Lupus headache
Lupus headache is a proposed, specific headache disorder in patients suffering from systemic lupus erythematosus (SLE).<ref name="pmid14714915">,
Headache and systemic lupus erythematosus, Lupus, 2003, Vol. 12(Issue: 12), pp. 943–6, DOI: 10.1191/0961203303lu506oa, PMID: 14714915, Full text,</ref><ref name="urlLupus Headache">
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Research shows that headache is a symptom commonly described by SLE patients —57% in one meta-analysis, ranging in different studies from 33% to 78%;<ref name="pmid18254898">,
The ACR classification criteria for headache disorders in SLE fail to classify certain prevalent headache types, Cephalalgia, Vol. 28(Issue: 3), pp. 296–9, DOI: 10.1111/j.1468-2982.2007.01510.x, PMID: 18254898,</ref> of which migraine 31.7% and tension-type headache 23.5%. The existence of a special lupus headache is contested, although few high-quality studies are available to form definitive conclusions.<ref name="pmid17665430">, The validity of the inclusion of "lupus headache" in the Systemic Lupus Erythematosus Disease Activity Index, Arthritis Rheum., Vol. 56(Issue: 8), pp. 2812–3, DOI: 10.1002/art.22798, PMID: 17665430,</ref><ref name="pmid15047589"/>
Definition
Lupus headache is an important item in the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), a scoring system often used in lupus research.<ref name="pmid12945718">,
Lupus headaches in childhood-onset systemic lupus erythematosus: relationship to disease activity as measured by the systemic lupus erythematosus disease activity index (SLEDAI) and disease damage, Lupus, 2003, Vol. 12(Issue: 8), pp. 600–6, DOI: 10.1191/0961203303lu430oa, PMID: 12945718, Full text,</ref> The SLEDAI describes lupus headache as a "severe, persistent headache; may be migrainous, but must be nonresponsive to narcotic analgesia".<ref name="pmid17665430"/> A score of 8 is given to this item (items are given a relative weight of 1, 2, 4 or 8).
The 1999 American College of Rheumatology case definitions of neuropsychiatric syndromes in SLE do not define lupus headache, but rather propose several headache disorders loosely based on the International Headache Society (IHS) classification.
In the IHS scheme, headache due to lupus would be classified as "Headache attributed to other non-infectious inflammatory disease" (7.3.3). This label requires evidence of a disease flare accompanying the headache, and resolution of the headache with immunosuppressant treatment. However, a meta-analysis found no correlation between headaches and disease activity.<ref name="pmid15047589">,
A meta-analysis for headache in systemic lupus erythematosus: the evidence and the myth, Brain, Vol. 127(Issue: Pt 5), pp. 1200–9, DOI: 10.1093/brain/awh146, PMID: 15047589,</ref>
Criticism
Critics of this concept argue that there are no quality studies showing that headaches in patients with SLE differ from those in the general population. A detailed definition of the term lupus headache is lacking, since the terms "severe" and "persistent" are not quantified. Narcotic analgesics are not recommended for migraines or other common headache types. Other definitions from the IHS do not include responsiveness to treatments as a diagnostic criterion.<ref name="pmid18254898"/> Migraine patients are typically adult women around age 40, a demographic group in which SLE is also more common.<ref name="pmid12369649">Omdal R,
Some controversies of neuropsychiatric systemic lupus erythematosus, Scand. J. Rheumatol., 2002, Vol. 31(Issue: 4), pp. 192–7, DOI: 10.1080/030097402320318369, PMID: 12369649,</ref>
Mechanism
Some (but not all) studies have shown an association between (migraine) headaches in SLE and associated Raynaud's phenomenon and/or anti-cardiolipin antibodies.<ref name="pmid17120594">,
Headache, Raynaud's syndrome and serotonin receptor agonists in systemic lupus erythematosus, Lupus, 2006, Vol. 15(Issue: 10), pp. 671–4, DOI: 10.1177/0961203306069997, PMID: 17120594, Full text,</ref><ref name="pmid18345429">, Headache and systemic lupus erythematosus: prevalence and associated conditions, Arq Neuropsiquiatr, Vol. 65(Issue: 4B), pp. 1196–9, DOI: 10.1590/S0004-282X2007000700020, PMID: 18345429,</ref><ref name="pmid16639488">, [Migraine in SLE: role of antiphospholipid antibodies and Raynaud's phenomenon], Reumatismo, 2006, Vol. 58(Issue: 1), pp. 50–8, DOI: 10.4081/reumatismo.2006.50, PMID: 16639488, Full text,</ref><ref name="pmid16437362">, Prevalence and classification of headache in patients with systemic lupus erythematosus, Clin. Rheumatol., Vol. 25(Issue: 6), pp. 850–3, DOI: 10.1007/s10067-005-0186-x, PMID: 16437362,</ref><ref name="pmid15566417">, Prevalence and factors associated with headache in patients with systemic lupus erythematosus, Cephalalgia, Vol. 24(Issue: 12), pp. 1031–44, DOI: 10.1111/j.1468-2982.2004.00822.x, PMID: 15566417,</ref><ref name="pmid15566416">, Clinical implications of migraine in systemic lupus erythematosus: relation to cumulative organ damage, Cephalalgia, Vol. 24(Issue: 12), pp. 1024–30, DOI: 10.1111/j.1468-2982.2004.00785.x, PMID: 15566416,</ref><ref name="pmid15352420">, Headaches in patients with systemic lupus erythematosus: a comparative study, Lupus, 2004, Vol. 13(Issue: 7), pp. 501–5, DOI: 10.1191/0961203304lu1050oa, PMID: 15352420, Full text,</ref><ref name="pmid9566671">, Headache in systemic lupus erythematosus: a controlled study, Br. J. Rheumatol., Vol. 37(Issue: 3), pp. 300–3, DOI: 10.1093/rheumatology/37.3.300, PMID: 9566671, Full text,</ref>
Further studies are needed however to prove the underlying assumption that cerebral vasospasm causes migraines in lupus patients.
Diagnosis
Although specific complications of SLE may cause headache (such as cerebral venous sinus thrombosis or posterior reversible encephalopathy syndrome), it remains unclear whether specific investigations (such as lumbar puncture or magnetic resonance imaging, MRI) are needed in lupus patients presenting with headache. Although studies using MRI or single-photon emission computed tomography (SPECT) often find abnormalities,<ref name="pmid17086605">,
Migraine during systemic lupus erythematosus: findings from brain single photon emission computed tomography, J. Rheumatol., Vol. 33(Issue: 11), pp. 2184–91, PMID: 17086605, Full text,</ref><ref name="pmid7698150">, Brain single-photon emission tomography with 99mTc-HMPAO in neuropsychiatric systemic lupus erythematosus: relations with EEG and MRI findings and clinical manifestations, Eur J Nucl Med, Vol. 22(Issue: 1), pp. 17–24, DOI: 10.1007/BF00997243, PMID: 7698150,</ref> the value of these findings remains unclear, and they have not been able to distinguish a special "lupus headache" from other headache types in people with lupus.<ref name="pmid18218648">, Single photon emission computed tomography and magnetic resonance imaging evaluation in SLE patients with and without neuropsychiatric involvement, Rheumatology (Oxford), Vol. 47(Issue: 3), pp. 319–23, DOI: 10.1093/rheumatology/kem354, PMID: 18218648,</ref><ref name="pmid7562753">, The use of single photon emission computerized tomography in neuropsychiatric SLE: a pilot study, J. Rheumatol., Vol. 22(Issue: 7), pp. 1247–53, PMID: 7562753,</ref>
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