A Case Report of a Very Late

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A Case Report of a Very Late-Onset Systemic Lupus Erythematous

Authors:

Alice Breining, BS, Anne Sophie Carret-Rebillat, MD, Nathalie Costedoat-Chalumeau, MD,

PhD,

Gaelle Leroux, MD, Franois Piette, MD, and Pascal Chaibi, MD

Introduction

Late-onset systemic lupus erythematous (SLE) is most often a drug-induced SLE, and is

usually a benign form. Use of immunosuppressive drugs is rarely necessary. We report an

unusual case of a late-onset, drug-induced neuropsychiatric SLE (NPSLE).

Case Presentation

Mrs. R, an 80-year-old Caucasian woman, was hospitalized in 2006 for fever. Her familial

history included a sister who had SLE and a daughter who had Basedow disease and multiple

sclerosis. Her past medical history included a diagnosis of coronary heart disease in 2003,

which justified an angioplasty with the implementation of two stents and treatment with beta

blockers, statins, and platelet antiaggregants. In 2004, the patient had an autoimmune

hemolysis without anemia but with positive antinuclear antibody (ANA) (titer 1:320),

positive anti-DNA antibodies (titer 1:40), low haptoglobin, and highly positive direct

Coombs test.

For the 6 months prior to her 2006 hospitalization, Mrs. R had been experiencing a

progressive loss of autonomy associated with a cognitive dysfunction. She arrived at a

geriatric unit for a urinary tract infection with fever. On examination, she was confused and

showed an erythematosquamous eruption of the upper chest and cheeks associated with

alopecia. She had no arthritis, serositis, or kidney disease. After the pyelonephritis was

treated, the patient remained confused, which triggered further investigations.

Biochemistry showed a low sodium level (125 mEq/L) related to a syndrome of inappropriate

secretion of anti-diuretic hormone. Mrs. R had a lymphopenia (360/mm), a hemolysis

without anemia but with positive direct Coombs test, ANA (titer > 1:640 with a homogene

appearance), anti-DNA antibodies (Farr test > 50), anti-nucleosome antibodies, anti-

cardiolipin antibodies, and low complement levels. Anti-beta 2-glycoprotein 1 and anti-

histone antibodies were negative. Cerebrospinal fluid showed a sterile and acellular liquid.


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An electroencephalogram denoted a global deterioration evocating an encephalopathy,

associated with paroxystic comitiality. Finally, the magnetic resonance imaging (MRI) scan

did not reveal any acute vascular lesion. The cutaneous biopsy showed a direct

immunofluorescence compatible with a lupic band.

Based on the above, Mrs. R met 5 out of 11 SLE criteria from the American College of

Rheumatology1 (Table), namely: malar rash, neurological and hematological manifestations,

and positive ANA and anti-DNA antibodies. A diagnosis of NPSLE was made.

Electrolyte abnormalities were corrected without any influence on the confusion that became

even worse. Drugs that may cause SLE, that is beta blockers and statins, were stopped. She

was treated with a high oral dose of corticosteroids (prednisone 1 mg/kg/day), and

antiepileptic treatment (lamotrigine 75 mg/day).

Mrs. R was soon discharged from the acute geriatric unit and went to a convalescent home.

All clinical symptoms, except the cognitive dysfunction, and biological abnormalities rapidly

began to improve. The autoimmune antibodies decreased (Figure).

Mrs. R later presented with numerous complications of corticosteroids: urinary tract infection

with recurrence of coronary heart disease after 2 months of treatment, herpes simplex virus

pneumopathy and perforated ulcer with peritonitis after 3 months, and tuberculosis and

diabetes after 4 months. The dosage of prednisone was reduced gradually to 10 mg/day, and

hydroxychloroquine (400 mg/day) was introduced. After 8 months, her cognitive dysfunction

had improved, with a Mini-Mental State Examination score that increased from impracticable

to 25/30. At last follow-up 2 years later, the patient was doing well and was living at home.

Discussion

NPSLE in very old persons is difficult to diagnose unless suspected because it is generally

considered a disorder of youth and because of its clinical presentation, which is not specific.

A review of the literature reveals many cases of late-onset SLE, showing a wide range of

clinical manifestations.2-9 Occurrence of neurological manifestations in patients with late-

onset SLE varies from 4.8%10 to 90%,11 according to the studies. In elderly persons,

neuropsychiatric manifestations due to SLE are nonspecific.12 They include cognitive

dysfunction, cerebrovascular manifestations, and encephalopathy. Seizures and parkinsonism

have also been described.13 Polyradiculopathy, demyelinating syndrome, headache, cranial


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Leroux are from the Department of Internal Medicine 2, Piti-Salptrire Hospital, Paris,

France.

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A Case Report of a Very Late

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