Lupus Headache

What is the difference between a Lupus headache and other types of headache? It's an important question, because different types of headaches require different kinds of treatment. But it's not an easy question! To get some answers, I'm inviting an experienced neurologist to give us some of his thoughts. Please welcome Dr. Marc Gotkine...

There is no doubt that a variety of neurological conditions (including so-called lupus headache) occur in people with systemic lupus erythematosus (SLE). It has been generally accepted for many years that there is an association between primary headaches (headaches that are not a symptom of disease) and SLE.

Nevertheless there is great controversy regarding the concept of "lupus headache" and whether it exists at all.

There is little doubt that many patients with lupus have headaches so why the controversy and does it actually matter?

The concept of "lupus headache" is important because if there is such an association, then headaches could be an early symptom of brain involvement in SLE and could therefore be a good reason to perform more tests such as an MRI of the brain or a lumbar puncture.

Headaches, including severe debilitating migraines, are very prevalent in the general population. This means that when a patient with migraines develops lupus or vice versa it does not necessarily mean the 2 conditions are related.

The issue (as always with medicine and science in general) can only be solved by statistics. If migraines are significantly more common in patients with SLE than in those without SLE, then this would support the view that migraines and lupus are related and that there is value in the concept of "lupus headache". However, if headaches are no more common in people with SLE than anyone else it, this suggests that "lupus headache" does not exist and lupus is no more likely to be related to headaches than is brown hair, ingrowing toe-nails or a name beginning with the letter "M".

In essence the controversy rests on the fact that some studies have show that there is an association whilst some studies have proved the opposite.

A big "meta-analysis" study (one where all the important studies are lumped into one big study) published in the prestigious journal Brain (reference below) has finally made some important conclusions and dealt with a variety of issues.

Tension-type headache prevalence in SLE

Tension-type headache (TTH) is the most frequent primary headache syndrome in the general population. When all the big studies are combined, there is no difference in the prevalence of TTH in patients with SLE when compared to the rest of the population.

Migraine prevalence in SLE

Migraine with aura is probably more frequent among SLE patients.  Migraine without aura is no more common than in the general population.

Is headache a sign that SLE is involving the brain?

In adults, there is no link between brain involvement in SLE and headache.

Headache may be related to the presence of certain antibodies known as anti-phosphalipid antibodies which can be related to blood clotting problems and other neurological disorders.

Taking all this into account, it is reasonable to conclude that migraine patients with strange or "atypical" auras (for example not just visual, prolonged, occurring at the same time as the headache, occurring without any headache at all) should be investigated for SLE and more specifically for the associated anti phosphalipid antibodies.

In adults with SLE, a headache without any "atypical" features is probably no different to a headache in people without SLE and therefore should be approached in a similar way.

In children with SLE however, the appearance of a headache could signal the need to perform tests to investigate whether the disease has started to affect the brain.

It goes without saying that if a headache occurs with other neurological signs (weakness, confusion, double vision, strange sensations etc) then this takes on a whole new significance and requires immediate investigation in an ER.

Primary Reference: (Brain, Vol. 127, No. 5, 1200-1209, 2004) A meta-analysis for headache in systemic lupus erythematosus: the evidence and the myth Dimos D. Mitsikostas, Petros P. Sfikakis and Peter J. Goadsby

Dr Gotkine works in the Department of Neurology at the Hadassa University Hospital. Visit his website, AsktheNeurologist.com.