Successful pregnancy after cyclophosphamide therapy for lupus nephritis.

BACKGROUND: Systemic lupus erythematosus (SLE) often requires administration of cyclophosphamide (CYC), especially for severe glomerulonephritis. As this disease usually affects young women in reproductive age, pregnancy, though not recommended may occur. The teratogenic effects of this drug make pregnancy prognosis and fetal survival indeterminate. METHODS: We reviewed retrospectively the medical records of five patients with SLE who received inadvertently CYC during pregnancy and analyzed fetal outcome. RESULTS: All patients were exposed at the first trimester. Two patients suffered miscarriages, two went to full term and one presented premature labor. CONCLUSION: In spite of potential successful pregnancies after CYC exposure, this drug has teratogenic effects and prescription must be avoided during the pregnancy period. At the same time, the occurrence of these reported unplanned pregnancies strengthen the need of improving patients' education on pregnancy risks during immunosuppressive treatment.

Coexistence of generalized morphea and lichen sclerosus et atrophicus mimicking systemic disease.

A 70-year-old white housewife presented with a rare presentation of coexistent generalized morphea and lichen sclerosus et atrophicus with unusual clinical aspects. The patient had disseminated erythematous lesions that evolved into indurated large plaques. Hypopigmentation and hyperpigmentation developed later, in addition to ivory, white, and shiny plaques on the trunk (Figure 1). The skin of the arms and legs showed a wavy contour (Figure 2). Various areas were markedly sclerotic and some had edematous papules (Figure 3). Multiple indurated, ivory, white, shiny, large, and hypochromic plaques were seen on the trunk Laboratory examinations showed increased immunoglobulin A and antinuclear antibodies 1:200 speckled. Scl-70, anti-centromere, anti-ribonucleoprotein, and anti-DNA tests were negative. Esophageal manometry and abdominal and pelvic ultrasound findings were all normal. Complete blood cell count, blood profile, and urinalysis were also within normal limits. Skin biopsy of an arm lesion showed an atrophic epidermis with orthokeratotic hyperkeratosis and follicular plugging. A broad area of homogenization and edema was seen in the papillary dermis with dilated capillaries and a perivascular lymphocytic infiltration. There was also collagen sclerosis throughout the reticular dermis, and thickened, homogenized collagen bundles replaced the subcutaneous fat. Vessel walls showed proliferated intima with mucin deposition and sclerosis, as well as multiplied elastic layers. There was edema of the papillary dermis in some areas and incipient deposition of calcium (Figures 4-7).

Is higher prevalence of cervical intraepithelial neoplasia in women with lupus due to immunosuppression?

BACKGROUND: Cervical cancer (CC) is still the second in prevalence and mortality among women. In spite of previously observed higher incidence of cervical dysplasia among systemic lupus erythematosus (SLE) patients, few studies have considered the influence of classic risk factors and the use of immunosuppressors (IM). OBJECTIVES: To study cervical dysplasia prevalence among SLE patients submitted or not to immunosuppression and to evaluate its association with classic risk factors. METHODS: A group of 171 SLE patients including 87 who were receiving IM continuously for at least 1 year was compared with 222 age- and sociocultural-paired women (control group) submitted to routine cervical cytopathology. Statistical methods included univariate and multivariate analysis, besides parametric and nonparametric tests. RESULTS: The prevalence of atypical squamous cells of undetermined significance, low-grade and high-grade intraepithelial lesions were significantly increased in SLE patients (12.8%, 5.8%, and 3.5%, respectively) compared with controls (3.1%, 0.9%, and none, respectively, P = 0.0001), although they presented significantly fewer classic risk factors for CC. Multivariate analysis showed that SLE women had a 7-fold higher prevalence of cervical dysplasia (OR: 7.23, 95% IC: 3.40-15.38) and an 11-fold higher prevalence of premalignant cervical dysplasia (OR: 11.36, 95% IC: 2.57-50.10) compared with controls. SLE patients with long-term use of IM presented even higher prevalence of low-grade and high-grade intraepithelial lesions in comparison with those without long-term use of these agents (68.7% vs. 31.1%, P = 0.03). CONCLUSIONS: This study provides evidence that even though not presenting the classic risk factors for CC, SLE patients, especially those exposed to long-term immunosuppression, have increased chances of presenting more premalignant lesions than the general population and they probably need to follow a more stringent CC prevention program.

