RESUMO
Susac syndrome is an occlusive arteriolar disease that provokes infarcts in the cochlea, retina, and brain of young subjects, mostly women. Its cause is unknown. Some infarcts may be asymptomatic and only revealed by ancillary investigation: 1) audiogram that shows bilateral sensorineural hearing loss predominating on low frequencies, 2) funduscopy and fluorescein retinal angiography demonstrating bilateral distal branch retinal artery occlusions, and 3) brain MRI T2-weighted images disclosing small multifocal hyperintensities in white and gray matter. Treatment options are not codified, ranging from antithrombotic drugs to immunomodulatory therapy. Course is self-limited after an active fluctuating phase. Dementia, blindness, and deafness are rare late sequelae, and half of patients return to normal life.
Assuntos
Encefalopatias , Perda Auditiva Neurossensorial , Oclusão da Artéria Retiniana , Adulto , Anti-Inflamatórios/uso terapêutico , Audiometria de Tons Puros , Encefalopatias/patologia , Ciclofosfamida/uso terapêutico , Diagnóstico Diferencial , Quimioterapia Combinada , Feminino , Angiofluoresceinografia/métodos , GABAérgicos/uso terapêutico , Perda Auditiva Neurossensorial/diagnóstico , Humanos , Imageamento por Ressonância Magnética , Metilprednisolona/uso terapêutico , Prednisona/uso terapêutico , Oclusão da Artéria Retiniana/tratamento farmacológico , Oclusão da Artéria Retiniana/patologia , Síndrome , Ácido Valproico/uso terapêuticoRESUMO
We report 8 patients with Wegener granulomatosis (WG) who suffered from symptomatic urogenital involvement including acute urinary retention related to prostatitis, orchitis, ureteral stenosis, bladder pseudotumor, and penile ulceration. Urogenital manifestations occurred as an isolated manifestation of WG in 4 patients, at the onset of the disease in 1 patient, and as the only symptom of relapse in 3. Data used to distinguish specific WG involvement from infection or cyclophosphamide urothelial toxicity are discussed. Four patients needed a surgical procedure consisting of suprapubic cystostomy for acute urinary retention, bilateral ureteral double J stents for bilateral ureteral stenosis, and prostate transurethral resection. Urogenital symptoms promptly resolved with medical therapy. High-dose corticosteroids and immunosuppressive drugs should be used as first-line therapy to avoid unnecessary surgery.
Assuntos
Cistite/etiologia , Glomerulonefrite/etiologia , Granulomatose com Poliangiite/complicações , Prostatite/etiologia , Obstrução Ureteral/etiologia , Adulto , Ciclofosfamida/uso terapêutico , Quimioterapia Combinada , Feminino , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Masculino , Pessoa de Meia-Idade , Prednisona/uso terapêutico , Estudos RetrospectivosRESUMO
Charts of 180 patients (147 women, 33 men) with systemic lupus erythematosus (SLE) complicated by renal involvement were retrospectively analyzed from a series of 436 patients. Mean age at renal disease onset was 27 years. Thirty-six percent of the patients had renal involvement after diagnosis of lupus, for 30.7% of that group it was more than 5 years later. Renal involvement occurred more frequently in young male patients of non-French non-white origin. Patients with renal involvement suffered more commonly from malar rash, psychosis, myocarditis, pericarditis, lymphadenopathy, and hypertension. Anemia, low serum complement, and raised anti-dsDNA antibodies were more frequent. According to the 1982 World Health Organization classification, histologic examination of initial renal biopsy specimen in 158 patients showed normal kidney in 1.5% of cases, mesangial in 22%, focal proliferative in 22%, diffuse proliferative in 27%, membranous in 20%, chronic sclerosing glomerulonephritis in 1%, and other forms of nephritis in 6.5%. Distribution of initial glomerulonephritis patterns was similar whether renal involvement occurred before or after the diagnosis of lupus. Transformation from 1 histologic pattern to another was observed in more than half of the analyzable patients (those who underwent at least 2 renal biopsies). Nephritis evolved toward end-stage renal disease in 14 patients despite the combined use of steroids and cyclophosphamide in 12. Initial elevated serum creatinine levels, initial hypertension, non-French non-white origin, and proliferative lesions on the initial renal biopsy were indicators of poor renal outcome. Twenty-four patients died after a mean follow-up of 109 months from SLE diagnosis. Among our 436 patients, the 10-year survival rate was not significantly affected by the presence or absence of renal involvement at diagnosis (89% and 92%, respectively).
