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Published Online: 1 January 2013

Efficacy of Plasma Exchange in Pharmaco-Resistant Acute Mania in Systemic Lupus Erythematosus

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Publication: The Journal of Neuropsychiatry and Clinical Neurosciences
To the Editor: Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect any part of the body, including the central nervous system. Psychiatric manifestations during SLE are not unusual; their characteristic features are early occurrence and clinical polymorphism.1,2 This clinical case shows a picture of SLE in which the first manifestations were psychiatric, with, in particular, an episode of mania3 with delirium that was resistant to drug treatment but responded well to plasmapheresis.
A 28-year-old, socially well-integrated woman without psychiatric antecedents, was admitted in restraint to our psychiatric ward, after presenting herself at a police station clad only in an overcoat and unable to give her identity. She presented with spatiotemporal disorientation, muddled thinking, mental automaticity, vivid delirium of polymorphic content, and hetero-aggressivity. Somatic and neurological examination results were unexceptional, and the biological analysis results showed leuconeutropenia and a high sedimentation time. Serological tests for syphilis, HIV, HVB, and HVC were negative, and a brain scan and EEG showed no anomalies. A nonspecified psychotic state was diagnosed, and an antipsychotic treatment combining risperidone and loxapine was given.
After 3 weeks in the hospital, with a favorable progression and near-complete regression of the delirium, the patient suddenly relapsed, presenting a manic state with psychotic symptoms and megalomaniac delirium.
Treatment with olanzapine was started, and loxapine was maintained at a higher dose of 600 mg/day. A thymoregulator (valproic acid) was added 2 weeks later, as the symptoms did not improve.
The symptoms of acute mania were accompanied by an episode of fever, a malar erythema, multiple joint pain of the upper limbs, ulceration of buccal and vulvar mucosae, and Raynaud’s syndrome. Biological analysis showed a slight normocytic anemia and leucopenia with neutropenia and lymphopenia. Strongly positive antinuclear antibodies were found (titer above 1/1,280, speckled type). Native anti-DNA antibodies also tested positive, together with anti-RNP/Sm and anti-SSA/RO antibodies. The total complement was lowered, with a collapse of fraction C4.
The diagnosis of a severe flare-up of SLE was therefore made. In association with the psychotropic treatment, a course of cyclophosphamide was given every 3 weeks at 0.6 mg/m3. Progression in 1 month was marked by normalizing of biological analysis, but mania symptoms worsened, with more severe behavioral disorders requiring near-continual isolation of the patient. Brain MRI still showed no sign of vascularity.3
Therefore the psychotropic treatments were discontinued, and, for maniac symptoms, indication was given for a series of six plasma exchanges.
We noted an improvement in the psychiatric symptoms immediately after the first plasmapheresis. After the third plasma exchange session, a regression of the mania symptoms was observed. There still remained brief and fluctuating auditory hallucinations, which totally disappeared 2 weeks after the last plasmapheresis session, marking the total regression of all psychiatric symptoms.
No psychiatric treatment was resumed, and no psychiatric relapse has been reported after 1 year of follow-up.
To our knowledge, this clinical case is the first description of indication and utilization of plasma exchange for a manic state secondary to an SLE flare-up.

References

1.
Waterloo K, Omdal R, Husby G, et al.: Neuropsychological function in systemic lupus erythematosus: a five-year longitudinal study. Rheumatology (Oxford) 2002; 41:411–415
2.
Fernández Ga de Las Heras V, Gorriti MA, García-Vicuña R, et al.: Psychosis leading to the diagnosis of unrecognized systemic lupus erythematosus: a case report. Rheumatol Int 2007; 27:883–885
3.
Brey RL: Neuropsychiatric lupus: clinical and imaging aspects. Bull NYU Hosp Jt Dis 2007; 65:194–199
4.
Alao AO, Chlebowski S, Chung C. Neuropsychiatric systemic lupus erythematosus presenting as bipolar I disorder with catatonic features. Psychosomatics 2009; 50:543–547

Information & Authors

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Published In

Go to The Journal of Neuropsychiatry and Clinical Neurosciences
Go to The Journal of Neuropsychiatry and Clinical Neurosciences
The Journal of Neuropsychiatry and Clinical Neurosciences
Pages: E02 - E03
PubMed: 23686040

History

Published online: 1 January 2013
Published in print: Winter 2013

Authors

Details

R. Schwan, M.D., Ph.D.
J. Lerond, M.D., Ph.D.
V. Laprevote, M.D., Ph.D.
University Hospital of Psychiatrie and Psychothérapie, Laxou, France (RS, JL)
Faculty of Medicine, Lorraine-University, Vandoeuvre-lès-Nancy, France (RS, SZ)
Inserm 9501, University Hospital of Nancy, Nancy, France (RS, MT, SZ, JL, VL)
CSAPA (Health Care Centre of Accompaniment and Prevention in Addictology), University Hospital of Nancy, Nancy, France (RS, VL)
CHU de Nancy, service de médecine vasculaire, Vandoeuvre-les-Nancy, F-54500, France (SZ)

Notes

Correspondence: Professor Raymund Schwan, CHU de Nancy; e-mail: [email protected]

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