Lupus revealing itself after a stiff-person syndrome.
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Biomedical subjects
Publications and source records attributed to S Arfi.
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PURPOSE: To review epidemiological and clinical aspects of systemic lupus erythematosus (SLE) in Martinique, French West Indies. METHODS: Cases of SLE were identified by attending physicians. Patients who presented with at least four of the criteria defined by the American College of Rheumatology were included. Determination of incidence and prevalence included the new cases arising during the 1990-1999 period and 1999 population census results. Probability of survival was based on the use of the Kaplan-Meier estimator. RESULTS: Two hundred and eighty-six patients were studied, including 265 females (92.7%). The average annual incidence was 4.7 cases per 100,000 inhabitants (95% confidence interval [CI]: 2.5-6.9). The prevalence for 1999 was 64.2 cases per 100,000 inhabitants (CI: 56.2-72.2). The mean age at onset was 30 years. Eleven percent of all patients had at least one parent with SLE. Renal disease was present in 139 patients (48.6%), and neurological disorders were diagnosed in 70 patients (24.5%). Patients tested positive for the following antibodies: anti-Sm (37.1%), anti-RNP (58.7%), anti-SSA (47.2%). Mean survival time was: 96.4% (CI: 94.1-98.7) at 5 years, 91.8% (CI: 87.9-95.7) at 10 years. Survival was significantly reduced in patients with end-stage renal disease (n = 40, chi 2 = 6.96, P < 0.01). CONCLUSION: The high incidence of SLE in Martinique and the immunological characteristics of patients were found to be similar to those described in other populations of African descent. The frequency of renal disease and survival rates were identical to those reported in Caucasians.
A 49-year-old man presented with a 8-month history of gait and sphincter disturbances. Examination revealed a spastic paraplegia predominant on the left limb, associated with decreased tactile sensitivity below T10. MRI showed a right posterolateral intramedullary lesion with an isosignal on T1-weighted images and a mild hypersignal on T2-weighted images, with an intense contrast enhancement; the spinal cord presented with an hypersignal on T2-weighted images. At operation, a vascular tumor presented at the spinal cord surface, was well demarcated from the parenchyma and was easily removed. Microscopic examination showed a capillary hemangioma. Three months later the patient exhibited a motor improvement with a right spasticity, deficits in proprioception, without sphincter abnormalities. A new MRI showed the disappearance of the hemangioma and of the preoperative spinal cord hypersignal. Capillary hemangiomas are unusual on nerves or roots. Cases found in the spinal cord are extremely rare: to our knowledge, 5 cases have been reported. On MRI, they are easily distinguished from cavernous hemangiomas, but microscopic examination is necessary to distinguish them from hemangioreticulomas. The postoperative prognosis is generally good. However, a report of a multiple location case, on roots and spinal cord, suggests the need for long follow-up.
OBJECTIVE: Calcification of the ligamentum flavum at the cervical spine is an uncommon condition reported mainly in Japanese patients. We describe the clinical manifestations, imaging study findings, and outcomes in six cases seen in the French West Indies. MATERIAL AND METHODS: We retrospectively reviewed the medical charts of six patients admitted to an orthopedics department for spinal cord compression shown upon computed tomography to be caused by calcification of the ligamentu flavum. There were five women and one man, mean age at admission was 71.7 years (range, 64-79 years) and all six patients were Black. RESULTS: Five patients had cervical myelopathy and one was asymptomatic. All five symptomatic patients had cervical spinal stenosis, explaining the rapid symptom onset (within six and a half months) and severe motor loss. Computed tomography reconstruction in the sagittal plane ruled out ossification of the ligamentum flavum. Magnetic resonance imaging of the neck failed to demonstrate the calcifications but was useful in evaluating the severity of the spinal cord compression. One patient had articular chondrocalcinosis in both knees and another had calcifications in the basal ganglia. Surgical decompression by the posterior route was performed in two patients and was effective in both, whereas two of the three symptomatic patients who did not have surgery experienced worsening neurological loss. Analysis of the operative specimens from the two surgically treated patients showed a mixture of calcium pyrophosphate dihydrate crystals and apatite microcrystals. CONCLUSION: Calcification of the ligamentum flavum is probably underrecognized in blacks. This condition causes severe neurological loss. Imaging studies provide the diagnosis. The pathogenesis remains unclear.