Assuntos
Nefrite Lúpica , Adolescente , Adulto , Fatores Etários , Idade de Início , Anti-Inflamatórios/uso terapêutico , Biópsia , Causas de Morte , Criança , Creatinina/sangue , Feminino , Seguimentos , Humanos , Imunossupressores/uso terapêutico , Falência Renal Crônica/etiologia , Nefrite Lúpica/metabolismo , Nefrite Lúpica/mortalidade , Nefrite Lúpica/patologia , Nefrite Lúpica/terapia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prednisona/uso terapêutico , Prevalência , Proteinúria/etiologia , Estudos Retrospectivos , Análise de Sobrevida , Resultado do TratamentoRESUMO
STUDY OBJECTIVE: s: To assess the affordability of health care to poor rural households in Vietnam under conditions of transition from a planned to a market economy and, in light of other transitional experience, inform policy on increasing access of the poor to affordable care of acceptable quality. DESIGN: Observational study by cross sectional socioeconomic survey, longitudinal healthcare seeking survey, and qualitative semi-structured interviews and focus group discussions; qualitative follow up over six years. SETTING: Four rural communes in north of Vietnam between 1992 and 1998. SURVEY PARTICIPANTS: 656 households (2995 people) selected by systematic random sampling. MAIN RESULTS: Compared with non-poor households, poor households had significantly lower average per capita rates of healthcare consultation and expenditure (p<0.01 in both cases). Poor households delayed and minimised healthcare seeking, especially of expensive hospital services. Two thirds of average healthcare spending by poor households was on relatively inexpensive but frequent acts of local ambulatory care. The poor restrained their healthcare seeking but not in proportion to income: for households reporting illness, the average proportion of income devoted to health care was 21.9% for the poor compared with 8.2% for the non-poor (p<0.01). To meet healthcare costs, many poor households reduced essential consumption, sold assets and incurred debt, threatening their future livelihood. CONCLUSIONS: In the short-term the poor need exemption from public sector user fees in both primary and hospital care. In the longer run the government budget and prepayment schemes should replace direct user charges in healthcare finance. Transitional economies like Vietnam should preserve the public health services built up under the planned economy. Market reforms that stimulate growth in the economy appear inappropriate to reform of social sectors.
Assuntos
Transição Epidemiológica , Serviços de Saúde Rural/economia , Assistência Ambulatorial/economia , Assistência Ambulatorial/estatística & dados numéricos , Países em Desenvolvimento/economia , Seguimentos , Custos de Cuidados de Saúde , Reforma dos Serviços de Saúde/economia , Gastos em Saúde , Política de Saúde , Acessibilidade aos Serviços de Saúde/economia , Nível de Saúde , Hospitalização/economia , Hospitalização/estatística & dados numéricos , Humanos , Renda , Estudos Longitudinais , Aceitação pelo Paciente de Cuidados de Saúde/estatística & dados numéricos , Pobreza , Saúde Pública/economia , Serviços de Saúde Rural/estatística & dados numéricos , Fatores de Tempo , VietnãRESUMO
OBJECTIVE: To report 4 cases of refractory panuveitis due to Behçet's disease treated with a novel therapy: infliximab. METHODS: Retrospective study of 3 women and 1 man of Causasian origin with Behçet's disease complicated with panuveitis. Their uveitis was relapsing from 48 to 96 months and was resistant to the combination of colchicine (n = 4), high-dose prednisone (n = 4), pentoxyphilline (n = 2) and various immunossuppressors and/or immunomodulators given successively: intravenous cyclophosphamide (n = 4), azathioprine (n = 3), interferon alpha (n = 3), cyclosporine A (n = 2), oral cyclophosphamide (n = 1), mycophenolate mofetil (n = 1), methotrexate (n = 1), high-dose immunoglobulin (n = 1). Combination with respectively 1, 3, 4 and 5 immunossuppressors and/or immunomodulators failed before institution of infliximab. After informed consent was obtained, infliximab was administered as a single infusion of 5 mg/kg (maximum dose: 400 mg) at day 1, at week 2, 6 and then every 8 weeks. RESULTS: With a follow-up ranging from 7 to 22 months, infliximab was efficient in all cases. The mean prednisone dose decreased from 45 mg to 13 mg daily. Total recovery of visual acuity was observed in half of the cases. Infliximab was well tolerated without fever, severe sepsis or autoimmune manifestation. CONCLUSION: Infliximab may be efficient in refractory uveitis due to Behçet's disease. The optimal dose, rhythm and duration of infliximab infusions need to be standardized.
