Despite finishing my residency in 1978 and writing many papers about strep pharyngitis, I first heard of Lemierre’s syndrome in the late 1990s.  During my training, I suspect the incidence of Lemierre’s syndrome was extremely low.  As I slowly became more aware of Lemierre’s syndrome, I started to put this disease into context.

In 2002, during the early days of this blog, I wrote this rant – Some sore throats are VERY serious.

Very interesting story appears on the BBC site – Warning over killer throat disease. I have done sore throat research early in my career. This article describes a condition so unusual that I know little about it.

It follows a significant rise in the number of cases of Lemierre’s disease this year.

The disease, which is most common in young adults, can cause serious illness and even death if left untreated.

With this disease patients can go downhill quite quickly

The disease is cause by a bacterium called Fusobacterium necrophorum that normally lives harmlessly in people’s mouths.

However, for reasons unknown to scientists, it can start to attack the body of previously healthy people.

It mostly affects young people between the ages of 16 and 23 and is more common in men.

The disease is rare and affects just a handful of people each year. However, there have been 30 cases so far this year – as much as the total for all of last year.

…

“Most viral sore throats get better of their own accord in a few days but with this disease patients can go downhill quite quickly.”

Lemierre’s disease starts off as a very sore throat and leads to a fever, swollen glands and a general feeling of being unwell.

During this time my awareness of Lemierre’s syndrome continued to grow.  In 2006 my focus on this disease solidified – More on the “forgotten disease”.

For the past 30 years, the infectious disease community has worked to decrease the use of unnecessary antibiotics. They have assumed that group A beta hemolytic streptococcal infection is the only pharyngitis cause which needs “necessary antibiotics”. They have assumed that group C and group G streptococci do not need antibiotics. They have excluded the possibility of unknown bacterial infections. Now it appears that Fusobacterium necrophorum may indeed be an “unknown bacterial cause” of pharyngitis.

Kristie Estes, who daughter had Lemierre’s syndrome 3 years ago, had written me about their ordeal.  Lemierre’s syndrome started to become very personal.  I received a number of emails and comments from patients and families.  At the same time, the Europeans wrote several very important articles about Lemierre’s syndrome and Fusobacterium necrophorum pharyngitis. Fusobacterium necrophorum is the organism responsible for most cases of Lemierre’s syndrome.  As I wrote in 2006,

The problem with Lemierre’s Disease is that it represents a “long tail” disease. Most sore throats are viral or due to streptococcal disease. At least we thought that until recently. Evidence from 2005 in two articles suggests that the organism thought responsible for most Lemierre’s Disease – Fusobacterium necrophorum – may cause as much as 10% of pharyngitis.

During the next 2 years I voraciously read the appropriate literature.  I was slowly becoming an expert on this condition, despite never personally seeing a patient suffering from Lemierre’s syndrome.

Important ideas take time to mature.  I was fascinated with this syndrome, and believed that we could markedly decrease both the morbidity and mortality from this disease.  This year, an article appeared which crystallized my passion, and provided me with a cause.

Hagelskjær Kristensen L, Prag J. Lemierre’s syndrome and other disseminated Fusobacterium necrophorum infections in Denmark: a prospective epidemiological and clinical survey. European Journal of Clinical Microbiology & Infectious Diseases 2008; 779-789.

Finally we had an epidemiologic study which defined the extent of this disease.  I have extrapolated the Danish data to the United States.  Assuming the same incidence, I estimate that we have approximately 1000 patients with Lemierre’s yearly and 100 deaths.  I would put the confidence range of these estimates at 50% greater or less than this estimate.

So Lemierre’s is truly an orphan disease if you just look at the numbers.  We have some advantages though in attacking this disease.  Routine antibiotics can treat this syndrome very well.  We do not need new drugs.

As readers know, I gave my new Grand Rounds presentation on Adult Pharyngitis: Morbidity and Mortality in late July.  I am already scheduled to give this talk 4 more times (Oct and Nov.)  I hope to find venues to present this talk much more over the next year or two.

I am currently finishing an article (for submission) titled “Can we decrease the morbidity and mortality from Lemierre’s syndrome?”  Giving this Grand Rounds and writing this article have inspired me to champion this orphan disease on a national stage.  Fortunately, my name is well known in the sore throat literature, so I hope that other physicians will listen.

For my article I have developed a 5 step plan to decrease morbidity and mortality from Lemierre’s syndrome.  I will share my plan after I submit my article for publication (or perhaps sooner.)  I would love for you, the readers and thinkers, to suggest action steps for addressing Lemierre’s.  All thoughts are greatly appreciated.

I will say that the first step must be education.  I know that my blog is fairly well read, and thus some physician have learned.about Lemierre’s from reading my rants.

My 2007 perspective on adult pharyngitis –  Pharyngitis Management: Defining the Controversy  –  included this paragraph:

Another potential reason for antibiotic therapy for severe pharyngitis is to treat Fusobacterium necrophorum. Recent data suggest that these bacteria may cause endemic acute pharyngitis. F. necrophorum infections can cause Lemierre’s Disease, peritonsillar abscess and persistent sore throat symptoms. While we do not yet know the probability of progression to these complications, certainly empiric antibiotic treatment would likely decrease their incidence. A recent pediatric paper has documented the increasing incidence of F. necrophorum infections (including Lemierre’s Syndrome) over a recent 6-year period. The authors speculate that decreased empiric antibiotic use may be contributing to the resurgence of this infection.

As I enter the final decade of my full time career (I doubt that I will soon leave medicine completely), addressing Lemierre’s syndrome has become my passion.  I do not want to hear from families or patients.  I do not want to read newpaper articles.  I want us to either totally prevent Lemierre’s or at least consistently diagnosis it soon enough to have no major morbidity or mortality.  I believe this is an achievable goal.  I pledge myself to that goal.