Prof Graham Hughes introduces Molly, a 14-year-old girl from Ireland. She had a butterfly rash, hair loss, DNA binding (Anti- DNA antibodies), low C3 but no renal disease. She did not respond to steroid treatment and azathioprine but when Pulse cyclophosphamide 500mgs was administered as treatment, she had clear cerebral improvement and at a 2-year follow-up had clinical and serological improvement.

What is this 14-year-old girl telling us? What is her long term prognosis? She is now 44 and a Professor of History Cambridge.

What is this patient teaching us? –

We don’t know how common CNS involvement is in lupus and this is where the fiction comes in. There are so many different figures.

According to Estes & Christian (1971), cerebral involvement occurs in up to 60% of SLE patients.

The Study by Jarpa et al., (2011) found that:

Psychiatric diagnoses occurred in 44.6% of patients; the most frequent was major depressive episode (21.7%). No association with lupus activity was observed in patients with a DSM-IV diagnosis or MDE or psychological suffering. ACR 1999 NPSLE criteria were present in 42.2% of patients, the majority corresponding to mood (28.9%) or anxiety disorders (15.6%). Suicidal risk was present in 9.6% of patients. Anti-ribosomal P antibodies (13.3%) were not associated with DSM-IV diagnosis.

They concluded –

Severe psychiatric disorders in SLE are common and not associated with disease activity.

According to Calderon –

Depression has only a marginal contribution to cognitive impairment in SLE, suggesting that different pathogenic mechanisms drive this Neuropsychiatric manifestation in this autoimmune disease.

Other neurological features have also been described such as a decreased sense of smell. The ACR has classified Neuropsychiatric Lupus into 19 syndromes.

Quiz

Learn more

1. Antiphospholipid Syndrome (APS) Case Files (Stroke and Thrombosis) By Prof Graham Hughes
2. Antiphospholipid Syndrome (APS) Case Files (Stillbirths and Epilepsy) By Prof Graham Hughes
3. Antiphospholipid Syndrome (APS) Case Files (Gut, Tinnitus, and Migraine) – Prof Graham Hughes
4. Antiphospholipid Syndrome (APS): The Clinical Experience – Dr. Graham Hughes
5. Antiphospholipid Syndrome: 30 Years On – Dr. Graham Hughes
6. Central Nervous System (CNS) Involvement in Antiphospholipid (Hughes) Syndrome – Prof Graham Hughes
7. Clues to the Diagnosis of Antiphospholipid Syndrome – Prof Graham Hughes
8. Pathogenesis of Neuropsychiatric Systemic Lupus Erythematosus – Prof Graham Hughes
9. What is Lupus? – Prof Graham Hughes
10. Prof Graham Hughes Introduces You To The World of Lupus and Antiphospholipid Syndrome – Prof Graham Hughes
11. APS and CNS Lupus: Anticoagulation Treatment By Prof Graham Hughes
12. Neuropsychiatric Manifestations in APS By Prof Graham Hughes
13. Q & A session on Lupus, Hughes Syndrome and the Brain – Prof Hughes

References

1. Estes, D., & Christian, C. L. (1971). The natural history of systemic lupus erythematosus by prospective analysis. Medicine, 50(2), 85-96.
2. Jarpa, E et al.,  (2011). Common mental disorders and psychological distress in systemic lupus erythematosus are not associated with disease activity. Lupus, 20(1), 58-66.
3. Calderón, J et al., 2014). Systemic lupus erythematosus impairs memory cognitive tests not affected by depression. Lupus, 23(10), 1042-1053.
4. Shoenfeld, N., Agmon‐Levin, N., Flitman‐Katzevman, I., Paran, D., Katz, B. S. P., Kivity, S., … & Shoenfeld, Y. (2009). The sense of smell in systemic lupus erythematosus. Arthritis & Rheumatism, 60(5), 1484-1487.
5. Hanly, J. G. (2004). ACR classification criteria for systemic lupus erythematosus: limitations and revisions to neuropsychiatric variables. Lupus, 13(11), 861-864.