Assuntos
Anticorpos Monoclonais/uso terapêutico , Antirreumáticos/uso terapêutico , Síndrome de Behçet/tratamento farmacológico , Pan-Uveíte/tratamento farmacológico , Fator de Necrose Tumoral alfa/imunologia , Adolescente , Adulto , Anticorpos Monoclonais/administração & dosagem , Antirreumáticos/administração & dosagem , Síndrome de Behçet/complicações , Criança , Resistência a Múltiplos Medicamentos , Quimioterapia Combinada , Feminino , Humanos , Fatores Imunológicos/farmacologia , Imunossupressores/farmacologia , Infliximab , Infusões Intravenosas , Masculino , Pan-Uveíte/etiologia , Recidiva , Estudos Retrospectivos , Resultado do TratamentoRESUMO
OBJECTIVE: Adult onset Still's disease (AOSD) is a systemic disorder with an uncertain outcome at the time of diagnosis. The clinical response to nonsteroidal antiinflammatory drugs (NSAIDs) is often unsatisfactory in adult patients. Chronic use of steroids is frequently required, but may result in severe side effects. We report the results of an uncontrolled, unblinded trial of intravenous immunoglobulin (IVIG) in 7 patients with AOSD. METHODS: Seven consecutive patients unresponsive or poorly responsive to NSAIDs were enrolled. None of them had received steroids previously. AOSD was defined by the Yamaguchi criteria (J Rheumatol 1992; 19: 424). IVIG were administered every 4 weeks. NSAIDs were initially maintained. A positive response was defined by the disappearance of fever and arthritis within 2 weeks after the first IVIG infusion. RESULTS: Two patients failed to respond. Five patients initially considered to be good responders were given a total of 4 to 6 IVIG infusions. One of them relapsed at the time of the fourth IVIG infusion. The four others had a favourable clinical and biological course. At the time of evaluation 3 patients were symptom-free and off therapy, while one was still receiving low dose NSAIDs. CONCLUSION: This short, uncontrolled, unblinded study suggests that IVIG might represent a potential alternative to classical steroid therapy in patients with AOSD refractory to NSAIDs. These preliminary results need to be confirmed, however, in a double-blind randomized study.
Assuntos
Anti-Inflamatórios não Esteroides/administração & dosagem , Imunoglobulinas Intravenosas/administração & dosagem , Doença de Still de Início Tardio/tratamento farmacológico , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Falha de TratamentoRESUMO
PURPOSE: To evaluate the efficacy of interferon alfa-2a (IFN alfa) in severe uveitis associated with Behçet's disease, that is refractory to steroids and conventional immunosuppressive agents. PATIENTS AND METHODS: Patients with Behçet's disease (according to the International Study Group criteria), who relapsed despite steroids and immunosuppressive agents, were included in this retrospective study. Ophthalmological examination, laser flare photometry, and fluorescein angiography associated with laboratory tests were performed at regular intervals. IFN alfa (3 millions units thrice a week) was injected subcutaneously. RESULTS: Eight patients (sex ratio: 1) were included between May 1995 and January 1999. The mean age was 29.1 years (14-54 years) and the disease was present between 11 and 167 months before the administration of IFN alfa. IFN alfa was efficient in all cases with a mean follow-up of 22 months (10-55 months). Steroids were tapered from a mean dosage of 47 mg/d to a mean dosage of 8.5 mg/d. Ocular inflammation was controlled and visual acuity improved in all cases. Treatment was generally well tolerated despite a constant but transient flu-like syndrome. IFN alfa was tapered in three patients and stopped in one case without any relapse after five months. CONCLUSIONS: Within the limitations of this retrospective study, low-dose IFN alfa seems to be well tolerated, promising in the management of refractory forms of uveitis due to Behçet's disease, and effective in allowing a reduction of steroid dosage. A prospective controlled study is necessary to confirm these preliminary results.
