Intractable headaches, the so-called 'lupus headaches', have been long
thought of as a common and characteristic manifestation of systemic l
upus erythematosus (SLE). Seventy-eight patients with SLE, including 1
0 patients with definite central nervous system (CNS) involvement, and
89 healthy individuals matched for age, sex and socioeconomic status,
were studied by a specific questionnaire addressing the characteristi
cs and type of headache. Clinical features of SLE, neurological manife
stations and treatment, disease severity and autoantibody profiles wer
e correlated to the presence of headache. One year prevalence of heada
che was similar between patients (32%) and otherwise healthy individua
ls (30%). No significant differences regarding frequency, family histo
ry of headache and need for analgesic medication were observed. Headac
he refractory to analgesic treatment, but responsive to corticosteroid
regimen, was recorded in only one patient. Clinical and serological f
eatures of SLE, including Raynaud's phenomenon and the presence of and
icardiolipin antibodies, were not significantly different between head
ache sufferers and non-sufferers. In the majority of patients reportin
g headache, anxiety and/or depression co-existed. Episodic tension hea
dache was the most frequent type, while migraine was traced in a quart
er of headache sufferers. Neither the presence nor the clinical type o
f headache was related to, or predictive of, the development of seizur
es or psychosis. These results indicate that headache is not specifica
lly related to SLE expression or severity, and suggest that accepting
the presence even of a severe headache as a neurological manifestation
of SLE in the absence of seizures or overt psychosis may result in ov
erestimation of the disease status.