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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, anticardiolipin antibodies, and low complement levels. Anti-beta 2-glycoprotein 1 and antihistone antibodies were negative. Cerebrospinal fluid showed a sterile and acellular liquid.

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 lateonset 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

neuropathy, autonomic central nervous system, and personality or movement disorders are rare. The diagnosis is important to make since the symptoms respond in most cases to corticosteroids, as illustrated in the case patient.12 The classical characteristic of drug-induced SLE14,15 is the combination, to various degrees, of arthralgia, skin involvement, serositis, positive anti-histone antibodies, and negative antiDNA antibodies. On the other hand, malar rash, photosensitivity, alopecia, and renal or neurological dysfunctions are rare. Despite the fact that neurological involvement is rare in drug-induced SLE,14,15 this origin was assumed for the case patient from the facts that very few cases of spontaneous SLE have been described at this age, beta blockers are among the main causes of drug-induced SLE, and from the chronology of events. In this particular case, given the uncertainty of the origin of the disease (ie, drug-induced or not) and the severity of SLE manifestations, the patient was treated with corticosteroids in addition to the withdrawal of potential inducers of SLE. Many studies have compared the severity of SLE depending on the age at onset (after vs before age 50 yr). They are not clearly conclusive, since half of them found that there is no significant difference,16-19 whereas the other half conclude that the severity was lower for late-onset cases.20-24 Additionally, as illustrated by the case patient, four articles emphasize that late-onset SLE can be severe because of the clinical context and complications of treatments, especially corticosteroids.10,12,20,24 This higher mortality was demonstrated in patients with SLE diagnosed both after the age of 50 years20,24 and after the age of 65 years.10,12 Conclusion Even though SLE affects mostly younger persons, it can also be diagnosed in elderly individuals. In such cases, the diagnosis is more difficult, as the clinical presentation may be atypical. Confusion can be the main symptom and, after eliminating the frequent etiologies, rare causes such as SLE must be evoked. Treatment must be adapted to the severity of SLE and to the comorbidities of patients. The authors report no relevant financial relationships. Drs. Breining, Carret-Rebillat, Piette, and Chaibi are from the Department of Internal Medicine, Charles Foix Hospital, Ivry-sur-Seine, France; and Drs. Costedoat-Chalumeau and

Leroux are from the Department of Internal Medicine 2, Piti-Salptrire Hospital, Paris, France. References: 1. Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997;40:1725. 2. Beyan E, Uzuner A, Beyan C. An uncommon cause of fever in the elderly: Late-onset systemic lupus erythematosus. Clin Rheumatol 2003;22:481-483. Published Online: November 7, 2003. 3. Takeda N, Teramoto S, Ihn H, et al. A case of very late onset overlap syndrome of systemic sclerosis and systemic lupus erythematosus [article in Japanese]. Nippon Ronen Igakkai Zasshi 2000;37:74-79. 4. Ito H, Nanamiya W, Kuroda N, et al. Chronic lupus peritonitis with massive ascites at elderly onset: Case report and review of the literature. Intern Med 2002;41:1056-1061. 5. Chan KM. Systemic lupus erythematosus--not to be forgotten in the elderly. Singapore Med J 1995;36:105-106. 6. Mirsattari SM, Power C, Fine A, et al. Neuropsychiatric systemic lupus erythematosus and the syndrome of inappropriate secretion of antidiuretic hormone: A case report with very late onset systemic lupus erythematosus. Br J Rheumatol 1998;37:1132-1134. 7. Koga T, Kubota Y, Kiryu H, et al. Late onset systemic lupus erythematosus with lichen planus-like eruption and cardiac tamponade. Eur J Dermatol 2000;10:620-622. 8. Iijima S, Takita T, Otsuka F. Late onset systemic lupus erythematosus diagnosed in an elderly man with unusual skin eruptions and sudden death. J Dermatol 1995;22:943-947. 9. Gordon CD, Lozada CJ. Hydronephrosis and painless ascites: Rare features of late-onset SLE. Clin Exp Rheumatol 2005;23:551. 10. Pu SJ, Luo SF, Wu YJ, et al. The clinical features and prognosis of lupus with disease onset at age 65 and older. Lupus 2000;9:96-100.

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