Assuntos
Inibidores da Angiogênese/uso terapêutico , Síndrome de Behçet/tratamento farmacológico , Interferon-alfa/uso terapêutico , Retina/patologia , Uveíte Posterior/tratamento farmacológico , Corpo Vítreo/patologia , Adolescente , Adulto , Síndrome de Behçet/complicações , Síndrome de Behçet/diagnóstico , Diagnóstico Diferencial , Feminino , Angiofluoresceinografia , Fundo de Olho , Humanos , Interferon alfa-2 , Masculino , Pessoa de Meia-Idade , Prognóstico , Proteínas Recombinantes , Estudos Retrospectivos , Índice de Gravidade de Doença , Uveíte Posterior/diagnóstico , Uveíte Posterior/etiologia , Acuidade VisualRESUMO
INTRODUCTION: We report 2 cases of bilateral carotid thrombosis secondary to repeated attempts of strangulation. EXEGESIS: The first case was discovered in a 31-years-old woman who complained of psychiatric manifestations, memory loss and aphasia. Subsequently she developed hemiplegia which worsened under low dose aspirin. The second case occurred in a 41-years-old woman who presented with sudden hemiplegia and aphasia. Diagnosis of bilateral carotid thrombosis based on angiocomputerized tomography or angioRMI data which suggested dissection. Thrombosis location was similar: bilateral, symmetric, close to the carotid bifurcation. Both women had atherosclerosis risk factors: current smoking, use of estrogen containing pill, dyslipidemia and/or familial cardiovascular history. Search for thrombophilia was negative. Both women had been victims of intimate partner violence with repeated attempts of strangulation, but they were stopped 8 and 6 years before the diagnosis of carotid thrombosis. Several private interviews were necessary before knowing the exact cause. CONCLUSION: Symmetrical bilateral thrombosis of internal carotids near the bifurcation should lead to evoke repeated attempts of strangulation, even if there are arterial risk factors, and especially if the patient is a middle-aged female. The therapy is not codified but prolonged anticoagulation is generally prescribed.
Assuntos
Asfixia/complicações , Trombose das Artérias Carótidas/etiologia , Violência Doméstica , Adulto , Afasia/etiologia , Trombose das Artérias Carótidas/patologia , Feminino , Hemiplegia/etiologia , Humanos , Fatores de RiscoRESUMO
Pleuropulmonary manifestations of systemic vasculitis are common, polymorphic and of ambiguous significance: the same pulmonary lesion may reveal a specific manifestation of vasculitis as well as a therapy-induced complication, especially infection which may favor per se a flare-up. Two questions will be successively studied: what are the pleuropulmonary characteristics of Wegener's granulomatosis, Churg-Strauss syndrome, periarteritis nodosa, Behçet's disease, Takayasu's disease and temporal arteritis? What are the major adverse effects that may occur in the course of a treated systemic vasculitis?
Assuntos
Pneumopatias/etiologia , Vasculite/complicações , Humanos , Pneumopatias/diagnóstico por imagem , Pneumopatias/patologia , Doenças Pleurais/diagnóstico por imagem , Doenças Pleurais/etiologia , Doenças Pleurais/patologia , Radiografia , Vasculite/terapiaAssuntos
Uveíte/tratamento farmacológico , Uveíte/etiologia , Adulto , Catarata/complicações , Doença Crônica , Feminino , Humanos , Imunossupressores/uso terapêutico , Infecções/complicações , Edema Macular/complicações , Masculino , Esteroides/uso terapêutico , Resultado do Tratamento , Uveíte/complicações , Uveíte/fisiopatologia , Acuidade VisualAssuntos
Aborto Habitual/prevenção & controle , Anticoagulantes/administração & dosagem , Síndrome Antifosfolipídica/complicações , Heparina/administração & dosagem , Anticorpos Antifosfolipídeos , Anticoagulantes/uso terapêutico , Aspirina/uso terapêutico , Feminino , Humanos , Trabalho de Parto , Período Pós-Parto , GravidezAssuntos
Síndrome Antifosfolipídica/complicações , Infertilidade Feminina/etiologia , Infertilidade Feminina/terapia , Lúpus Eritematoso Sistêmico/complicações , Indução da Ovulação/métodos , Feminino , Fertilização in vitro , França/epidemiologia , Humanos , Indução da Ovulação/efeitos adversos , Gravidez , Resultado da Gravidez/epidemiologiaRESUMO
We report three cases of severe haemorrhagic rupture of luteal ovarian cyst requiring surgical haemostasis in young women treated with long-term oral anticoagulation for antiphospholipid syndrome (APS) who used no contraception. At the time of bleeding, the international normalized ratios were 3.78, 4.24, and 7.11. Anticoagulation was resumed post-operatively, in association with antigonadotropic progestins to induce ovulatory suppression. A systematic use of these progestins should probably be discussed in young women receiving long-term warfarin for APS. Ovarian haemorrhage must be considered when such patients develop acute abdominal pain.
