Rheumatic Fever In The Medical Casebook

 

This Week

This week we are going to take a look at rheumatic fever. I have never seen a case, though I have a few elderly patients who have heart valve problems as a result of catching rheumatic fever as a child.

 

Rheumatic Fever

Rheumatic Fever + Sherlock

Rheumatic fever makes an appearance in the canon in “The Adventure Of The Lion’s Mane”. We had our main look at this story when we looked at Jellyfish Stings.

“Fitzroy McPherson was the science master, a fine upstanding young fellow whose life had been crippled by heart trouble following rheumatic fever. He was a natural athlete, however, and excelled in every game which did not throw too great a strain upon him. Summer and winter he went for his swim, and, as I am a swimmer myself, I have often joined him.”

You will remember that the wonderfully named Fitzroy Macpherson is the first victim of the fatal jellyfish sting. Conan Doyle is maybe using the damage caused by rheumatic fever as a reason for McPherson to be indulging in the then unconventional, but now very trendy, sport of open-water swimming. It is also a possible reason why he died from the jellyfish sting.

One of the manifestations of rheumatic fever, St Vitus’s dance, also makes 2 canonical appearances. 

Firstly in “The Stockbroker’s Clerk”, we hear how Dr Watson sets himself up for the first time in his own general practice.

Shortly after my marriage I had brought a connection in the Paddington district. Old Mr Farquhar, from whom I purchased it, had at one time an excellent general practice, but his age, and an affliction of the nature of St Vitus’s dance from which he suffered, had very much thinned it. The public not unnaturally goes on the principle that he who would heal others must himself be whole, and looks askance at the curative powers of the man whose own case is beyond the reach of his drugs.

Secondly in “The Greek Interpreter,” the interpreter of the title, Mr Melas, details his meeting with the imprisoned Paul Kratides.

“I cannot tell you the loathing and horror with which this insignificant-looking man inspired me. I could see him better now as the lamp-light shone upon him. His features were peaky and sallow, and his little pointed beard was thready and ill-nourished. He pushed his face forward as he spoke and his lips and eyelids were continually twitching like a man with St Vitus’s dance. I could not help thinking that his strange, catchy little laugh was also a symptom of some nervous malady. The terror of his face lay in his eyes, however, steel gray, and glistening coldly with a malignant, inexorable cruelty in their depths.”

This is our only visit to this story. Paul Kratides is kidnapped by Harold Latimer. The Kratides family is wealthy, and Paul’s sister, Sophy, has been seduced by Latimer, who then wants to get Paul to sign the family money over to him. Holmes exposes the plot with the help of Mr Melas, though too late to save Paul Kratides. The story is noteworthy as it marks the first appearance of Mycroft Holmes, older brother of Sherlock, and shadowy pillar of the British establishment.

  

Rheumatic Fever In Victorian Times

Rheumatic fever is a fascinating condition. Though I have a couple of patients who had the condition 50 or more years ago, I have never seen a fresh case. The disease has effectively died out in the UK, and we don’t entirely know why.

Early on I need to make clear that rheumatic fever is a completely different condition to rheumatoid arthritis, which we looked at in a previous chapter. The derivation of the words is very interesting. They all stem from “rheum”, which is a very old term for watery or mucous secretions. It was believed that rheumatic conditions stemmed from the abnormal production or flow of rheum. Thus our official disease terms still use a very outmoded concept of disease.

Acute rheumatism [rheumatic fever] is believed by many pathologists to arise from the presence of lactic acid in the blood. It is often hereditary, usually follows exposure to wet and cold, and is occasionally a sequence of scarlatina. [10] 

Rheumatic fever is an inflammatory condition triggered by a specific bacteria called Streptococcus pyogenes, otherwise known as Group A Streptococcus. This is one of the common causes of sore throats and tonsillitis. Previously, up to 3% of untreated infections led to the development of rheumatic fever.

