoalbuminemia, and azotemia.
The diagnostic panel consists of indirect immunofluorescence assay (IFA) and enzyme immunoassay (EIA), which are used to measure immunoglobulin (Ig) M antibodies. Serological testing is not useful in acute cases since the immune response is not demonstrable during this period. Moreover, polymerase chain reaction (PCR) performed on blood or skin biopsy samples suggests Rickettsia and the specific species of this pathogen [10]. Culture is not helpful.
Brill-Zinsser disease is identified in patients with recurrent manifestations and a measurable elevation of IgG titer.
Imaging
A chest X-ray should be obtained in those with pneumonia [11].
Serum
- Hyponatremia
Common findings may include but are not limited to leukopenia, leukocytosis, thrombocytopenia, mild increase in transaminases, hyponatremia, hypoalbuminemia, and azotemia. [symptoma.com]
[…] late rash dull red that does not fade scattered petechiae may be observed in patients with severe disease Evaluation Labs CBC may show anemia and thrombocytopenia typhus antibody present in individuals with active or previous infection hypoalbuminemia hyponatremia [medbullets.com]
Microbiology
- Rickettsia Prowazekii
exanthematisch; vlektyfus, klassiek; vlektyfus, luizen; tabardillo, luizen; vlektyfus, prowazekii; Rickettsia prowazekii, vlektyfus, rickettsiose; Rickettsia prowazekii, tabardillo; door luizen overgebracht, vlektyfus; Rickettsia prowazekii, vlektyfus [fpnotebook.com]
Abstract: Epidemic typhus caused by Rickettsia prowazekii is one of the oldest pestilential diseases of humankind. [asmscience.org]
Epidemic typhus is another life-threatening louse-borne disease caused by Rickettsia prowazekii and known to occur in conditions of war, famine, refugee camps, cold weather, poverty, or lapses in public health. [ncbi.nlm.nih.gov]
Treatment
Treatment of this disease with oral doxycycline, tetracycline, or chloramphenicol prevents complications and results in prompt resolution of symptoms. [ncbi.nlm.nih.gov]
Doxycycline Primary treatment of epidemic typhus is doxycycline 200 mg orally once followed by 100 mg twice a day in adults until the patient improves, has been afebrile for 24 to 48 hours, and has received treatment for at least 7 days. [msdmanuals.com]
Links: clinical features treatment General Practice Notebook General Practice Notebook The information provided herein should not be used for diagnosis or treatment of any medical condition. [gpnotebook.co.uk]
After the patient is stabilized then the treatment with antibiotics is started. [epainassist.com]
Prognosis
Prevention, and Comlications Prognosis epidemic typhus can be fatal is left untreated in 10-60% of patients caused by peripheral vascular collapse elderly patients are at greater risk murine typhus is fatal in less than 2% of patients if left untreated [medbullets.com]
Scrub Typhus and Murine Typhus, Vietnam, 2015-2017. ( 30882318 ) Trung NV...Van Kinh N 2019 18 Evaluation of Th1 and Th2 immune response in clinical and sub-clinical scrub typhus infection. ( 30904436 ) Bora T...Khan SA 2019 19 Indicators of severe prognosis [malacards.org]
Prognosis ,Prophylaxis and treatment Starting antibiotics early has significantly reduced mortality from about 20 to 7%. Early treatment prevents most complications. No effective vaccines are available. [the-travel-doctor.com]
Suppurative parotiditis claims an unfavorable prognosis and require surgical treatment as early as possible [ 24, 31 ]. [ncbi.nlm.nih.gov]
Etiology
Differences in IFA titers against R. typhi and R. prowazekii allowed the identification of the etiological agent in 8 of 12 patients. Western blot studies enabled the identification of the etiological agent in six patients. [ncbi.nlm.nih.gov]