Evidence for a genetic susceptibility to systemic lupus erythematosus (SLE) in humans is based on the high concordance rate observed in identical twins and on the relatively high incidence of familial cases. Although recent genetic studies have lead to significant advances in the identification of new susceptibility genes in SLE, no large clinico-pathologic study of familial SLE has been reported to date. In the present study, we describe the main clinical and immunologic features of 125 lupus multiplex families including at least 2 cases of SLE and/or discoid lupus erythematosus (DLE), recruited through a French national survey starting in July 1997. Medical records of all affected members were reviewed by the same investigator, all available family members were interviewed using the same standardized procedure, and blood was drawn for autoantibodies typing. Clinical and immunologic features of 90 probands from multiplex SLE families were compared with those of 100 sporadic SLE patients sharing the same French Caucasian origin. The 125 lupus multiplex families included 282 affected members (2.3 patients per family); of the 125 families, 96 were of French Caucasian origin. One hundred multiplex families included 2 affected relatives, while 25 included 3 or more affected individuals. The relationship between affected members was sibs (45%), parent-offspring (31%), and second-degree (24%). An autosomal dominant mode of inheritance was strongly suggested in 1 extended pedigree with 6 clinically affected members, and a recessive pattern was suspected in 5 other families. No obvious mode of inheritance could be suspected in most of the remainder. Among French Caucasians, sex ratio, mean age at onset, and clinical and biologic SLE-related manifestations were not significantly different in multiplex compared with sporadic SLE cases. The analysis of these 125 multiplex families suggests a genetic heterogeneity that should be considered for ongoing genomic screening.
STUDY DESIGN: A case of acute disseminated encephalomyelitis (ADEM) preceding the features of cutaneous lupus is reported. OBJECTIVES: To suggest that ADEM and cutaneous lupus are pathophysiologically related. SUMMARY OF THE BACKGROUND DATA: Neurological complications of systemic lupus erythematosus are common. However, demyelinative central nervous system manifestations are rare, and restricted to neuromyelitis optica (NMO). NMO is thought to be a partial form of ADEM. METHODS: A clinical, neuroradiological and immunological study was performed in this case. RESULTS: ADEM was diagnosed in a young black female. Three years later, she developed a cutaneous lupus without hypocomplementemia. CONCLUSION: A relationship between ADEM and cutaneous lupus is suggested. The normal serum complement level in our patient might be a predisposing factor for the development of ADEM.
We report on four women with systemic lupus erythematosus who developed two types of complications after ovulation-induction therapy for primary or secondary infertility. Primary infertility was associated with endometriosis in one patient. Three had previously known systemic lupus erythematosus. All had inactive disease at onset of ovulation-induction therapy. Three patients developed symptoms consistent with moderate lupus flare a few weeks after the onset of ovulation-induction therapy. One patient developed inferior vena cava and unilateral left renal vein thrombosis. No patient became pregnant. A high oestrogen level induced by ovulation-induction therapy may explain the occurrence of lupus flare in patients with prior inactive lupus. All our patients had prior asymptomatic antiphospholipid antibodies. One patient developed a major thrombotic event. The presence of antiphospholipid antibodies increases the thrombotic risk related to ovulation-induction therapy. We conclude that ovulation-induction therapy should be restricted to patients with long-standing inactive systemic lupus erythematosus. A preventive increase of the corticosteroid dosage should be proposed in addition to heparin or antiaggregant therapy for those with prior asymptomatic antiphospholipid antibodies, or with heparin therapy for those with prior antiphospholipid antibody-related events.
The authors report an open prospective study in a group of 16 patients who presented systemic lupus erythematosus with cutaneous and articular symptoms, and who required treatment with a minimum dose of 15 mg per day of prednisone. Methotrexate was given at a dose of 7.5 mg IM per week. Efficacy was demonstrated at the third month with a statistical analysis of four evolution parameters. Improvement was observed in 13 patients out of 16 and permitted the reduction of the amount of prednisone required. Secondary relapse occurred in four cases in spite of an increase in the dose of methotrexate (10 mg per week). Minor side effects were observed in four cases, and methotrexate was discontinued in only two cases. This study suggests that treatment by low doses of methotrexate is beneficial to patients with articular and cutaneous manifestations of corticodependent systemic lupus erythematosus and this therapy could be corticosteroid-sparing.