Victorian medical science had a limited understanding of rheumatic fever. However, they had cleverly noted the link with scarlatina, which we now call scarlet fever. Though they had no understanding either disease was caused by a bacteria, scarlet fever is also caused by Streptococcus pyogenes. 

The affection of the joints is generally preceded for twenty-four or forty-eight hours by chilliness, languor, heat of skin, and other symptoms of fever; in some cases the heart is affected in this period. [10]

Acute rheumatism usually produces very marked swelling, with considerable effusion into the joints, and along this there is often redness of the skin, and almost always great pain, especially on disturbing their position in any way, so that the patient becomes very helpless, and dreads the least shaking in bed. The mischief in the joints appears very suddenly; it is sometimes very fleeting, shifting about from one limb to one set of joints to another, or from one side of the body to the other. [15]

We must always make a careful search for the evidence of cardiac mischief; this may exist apart from any thoracic symptoms; there is, however, usually more or less pain in the chest when pericarditis is present [15]

Chorea not unfrequently follows the complaint in young subjects, but although often obstinate, it is seldom productive of danger. [6] 

A typical presentation of rheumatic fever would occur a few weeks after a throat infection, and consisted of fever and joint pain, sometimes with a rash [erythema marginatum] and involuntary muscle twitching [Sydenham’s chorea or St Vitus’ dance]. St Vitus was a very ancient saint, possibly from the 3rd century. He is the patron saint of dancing, as historically people used to dance in front of his statue. He is also said to protect against lightning strikes and oversleeping.

Historically rheumatic fever has been a very important condition, as repeated episodes led to a range of heart diseases, including heart valve damage, pericarditis [inflammation of the lining of the heart], and endocarditis [inflammation of the heart valves]. I still have one or two elderly patients with valvular heart disease stemming from contracting rheumatic fever as children.

Various remedies have been recommended as specifics for acute rheumatism; of these salicylate of soda and salicylic acid appear to be the most certain. Large doses of nitrate of potash have been also recommended, and the cases so treated generally do well, but the unpleasantness of the medicine, and the uncertainty of its action, are great objections against it. Quinine in two or three grain doses every three hours is another favourite method of treatment. [6]

The patient is obliged, on account of the suffering produced by motion, to remain at rest. He should be kept in bed for six or seven days after the pains and fever have entirely disappeared. [6]

Formerly venesection was generally employed, but it is now abandoned; leeches are rarely required on account of the fugitive character of the inflammation of the joints. It was formerly the custom to give mercury, so as to provide salivation whenever the heart was affected, but this is now rarely employed. [6]

Whenever the temperature is high you must employ frequent cold sponging, and if the heat of the body be excessive [104 - 105°F], and especially if there also be delirium, the cold bath is required, which must be repeated as often as necessary. [6]

Salicyclic acid is still used in acute joint inflammation, though we now know it as aspirin. Quinine is also still used in modern medicine, though for muscle cramps rather than joint pain. Mercury is, of course, highly poisonous. Being repeatedly forced into a cold bath when suffering from rheumatic fever can’t have been a whole lot of fun either.

Rheumatic fever is reliably prevented by treating the triggering Streptococcal infection. This is one of the reasons why it was previously very important to treat all throat infections with a course of penicillin.

However there is then a poorly understood mystery here. Strep pyogenes is still a cause of throat infections. But we now don’t treat the majority of throat infections with antibiotics, as part of the drive to reduce antibiotic use, and thence limit the development of antibiotic resistance. Yet we are not seeing cases of rheumatic fever, and medical science is very unclear why.    

 

Aspirin research:-

“Excerpt from one of the earliest works to look systematically at the use of salicylic acid as a medical treatment.” [55]

 

Next Week

I think it will be interesting to move from rheumatic fever to rheumatism – very similar names, both deriving from the Greek word rheuma – but completely different conditions.

Then the following week, we’ll venture into the wilds of Victorian psychiatry.

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