The patient had contracted the disease in Mexico. 1 The Centers' Virology Division of the Bureau of Laboratories described case histories of eight persons who had serologic reactions that indicated recent infection with Rickettsia prowazekii, the etiologic [nejm.org]
[…] emphasize that: (1) the present diagnosistic criteria must be revised; (2) the reported incidence of Brill's disease may be expected to increase; (3) Brill's disease may indeed be important in initiating new epidemics of louse-borne typhus, and (4) the etiologic [jamanetwork.com]
On the basis ofinteractions of host endothelial cells with R. prowazekii and R. typhi (the etiologic agent of endemic typhus), wehave identified activation of nuclear factor kappa B (NF- B) and stress-activated p38 protein kinase as criticallyimportant [grantome.com]
Epidemiology
Abstract Differentiation of murine typhus due to Rickettsia typhi and epidemic typhus due to Rickettsia prowazekii is critical epidemiologically but difficult serologically. [ncbi.nlm.nih.gov]
These 30 patients illustrate the clinical, epidemiologic and serologic characteristics of primary epidemic typhus. 2. [jimmunol.org]
Pathophysiology
Pathophysiology : The incubation period from the mite bite is 6-18 days following inoculation. Humans acquire the disease when an infected mite or chigger, bites them while feeding and inoculates R tsutsugamushi pathogens. [the-travel-doctor.com]
Prevention
These sources reveal why typhus was such a feared disease even after it was found to be preventable; in 1909 it was discovered that the disease was caused by body lice, although the cure was not known until after the war. Cambridge USA, 1920. [blogs.bl.uk]
Centers for Disease Control and Prevention, Kigali, Rwanda. 10 National Center for Emerging and Zoonotic Infectious Diseases, U.S. Centers for Disease Control and Prevention, Atlanta, Georgia. 11 University of British Columbia, Vancouver, Canada. [ncbi.nlm.nih.gov]
There is no safe vaccine for preventing epidemic typhus. The long term goal of this project is to understand the pathogenesis of rickettsial diseases and to prevent epidemic typhus. [grantome.com]
References
- Svraka S, Rolain JM, Bechah Y, Gatabazi J, Raoult D. Rickettsia prowazekii and Real-time Polymerase Chain Reaction. Emerg Infect Dis. 2006;12(3):428-432.
- Azizi MH, Bahadori M, Azizi F. An Overview of Epidemic Typhus in the World and Iran during the 19th and 20th Centuries. Arch Iran Med. 2016;19(10):747-750.
- Raoult D, Dutour O, Houhamdi L, et al. Evidence for louse-transmitted diseases in soldiers of Napoleon's Grand Army in Vilnius. J Infect Dis. 2006;193(1):112-20.
- Raoult D, Roux V. The body louse as a vector of reemerging human diseases. Clin Infect Dis. 1999;29(4):888-911.
- Mokrani K, Fournier PE, Dalichaouche M, et al. Reemerging threat of epidemic typhus in Algeria. J Clin Microbiol. 2004;42(8):3898-900.
- Raoult D, Ndihokubwayo JB, Tissot-Dupont H, et al. Outbreak of epidemic typhus associated with trench fever in Burundi. Lancet. 1998;352(9125):353-8.
- Tarasevich I, Rydkina E, Raoult D. Epidemic typhus in Russia. Lancet. 1998;352(9134):1151.
- Raoult D, Roux V. Rickettsioses as paradigms of new or emerging infectious diseases. Clin Microbiol Rev. 1997;10(4):694-719.
- Bechah Y, Capo C, Mege JL, Raoult D. Epidemic typhus. Lancet Infect Dis. 2008; 8(7):417- 426.
- Giulieri S, Jaton K, Cometta A, Trellu LT, Greub G. Development of a duplex real-time PCR for the detection of Rickettsia spp. and typhus group rickettsia in clinical samples. FEMS Immunol Med Microbiol. 2012;64(1):92-7.
- Chen HC, Chang HC, Chang YC, et al. Chest radiographic presentation in patients with scrub typhus. Trans R Soc Trop Med Hyg. 2012;106(1):48-53.