Assuntos
Anticoagulantes/efeitos adversos , Síndrome Antifosfolipídica/tratamento farmacológico , Hemorragia/induzido quimicamente , Cistos Ovarianos/induzido quimicamente , Fenindiona/análogos & derivados , Varfarina/efeitos adversos , Administração Oral , Adolescente , Adulto , Anticoagulantes/administração & dosagem , Feminino , Humanos , Cistos Ovarianos/patologia , Fenindiona/administração & dosagem , Fenindiona/efeitos adversos , Ruptura , Varfarina/administração & dosagemRESUMO
OBJECTIVE: To describe a French tertiary referral center experience in the treatment of pregnancies in patients with the antiphospholipid syndrome (APS). METHODS: Retrospective review of the data of 75 consecutive pregnancies in 47 women. RESULTS: After exclusion of induced abortions and pregnancies occurring before APS onset, the prior live birth rate was 7.9%. Forty-nine pregnancies occurred in women with history of vascular thrombosis, 17 with history of thrombocytopenia. Heparin was prescribed in 39 pregnancies, associated with aspirin in 35 cases, and aspirin alone was used in 36 as first-line therapy. Corticosteroids were prescribed in 38 pregnancies. Three pregnancies by in vitro fertilization led to one embryonic loss, one full term birth, and one premature birth. Six pregnancies treated with immunoglobulin ended in one fetal death, 2 premature and 3 full term deliveries. The outcome of the other 66 pregnancies was one embryonic loss, 8 fetal deaths, 16 prematurates, and 38 full term births. Use of corticosteroids correlated with severe prematurity (p = 0.005), preeclampsia (p = 0.014), intrauterine growth retardation (p = 0.005), and presence of disease associated to APS (p = 0.009). After exclusion of one fetal death associated with congenital anomaly, live birth rate was 72.9%. There was a trend for higher rate of fetal survival in patients without history of vascular thrombosis (84.6 vs 66.4%; p = 0.11). CONCLUSION: Obstetrical prognosis in APS was improved by antithrombotic therapy. Studies are needed to define individual risk and specific significance of the various antiphospholipid antibodies, in order to improve the respective indications for aspirin alone or with heparin in women without thrombotic events.
Assuntos
Síndrome Antifosfolipídica/tratamento farmacológico , Síndrome Antifosfolipídica/epidemiologia , Complicações na Gravidez/tratamento farmacológico , Complicações na Gravidez/epidemiologia , Resultado da Gravidez , Gravidez de Alto Risco , Adulto , Síndrome Antifosfolipídica/diagnóstico , Feminino , Morte Fetal/epidemiologia , Doenças Fetais/diagnóstico , Doenças Fetais/epidemiologia , Fibrinolíticos/administração & dosagem , Humanos , Gravidez , Complicações na Gravidez/diagnóstico , Prevalência , Probabilidade , Prognóstico , Estudos Retrospectivos , Fatores de RiscoRESUMO
OBJECTIVE: To study maternal and fetal outcome in women with past or present histologically proven systemic lupus erythematosus (SLE) nephritis. METHOD: Retrospective study of 32 pregnancies in 22 women with past or present histologically proven SLE nephritis in a single French centre. RESULTS: Pregnancy (25 planned and 7 not planned) occurred in a mean (SD) of 8 (5) years after SLE diagnosis and 6 (4) years after renal disease onset. Seven occurred in women with antiphospholipid syndrome. At pregnancy onset, all but one woman had creatininaemia below 100 micromol/l, five had proteinuria >0.5 g/day, none had hypertension. Twelve pregnancies occurred in women previously treated with immunosuppressant drugs. Treatment comprised prednisone (n=31), hydroxychloroquine (n=11), aspirin (n=22), heparin (n=12), and azathioprine in one patient with steroid resistant nephrotic syndrome disclosing SLE. No therapeutic abortion was done. During pregnancy or the postpartum period, or both, proteinuria >0.5 g/day occurred in 10 women (five related to pre-eclampsia, four to renal flare, one to stable nephrotic syndrome). One flare consisted of mild arthralgias. Pregnancy outcome comprised one feto-maternal death in SLE disclosed by pregnancy, five embryonic losses, two fetal deaths, and 18 premature (one neonatal death) and six full term births. No criterion appeared to influence fetal survival significantly. At long term, one patient died during an SLE flare, three women had renal relapses. At the last visit, all had creatininaemia below 100 micromol/l except one woman with creatinine level 115 micromol/l, nine had proteinuria >0.5 g/day, and one was treated for hypertension. CONCLUSION: Pregnancy need not be discouraged in women with a history of SLE nephritis with normal or mildly impaired renal function. Deterioration of renal function rarely occurs. However, these pregnancies are at high risk of pre-eclampsia and prematurity.
