Typhus

Typhus
Rash caused by epidemic typhus
Classification and external resources
Specialty	Infectious disease
ICD-10	A75
ICD-9-CM	080–083
DiseasesDB	29240
MedlinePlus	001363
eMedicine	med/2332
Patient UK	Typhus
MeSH	D014438

Typhus is any of several similar diseases caused by Rickettsia bacteria.^[1] The name comes from the Greek typhos (τύφος) meaning smoky or hazy, describing the state of mind of those affected with typhus. The causative organism Rickettsia is an obligate intracellular parasitic bacterium that cannot survive for long outside living cells. It is transmitted to humans via external parasites such as lice, fleas and ticks. While "typhoid" means "typhus-like", typhus and typhoid fever are distinct diseases caused by different genera of bacteria.

Signs and symptoms

The following signs/symptoms refer to epidemic typhus as it is the most important of the typhus group of diseases.^[2]

Signs and symptoms begin with sudden onset of fever, chills, headache, and other flu-like symptoms about 1–3 weeks after being infected. Five to nine days after the symptoms have started, a rash typically begins on the trunk and spreads out to the extremities. This rash will eventually spread over the entire body, sparing the face, palms, and soles. Signs of meningoencephalitis begin with the rash and continue into the 2nd or 3rd weeks. Other signs of meningoencephalitis include sensitivity to light (photophobia), altered mental status (delirium), or coma. If left untreated, one may eventually die.^[2]

Causes

Multiple diseases include the word "typhus" in their description.^[3] Types include:

Prevention

The most effective way to prevent typhus is inoculation with the typhus vaccine series before travelling to endemic areas, and to avoid contact with lice.

Treatment

Without treatment, death may occur in 10 to 60 percent of patients with epidemic typhus, with patients over age 60 having the highest risk of death. In the antibiotic era, death is uncommon if doxycycline is given. In one study of 60 hospitalized patients with Epidemic Typhus, no patient died when given doxycycline or chloramphenicol.^[7] Some patients also may need oxygen and intravenous (IV) fluids

Epidemiology

According to the World Health Organization, the current death rate from typhus is about one out of every 5,000,000 people per year.^[8]

Only a few areas of epidemic typhus exist today. Since the late 20th century, cases have been reported in Burundi, Rwanda, Ethiopia, Algeria, and a few areas in South and Central America.^{[9][10][11][12]}

Except for two cases, in the United States almost all cases of epidemic typhus have occurred east of the Mississippi River. An examination of a cluster of cases that occurred in Pennsylvania concluded that the source of the infection was flying squirrels.^[13] Sylvatic cycle (diseases transmitted from wild animals) epidemic typhus remains uncommon in the US. The Centers for Disease Control and Prevention have documented only 47 cases from 1976 to 2010.^[14]

History

Middle Ages

Civilian Public Service worker distributes rat poison for typhus control in Gulfport, Mississippi, c. 1945.

The first reliable description of the disease appears during the Spanish siege of Moorish Granada in 1489. These accounts include descriptions of fever and red spots over arms, back, and chest, attention deficit, progressing to delirium, gangrenous sores, and the stink of rotting flesh. During the siege, the Spaniards lost 3,000 men to enemy action, but an additional 17,000 died of typhus.

Typhus was also common in prisons, where it was known as 'Aryotitus fever.' It often occurred when prisoners were crowded together into dark, filthy rooms where lice spread easily. "Imprisonment until the next term of court" was often equivalent to a death sentence. Prisoners brought before the court sometimes infected members of the court itself.^[15] Following the assize held at Oxford in 1577, later deemed the Black Assize, over 300 died from epidemic typhus, including Sir Robert Bell, Lord Chief Baron of the Exchequer. During the Lent assize court held at Taunton (1730), typhus caused the death of the Lord Chief Baron, as well as the High Sheriff, the sergeant, and hundreds of others. During a time when there were 241 executed for capital offenses, more prisoners died from 'gaol fever' than were put to death by all the public executioners in the British realm. In 1759, an English authority estimated that each year a quarter of the prisoners had died from gaol fever.^[15] In London, typhus frequently broke out among the ill-kept prisoners of Newgate Prison and then moved into the general city population. In May 1750, the Lord Mayor of London, Sir Samuel Pennant, and a large number of court personnel were fatally infected in the courtroom of the Old Bailey, which adjoined Newgate Prison.^[16]

A US soldier is demonstrating DDT hand-spraying equipment. DDT was used to control the spread of typhus-carrying lice.

