Lupus Headaches in Children

What is a lupus headache?

Although headaches among children and adults with lupus are frequent, no universally accepted definition was available until recently, when the American College of Rheumatology (ACR) ad hoc Committee on Neuropsychiatric Lupus Nomenclature¹ adopted the classification of headaches suggested by the International Headaches Society2 for describing lupus headaches.

It is still unclear whether lupus headaches are a sign of active activity and how they should be best treated. In some studies, lupus patients who tested positive for antiphospholipid antibodies (APS) were at a higher risk of experiencing lupus headaches. Antiphospholipid antibodies are special proteins in the blood that increase the risk of developing blood clots and permanent damage from lupus. However, the degree of lupus activity cannot be measured by a certain laboratory test or by certain findings in a physical examination.

How is lupus disease activity measured?

Measurements of disease activity have been greatly aided by the development of so-called "disease activity indices," such as the SLEDAI³, or Systemic Lupus Erythematosus Disease Activity Index. The SLEDAI is one of the most frequently used disease activity indexes for both adults and children with lupus,^{3, 4} and under its criteria, lupus headaches are thought to be only a sign of active lupus if they do not respond to narcotic analgesia, such as morphine and codeine.

Of course, because narcotics are rarely used for headache therapy in children, the SLEDAI is unlikely to capture lupus headaches accurately. Dr. Brunner and co-investigators examined whether the SLEDAI is nonetheless a good measure of disease activity for children with lupus. The study was also aimed towards identifying certain risk factors that make children with lupus more prone to having lupus headaches.

Small study shows headaches are due to a variety of factors

Dr. Brunner and co-investigators reviewed 63 children with lupus. Data from 1,706 clinic visits and hospital admissions were reviewed and information on a total of 214 patient years was available for analysis.

After the children were diagnosed with lupus, lupus headaches occurred especially early in the course of the disease. Forty-three percent of the children developed new or significantly worse, persistent headaches that sometimes were similar to migraines. Only in rare cases were lupus headaches accompanied by disease flares. The headaches were not found to be related to hypertension or the use of other medications.

Children who tested positive for antiphospholipid antibodies had lupus headaches more frequently than those without a positive ANA.

Children with elevated levels of antiphospholipid antibodies together with lupus headaches experienced more neuropsychiatric signs of lupus, such as:

• strokes
• visual disturbances
• problems with thinking or memory.

Lupus headaches by themselves did not appear to be a risk factor for developing neuropsychiatric signs of lupus. However, headaches are known to occur together with other complications of lupus that can lead to permanent damage.

Indeed, permanent damage⁵, including long-standing neuropsychiatric problems, occurred in 13 percent of the children, especially in those who had very active disease for a prolonged time and who also tested positive for ANA.

The limitation of the study is that the data collection was retrospective in nature, so that patient assessment might have been incomplete and lupus headaches may not always have been identified.

Generally, when using the SLEDAI in children with lupus, the exact measurement of the headache appears not to be important for the determination of disease activity. Despite shortcomings in capturing lupus headaches, the SLEDAI remains a good index to measure the disease activity in children.

Conclusions

Lupus headaches most likely are multifactorial, and probably only a small proportion of them truly represent active lupus^{6, 7}. Given the frequency of lupus headaches among children with lupus, more research is required to find good treatments and to develop tests that can help physicians identify those children with lupus headaches who are at risk for developing permanent damage from the disease.

References

1. The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum 1999; 42:599-608.
2. Classification and diagnostic criteria for headache disorders, cranial neuralgias and facial pain. Headache Classification Committee of the International Headache Society. Cephalalgia 1988; 8 Suppl 7:1-96.
3. Bombardier C, Gladman DD, Urowitz MB, Caron D, Chang CH. Derivation of the SLEDAI. A disease activity index for lupus patients. The Committee on Prognosis Studies in SLE. Arthritis Rheum 1992; 35:630-40.
4. Brunner HI, Feldman BM, Bombardier C, Silverman ED. Sensitivity of the Systemic Lupus Erythematosus Disease Activity Index, British Isles Lupus Assessment Group Index, and Systemic Lupus Activity Measure in the evaluation of clinical change in childhood-onset systemic lupus erythematosus. Arthritis Rheum 1999; 42:1354-60.
5. Gladman DD, Urowitz MB, Goldsmith CH, et al. The reliability of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index in patients with systemic lupus erythematosus. Arthritis Rheum 1997; 40:809-13.
6. Abel T, Gladman DD, Urowitz MB. Neuropsychiatric lupus. J Rheumatol 1980; 7:325-33.
7. Omdal R, Waterloo K, Koldingsnes W, Husby G, Mellgren SI. Somatic and psychological features of headache in systemic lupus erythematosus. J Rheumatol 2001; 28:772-9.

About the Authors

Dr. Hermine I. Brunner, Dr. Daniel J. Lovell, and Anne L. Tomasi are with the Division of Rheumatology at Cincinnati Children's Hospital Medical Center, Department of Pediatrics, at the University of Cincinnati in Ohio. Dr. Lovell also serves as a member of the Lupus News Advisory Board.

Dr. Olcay Jones is with the Division of Immunology and Rheumatology at All Children's Hospital at the University of South Florida in St Petersburg, FL.

Dr. Marisa S. Klein-Gitelman is with the Division of Pediatric Immunology and Rheumatology at Children's Memorial Hospital, Northwestern University, in Chicago, IL.

Headache Classification Used by the ACR

There are different types of headaches observed in patients with lupus. They are:

• Migraine headaches: Recurrent headache with up to 72 hours duration; unilateral location (appears only on one side); worsens with routine physical activity; may be associated with nausea, vomiting, and sensitivity to light.
• Tension headache: Recurrent episodes of headaches lasting minutes to days; pain is typically bilateral (appears on both sides) in location; symptoms do not worsen with routine physical activity.
• Cluster headache: Attacks of severe, strictly unilateral pain, especially behind the eyes and/or at the temples, usually lasting 15-180 minutes and occurring up to 8 times per day; during headaches the eyes may become swollen, watery, and red; there may be facial sweating.
• Headache from intracranial hypertension: All of the following: increased intracranial pressure; edema (swelling of the eyes); no signs of tumor, thrombus, or bleeding seen by CT or MRI; normal cerebrospinal fluid.
• Intractable nonspecific headaches: Headaches not fulfilling the categories (I - IV) that occur with other CNS abnormalities.