Assuntos
Nefrite Lúpica/complicações , Complicações na Gravidez , Adolescente , Adulto , Feminino , Fertilidade , Morte Fetal/etiologia , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Nefrite Lúpica/tratamento farmacológico , Nefrite Lúpica/fisiopatologia , Masculino , Gravidez , Complicações na Gravidez/tratamento farmacológico , Resultado da Gravidez , Prognóstico , Estudos Retrospectivos , Fatores de RiscoRESUMO
A severe arterial stenosis involving the origin of the posterior and anterior basal branches of the right lower lobe artery was discovered in a 86 year-old Caucasian woman who presented with a typical periarteritis nodosa. We believe it was specific because its discovery was concomitant to the diagnosis of periarteritis nodosa and no other cause was obvious.
Assuntos
Arteriopatias Oclusivas/patologia , Poliarterite Nodosa/patologia , Artéria Pulmonar/patologia , Idoso , Idoso de 80 Anos ou mais , Angiografia , Arteriopatias Oclusivas/diagnóstico por imagem , Feminino , Humanos , Artéria Pulmonar/diagnóstico por imagemRESUMO
We report on a patient who presented with sustained fever, weight loss, haemoptysis and elevated erythrocyte sedimentation rate. The diagnosis of Behçet's disease was based on recurrent oral and genital aphthae, pseudofolliculitis and a history of thrombophlebitis. A right intraventricular thrombus and bilateral pulmonary aneurysms were discovered. Their complete recovery was observed within 6 months after a combination of prednisone, azathioprine, colchicine and aspirin therapy was started.
Assuntos
Arterite/complicações , Síndrome de Behçet/complicações , Trombose/complicações , Corticosteroides/uso terapêutico , Aneurisma/complicações , Aneurisma/diagnóstico por imagem , Aneurisma/tratamento farmacológico , Antirreumáticos/uso terapêutico , Arterite/tratamento farmacológico , Aspirina/uso terapêutico , Azatioprina/uso terapêutico , Síndrome de Behçet/tratamento farmacológico , Colchicina/uso terapêutico , Ecocardiografia , Supressores da Gota/uso terapêutico , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Inibidores da Agregação Plaquetária/uso terapêutico , Circulação Pulmonar , Trombose/tratamento farmacológico , Tomografia Computadorizada por Raios XRESUMO
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.
Assuntos
Lúpus Eritematoso Sistêmico/complicações , Indução da Ovulação/efeitos adversos , Adulto , Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/etiologia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/etiologia , Gravidez , Fatores de Risco , Tromboembolia/etiologiaRESUMO
OBJECTIVE: To report on four patients with Behçet's disease associated with endomyocardial fibrosis involving the right or the left ventricle. METHODS: Charts of more than 350 patients with Behçet's disease were reviewed. Endomyocardial fibrosis was confirmed because of cardiac failure in three patients and incidentally discovered by histological examination of an operative specimen in one patient. Echocardiography displayed bright echogen endocardium. Angiocardiography showed a reduced ventricular size. Electron beam computed tomography demonstrated a lowdense area involving the endocardium. Magnetic resonance imaging showed a mass of intermediate intensity on T1 weighted images. Diagnosis of endomyocardial fibrosis was based on histological study of a biopsy specimen in one patient and of an operative specimen in three. RESULTS: Six other similar cases of endomyocardial fibrosis complicating Behçet's disease were previously reported in the medical literature. Endomyocardial fibrosis predominantly involved the right ventricle. It can be considered a feature of Behçet's disease because: (a) no other cause was discovered; (b) arteritis, valvulopathy, and intraventricular thrombus were closely linked, and (c) all patients with endomyocardial fibrosis had vasculo-Behçet pattern. CONCLUSION: Endomyocardial fibrosis may be the sequelae of vasculitis involving endocardium or myocardium, or both and complicated with intraventricular thrombosis. Behçet's disease should be added to the list of causes of endomyocardial fibrosis.