Reprinted from "The Conversation"By Fabien Vincent, Monash University
and Fabienne Mackay, Monash University
On the television show House, doctors try to diagnose mysterious illnesses, and often when time is running out all other options have been exhausted, one of the characters will offer a diagnosis of last resort: lupus. But in oft-repeated words of main character Dr House, “It’s never lupus.” Or, with good cause, rarely ever.
The making of a meme
For readers not familiar with the show, Dr Gregory House is a cantankerousness but brilliant doctor who specialises in diagnostics. He works with, and plays mind games on, a team of other doctors.
Episodes run to a similar formula – a patient presents with an unusual set of symptoms and the team must solve the case before it proves fatal.
Occasionally, the those in the team run out of credible diagnoses and in their exasperation start to suggest more far-fetched conditions. Quite frequently, someone pipes up with “Maybe it’s lupus”.
Generally, House will give them a scornful look and reply “It’s not lupus”.
So why is it never lupus? Is it because its symptoms make it easily mistaken for other illnesses or the difficulty of making a diagnosis? Yes and yes but surely this can’t stand in the way of Dr House’s abilities.
Maybe lupus is invoked because it doesn’t have the option of a miracle cure at the end of the episode. At any rate, the show’s authors will soon have to coin a new meme because there’s now a new treatment for lupus.
Anatomy of a disease
Lupus, generically referred to as SLE for Systemic Lupus Erythematosus, is an autoimmune inflammatory disease affecting about five million people worldwide.
An autoimmune illness is one in which the immune system attacks the body, destroying healthy tissues.
In the case of lupus, any part of the body may be attacked. The irony of lupus is that the sufferer’s own “anti-sickness” system becomes the actual cause of illness.
Lupus has numerous causes but affects mainly women (9 women for every man) during child-bearing years – usually between 20 and 40.
Its prevalence is variable from one country to another, ranging from 15 to 50 cases for every 100 000 people.
Lupus patients often suffer unpredictable bouts of the disease, called flares, followed by periods of remission.
Of the two forms of lupus, the relatively benign and the more frequent one involves skin rashes – appearing mainly on the face and scalp – and joints, especially in the small joints of the hands and in the knees.
The second more rare and severe form affects internal organs, most often harming kidneys, heart, lungs, blood vessels and the brain.
SLE can be fatal, but its prognosis, which depends on the degree and nature of organ involvement – resulting in renal and vascular damage – has dramatically changed in the past 50 years.
Indeed, the five-year survival rate, which was less than half the number of sufferers in 1955, is currently over 90%. However, the death rate among lupus patients remains four times higher than the general population of the same age.
The two leading causes of death are kidney damage and infections in the first five years, and vascular damage and infectious complications after that.
Hidden grain of truth
The most likely reason for lupus being used as a joke in House is because of the difficulty in delivering a diagnosis of the disease.
Initial and chronic lupus symptoms mimic the symptoms of several other diseases, leading to misdiagnoses and making lupus extremely challenging to diagnose.
As lupus symptoms vary widely and come and go unpredictably, diagnosis is usually based on detailed clinical examination and adapted laboratory tests. These latter include anti-dsDNA or anti-Sm antibodies, which are characteristic of and specific to the disease.
Diagnosis at an early stage is particularly difficult because a number of general symptoms such as fatigue, weight loss, unexplained fever, may mislead the clinician.
The non-specific nature of this disease, especially in its initial form, explains the delay in diagnosis.
Over time, a variety of more specific symptoms will appear during flares involving various organs simultaneously or successively. After several years of evolution within a body, all organs may be affected.
Treating lupus patients
Treatment options are as varied as the multiplicity of clinical forms because each requires a tailored treatment strategy.
Fundamentally, treatment of SLE involves preventing flares and reducing their severity and duration when they occur; helping maintain normal function and; preventing serious complications.
The short-term goal of treatment is to quickly control involvement of the nervous system and weighing up the risks of immunosuppressive therapy.
In the long term, the aim is to define the minimum effective treatment to keep the disease in remission so the patient can have a better quality of life.
But the management of lupus now benefits from a new treatment that is an improvement on the current fairly toxic medications.
Hope on the horizon
In March of this year, the U.S. Food and Drug Administration approved Belimumab as new treatment for lupus.
This is the first treatment for the disease with proven efficacy in a large randomized trial. It is also the first new and effective treatment for it in 50 years.
Prescriptions have to be limited to systemic lupus with autoantibodies, which is active despite the usual treatment, and displays a high level of activity.
Fans of House will know that the first and only time lupus made its appearance in the show was in episode 8 of season 4, leading House to exclaim, “It’s finally lupus!”
And now you also know why this elusive disease remains an option when all others seem exhausted and indeed, why it’s always important to consider whether it may, in fact, be lupus.
The authors do not work for, consult to, own shares in or receive funding from any company or organisation that would benefit from this article. They also have no relevant affiliations.
This article was originally published at The Conversation. Read the original article.
NOTE; Since "The Conversation" published this article, The Australian Therapeutic Drugs Administration has decided against approving Benlysta for use in Australia. (The right-hand column of this blog has a link to a petition in relation to this.)