# Lupus headache

Lupus headache is a proposed, specific headache disorder in patients with systemic lupus erythematosus (SLE). 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%; 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.

## Definition

Lupus headache is an important item in the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), a scoring system often used in lupus research. The SLEDAI describes lupus headache as a "severe, persistent headache; may be migrainous, but must be nonresponsive to narcotic analgesia". 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.

## 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. Migraine patients are typically adult women around age 40, a demographic group in which SLE is also more common.

## 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. 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, 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.

## Footnotes

* ↑ Cuadrado MJ, Sanna G (2003). "Headache and systemic lupus erythematosus". Lupus. 12 (12): 943–6. doi:10.1191/0961203303lu506oa. PMID 14714915. S2CID 23123989.
* ↑ "Lupus Headache".
* 1 2 Davey R, Bamford J, Emery P (March 2008). "The ACR classification criteria for headache disorders in SLE fail to classify certain prevalent headache types". Cephalalgia. 28 (3): 296–9. doi:10.1111/j.1468-2982.2007.01510.x. PMID 18254898. S2CID 23938646.
* 1 2 Davey R, Bamford J, Emery P (August 2007). "The validity of the inclusion of "lupus headache" in the Systemic Lupus Erythematosus Disease Activity Index". Arthritis Rheum. 56 (8): 2812–3. doi:10.1002/art.22798. PMID 17665430.
* 1 2 Mitsikostas DD, Sfikakis PP, Goadsby PJ (May 2004). "A meta-analysis for headache in systemic lupus erythematosus: the evidence and the myth". Brain. 127 (Pt 5): 1200–9. doi:10.1093/brain/awh146. PMID 15047589.
* ↑ Brunner HI, Jones OY, Lovell DJ, Johnson AM, Alexander P, Klein-Gitelman MS (2003). "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. 12 (8): 600–6. doi:10.1191/0961203303lu430oa. PMID 12945718. S2CID 32927986. Archived from the original on 2019-12-09. Retrieved 2009-02-02.
* ↑ Omdal R (2002). "Some controversies of neuropsychiatric systemic lupus erythematosus". Scand. J. Rheumatol. 31 (4): 192–7. doi:10.1080/030097402320318369. PMID 12369649. S2CID 1057841.
* ↑ Bernatsky S, Pineau CA, Lee JL, Clarke AE (2006). "Headache, Raynaud's syndrome and serotonin receptor agonists in systemic lupus erythematosus". Lupus. 15 (10): 671–4. doi:10.1177/0961203306069997. PMID 17120594. S2CID 44643859.
* ↑ Bettero RG, Rahal MY, Barboza JS, Skare TL (December 2007). "Headache and systemic lupus erythematosus: prevalence and associated conditions". Arq Neuropsiquiatr (in Portuguese). 65 (4B): 1196–9. doi:10.1590/S0004-282X2007000700020. PMID 18345429.
* ↑ Annese V, Tomietto P, Venturini P, D'Agostini S, Ferraccioli G (2006). "[Migraine in SLE: role of antiphospholipid antibodies and Raynaud's phenomenon]". Reumatismo (in Italian). 58 (1): 50–8. doi:10.4081/reumatismo.2006.50. PMID 16639488.
* ↑ Lessa B, Santana A, Lima I, Almeida JM, Santiago M (November 2006). "Prevalence and classification of headache in patients with systemic lupus erythematosus". Clin. Rheumatol. 25 (6): 850–3. doi:10.1007/s10067-005-0186-x. PMID 16437362. S2CID 28044605.
* ↑ Weder-Cisneros ND, Téllez-Zenteno JF, Cardiel MH, et al. (December 2004). "Prevalence and factors associated with headache in patients with systemic lupus erythematosus". Cephalalgia. 24 (12): 1031–44. doi:10.1111/j.1468-2982.2004.00822.x. PMID 15566417. S2CID 1845267.
* ↑ Appenzeller S, Costallat LT (December 2004). "Clinical implications of migraine in systemic lupus erythematosus: relation to cumulative organ damage". Cephalalgia. 24 (12): 1024–30. doi:10.1111/j.1468-2982.2004.00785.x. PMID 15566416. S2CID 23055360.
* ↑ Whitelaw DA, Hugo F, Spangenberg JJ, Rickman R (2004). "Headaches in patients with systemic lupus erythematosus: a comparative study". Lupus. 13 (7): 501–5. doi:10.1191/0961203304lu1050oa. PMID 15352420. S2CID 44450325. Archived from the original on 2019-12-09. Retrieved 2009-02-02.
* ↑ Sfikakis PP, Mitsikostas DD, Manoussakis MN, Foukaneli D, Moutsopoulos HM (March 1998). "Headache in systemic lupus erythematosus: a controlled study". Br. J. Rheumatol. 37 (3): 300–3. doi:10.1093/rheumatology/37.3.300. PMID 9566671.
* ↑ Nobili F, Mignone A, Rossi E, et al. (November 2006). "Migraine during systemic lupus erythematosus: findings from brain single photon emission computed tomography". J. Rheumatol. 33 (11): 2184–91. PMID 17086605. Archived from the original on 2011-07-13. Retrieved 2009-02-02.
* ↑ Colamussi P, Giganti M, Cittanti C, et al. (January 1995). "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. 22 (1): 17–24. doi:10.1007/BF00997243. PMID 7698150. S2CID 19194183.
* ↑ Castellino G, Padovan M, Bortoluzzi A, et al. (March 2008). "Single photon emission computed tomography and magnetic resonance imaging evaluation in SLE patients with and without neuropsychiatric involvement". Rheumatology (Oxford). 47 (3): 319–23. doi:10.1093/rheumatology/kem354. PMID 18218648.
* ↑ Kovacs JA, Urowitz MB, Gladman DD, Zeman R (July 1995). "The use of single photon emission computerized tomography in neuropsychiatric SLE: a pilot study". J. Rheumatol. 22 (7): 1247–53. PMID 7562753.