Myositis linked to HTLV-1 is unfrequent. Over a period of 8 years, 14 patients with inflammatory myopathy were diagnosed in Martinique. Seven were seropositive for HTLV 1 antibody; the clinical and pathological data of whom are presented herein. Five patients presented with polymyositis, two with dermatomyositis. All seven patients had extra-muscular clinical features including neuropathy (4/7) and myelopathy (6/7), resulting in a quite peculiar clinical picture. Muscle biopsy showed a neurogenic process combined with myositic changes in 3/7 patients. Corticotherapy led to dramatic improvement in only one case, but with no sustained effect. HTLV 1 may be considered the etiological agent of this form of dermato-polymyositis, characterized by a clearly distinctive clinico-pathological picture, and a poor response to corticotherapy. As in the case of tropical spastic paraparesis/HTLV 1 associated myelopathy, careful assessment of non-steroidal therapy is now warranted.
A retrospective study was conducted in Martinique to identify patients with polyarthritis and positive serologic tests for the human T-cell lymphotropic virus Type I (HTLV-1). Patients with metabolic or bacterial causes of polyarthritis were excluded. We found 17 cases of HTLV-1-positive polyarthritis (6.7% of the total of polyarthritis patients followed in our department); there were 14 females and three males, and all the patients were West-Indian blacks. Mean age at diagnosis was 50 years. Five patients also had tropical spastic paraparesis. The polyarthritis was the inaugural manifestation of T-cell leukemia in one patient. Four patients had received blood transfusions. Fever, myalgia, and/or skin lesions were present at onset of the polyarthritis in seven cases. All 17 patients had peripheral, bilateral, symmetric polyarthritis; the most commonly involved sites were the hands (17/17) and knees (14/17). Three patients had rheumatoid factor and five had antinuclear antibody. Ten patients met at least four American Rheumatism Association criteria for rheumatoid arthritis; they accounted for 6.7% (10/150) of all polyarthritis patients managed in the same hospital department during the same period. We compared these ten patients with 20 HTLV-1-negative rheumatoid arthritis patients matched on gender, ethnic origin, and disease duration. There were no significant differences between the two groups for any of the parameters studied.
A retrospective study of 26 adults with acute T-cell leukemia showed that 14 patients (54%) had hypercalcemia at some point of the disease. Hypercalcemia was found at presentation in nine patients and revealed the disease in one. Eight patients had hypercalcemia at the time of death. Serum phosphorus and parathyroid hormone levels were normal. All patients with hypercalcemia tested positive for the HTLV-1 by Elisa and Western blot. Six patients had focalized or diffuse lytic roentgenographic bone lesions. Hypercalcemia in acute T-cell leukemia may involve production of interleukin-1-alpha and parathyroid hormone-related protein by HTLV-1-infected cells.
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The human retrovirus HTLV-1 (Human T-cell Lymphotrophic Virus) is responsible for malignant proliferations of mature T lymphocytes. It is also now implicated in neurological disorders dominated by spastic paraplegia. A study of 140 cases of lumbar and root pain enabled us to identify 8 of apparently idiopathic atypical lumbar and root pain which led to the discovery of a positive HTLV-1 serology performed routinely. These cases were remarkable in terms of the rarity and minimal extent of spinal cord signs and of Lasegue's sign, their duration of more than a year and the existence of neurological signs indicative of central involvement. In 2 patients there was secondary progression to a spastic paraparesis. It would appear that the neurotropism of HTLV-1 virus is not limited to the central nervous system but that it can also involve the peripheral nervous system as indicated by certain cases in the literature as well as those collected in Martinique, an endemic area for the virus. Rheumatologists should be aware of this possibility when confronted with such cases of atypical lumbar and root pain and should seek the existence of an HTLV-1 virus infection among other viral etiologies, in particular when the patient concerned comes from an endemic area.
As incidence of SLE is high in Blacks, we studied HLA and SLE associations in the French West Indies, whose population is racially mixed. Forty-seven coloured SLE patients have been typed in HLA A,B,C and DR. We observed B8 association in nearly all of the studies. B15 association, more frequent in Caucasians, was found, also B53 association, a Black variant of B5 more frequent in Blacks. We did not find any class II association.
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