Epidemics occurred routinely throughout Europe from the 16th to the 19th centuries, including during the English Civil War, the Thirty Years' War, and the Napoleonic Wars.^[17] Pestilence of several kinds raged among combatants and civilians in Germany and surrounding lands from 1618 to 1648. According to Joseph Patrick Byrne, "By war's end, typhus may have killed more than 10 percent of the total German population, and disease in general accounted for 90 percent of Europe's casualties."^[18]

19th century

During Napoleon's retreat from Moscow in 1812, more French soldiers died of typhus than were killed by the Russians.^[19]

A major epidemic occurred in Ireland between 1816 and 1819, during the famine caused by a world-wide reduction in temperature known as the Year Without a Summer. An estimated 100,000 Irish perished. Typhus appeared again in the late 1830s, and yet another major typhus epidemic occurred during the Great Irish Famine between 1846 and 1849. The Irish typhus spread to England, where it was sometimes called "Irish fever" and was noted for its virulence. It killed people of all social classes, as lice were endemic and inescapable, but it hit particularly hard in the lower or "unwashed" social strata.

In the United States, a typhus epidemic broke out in Philadelphia in 1837 and killed the son of Franklin Pierce (14th President of the United States) in Concord, New Hampshire in 1843. Several epidemics occurred in Baltimore, Memphis and Washington DC between 1865 and 1873. Typhus was also a significant killer during the US Civil War, although typhoid fever was the more prevalent cause of US Civil War "camp fever". Typhoid fever, caused by the bacterium Salmonella typhii (not to be confused with Salmonella enterica, the cause of Salmonella food poisoning), is a completely different disease from typhus.

In Canada alone, the typhus epidemic of 1847 killed more than 20,000 people from 1847 to 1848, mainly Irish immigrants in fever sheds and other forms of quarantine, who had contracted the disease aboard the crowded coffin ships in fleeing the Great Irish Famine. Officials did not know how to provide sufficient sanitation under conditions of the time, nor understand how the disease spread.^[20]

20th century

Charles Nicolle received the 1928 Nobel Prize in Medicine for his identification of lice as the transmitter of epidemic typhus.

Delousing stations were established for troops on the Western Front during World War I, but the disease ravaged the armies of the Eastern Front, with over 150,000 dying in Serbia alone. Fatalities were generally between 10 and 40 percent of those infected, and the disease was a major cause of death for those nursing the sick.

In 1922, the typhus epidemic reached its peak in Soviet territory, with some 25 to 30 million cases in Russia. Although typhus had ravaged Poland with some 4 million cases reported, efforts to stem the spread of disease in that country had largely succeeded by 1921 through the efforts of public health pioneers such as Hélène Sparrow and Rudolf Weigl.^[21] In Russia, during the civil war between the White and Red armies, typhus killed 3 million people,^[22][23] mainly civilians. During World War II, many German POWs after the loss at Stalingrad died of typhus. Typhus epidemics killed those confined to POW camps, ghettos, and Nazi concentration camps who were held in unhygienic conditions. Pictures of typhus victims' mass graves can be seen in footage shot at Bergen-Belsen concentration camp.^[24]

Among thousands of prisoners in concentration camps such as Theresienstadt and Bergen-Belsen who died of typhus^[24] were Anne Frank, age 15, and her sister Margot, age 19. Major epidemics in the postwar chaos of Europe were averted only by widespread use of the newly discovered DDT to kill the lice on millions of refugees and displaced persons.

The first typhus vaccine was developed by the Polish zoologist Rudolf Weigl in the period between the two world wars.^[25] Better, less-dangerous and less-expensive vaccines were developed during World War II. Since then, some epidemics have occurred in Asia, Eastern Europe, the Middle East, and parts of Africa.

References