Controversy exists concerning the prevalence of headache in systemic lupus erythematosus (SLE) and its importance as a neuropsychiatric manifestation, especially when it occurs independently of disease activity and with the typical characteristics of primary chronic headache. Most reports to date have either studied both types of headache, whether or not related to systemic lupus erythematosus, or have not used adequate controls. This study determines the prevalence and types of chronic primary headache in patients with systemic lupus erythematosus in a case-control study which we performed in a tertiary care hospital. We studied 71 consecutive patients with systemic lupus erythematosus and 71 healthy unrelated subjects matched for age and sex from the same geographical area. Clinical evaluation using a specific standard protocol for the presence and characteristics of headache according to the operative classification criteria of the International Headache Society. The overall prevalence of headache, tension-type headache, and migraine was similar among patients and controls [33 (46.5%), 17 (23.9%) and 16 (22.5%) vs 31 (43.7%), 17 (23.9%) and 13 (18.3%), respectively]. The mean age of onset of headache was higher in the patients (28.7 +/- 14.3 vs 18.5 +/- 5.6 y; P = 0.001) and the subjective response to analgesics was lower than in the controls [19 (63%) vs 28 (93.3%); P = 0.004]. There were no differences in the presence of precipitating factors or family history of headache. There were no relevant clinical or immunological differences among the patients regarding the presence or absence of tension-type headache or migraine. Patients with systemic lupus erythematosus do not differ from healthy controls for the presence and type of chronic or recurrent headache.