Freqüência de síndrome metabólica em pacientes com nefrite lúpica / Frequency of metabolic syndrome in patients with lupus nephritis

INTRODUÇÃO: Os pacientes com lúpus eritematoso sistêmico (LES) apresentam morbimortalidade importante por doenças cardiovasculares (DCV). A síndrome metabólica (SM) é um transtorno complexo representado por um conjunto de fatores de risco para DCV. OBJETIVOS: Avaliar a prevalência de SM em uma coorte de pacientes com nefrite lúpica em seguimento em um hospital universitário brasileiro, e o seu impacto na doença, além da freqüência de cada fator de risco cardiovascular analisado individualmente. MÉTODOS: Quarenta e seis pacientes com nefrite lúpica foram estudados e classificados de acordo o critério para SM da National Cholesterol Education Program - Adult Treatment Panel III (NCEP-ATP III). RESULTADOS: A SM esteve presente em 30,4 por cento dos pacientes. Foi observada correlação linear entre a presença de SM e níveis baixos de depuração de creatinina e valores altos de creatinina sérica (p < 0,04 e p < 0,008, respectivamente). Não houve correlação com a classificação de nefrite lúpica, duração da doença, dose cumulativa de prednisona, proteinúria de 24 horas e com o escore Systemic Lupus International Collaborating Clinics (SLICC). CONCLUSÃO: Os pacientes brasileiros com nefrite lúpica estudados apresentam alta prevalência de SM, comparável a de estatísticas internacionais. Os únicos fatores relacionados à SM nessa população foram os que definem a presença de dano renal crônico.

INTRODUCTION: patients with systemic lupus erythematosus (SLE) have n increased morbidity due to cardiovascular diseases (CVD). Metabolic syndrome (MS) is a complex syndrome composed by a cluster of cardiovascular risk factors. OBJECTIVES: study the prevalence of MS in a cohort of Brazilian patients with lupus nephritis in a university hospital; evaluate its impact in disease outcome, and the frequency of each specific risk factor. METHODS: 46 patients with lupus nephritis were studied and defined as having or not MS in accordance with the National Cholesterol Education Program - Adult Treatment Panel III (NCEP-ATP III) criteria. RESULTS: MS was present in 30.4 percent of patients. There was significant association between MS presence and low levels of creatinine clearance and high levels of blood creatinine (p < 0.04 and p < 0.008, respectively). The class of lupus nephritis, the disease duration, cumulative dose of prednisone, 24-hour urine protein and Systemic Lupus International Collaborating Clinics (SLICC) score were not significantly associated with MS. CONCLUSION: In our cohort of patients with lupus nephritis there was a high prevalence of MS, comparable to international data. The only factors associated with MS in that population were the ones that define chronic renal damage.

Impacto da nefrite sobre os resultados gestacionais de mulheres com lúpus eritematoso sistêmico / The impact of nephritis on gestational results in women with systemic lupus erythematosus

OBJETIVO: avaliar o impacto da nefrite lúpica e suas complicacões nos resultados gestacionais. MÉTODOS: avaliamos retrospectivamente 76 gestacões em 63 pacientes com lúpus eritematoso sistêmico (LES). RESULTADOS: a hipertensão arterial estava presente como complicacão clínica em 23 (30 por cento) gestacões. Vinte e sete (36 por cento) gestacões ocorreram em 19 pacientes com nefrite. Encontramos um número significativamente mais elevado de óbitos fetais quando avaliamos todas as pacientes com nefrite em comparacão com aquelas sem nefrite (37 por cento e 12,2 por cento, respectivamente [p=0,019]). CONCLUSÕES: além da atividade de nefrite e do diagnóstico de nefrite propriamente dito, tiveram relacão com pior sobrevida fetal a associacão da síndrome do anticorpo antifosfolipídeo ou deteccão de um dos anticorpos relacionados, a presenca HAS e insuficiência renal (mesmo em suas fases iniciais).

Tamponamento cardíaco no lúpus eritomatoso sistêmico. Relato de quatro casos / Cardiac tamponade in systemic lupus erythematosus. Report of four cases

OBJECTIVE: To report and assess the incidence of cardiac tamponade in systemic lupus erythematosus as a cardiac manifestation of the disease. METHODS: We reviewed the medical records of 325 patients diagnosed with systemic lupus erythematosus according to the American Rheumatism Association and their complementary laboratory tests compatible with cardiac tamponade. RESULTS: In the 325 medical recors reviewed, we found 108 patients with pericardial effusions corresponding to 33.2 percent of the total and 54 percent of the patients studied in the active phase of the disease. Clinical assessment and transthoracic echocardiogram allowed the clinical diagnosis of cardiac tamponade in only 4 (1.23 percent) patients, 3 of whom were females, white, with ages ranging from 25 to 44 years. The pericardial fluid was hemorrhagic or serosanguineous with high levels of FAN and positivity for LE cells. In the treatment, we successfully used pericardicentesis associated with high doses of corticosteroids. In clinical and laboratory follow-up performed for a period of 3 years, neither recrudescence of the pericardial effusion nor evolution to constriction occurred. CONCLUSION: Even though rare (1.23 percent), cardiac tamponade in patients with systemic lupus erythematosus has a benign evolution when properly treated, according to our experience

Artrite gonocócica e resistência à penicilina / Gonocccal arthritis and penicillin resistence

A artrite gonocócica é uma das formas clínicas de apresentaçao da infecçao gonocócica disseminada (IGD), que ocorre em cerca de 0,5 por cento a 3 por cento dos casos de IGD com resistência da Neisseria gonorrhoeae à penicilina. Relatamos um caso de IGD com poliartrite causada por cepa resistente à penicilina. A identificaçao deste novo perfil de sensibilidade modifica uma das características mais típicas das cepas de N Gonorrhoeae causadoras da IGD e tem implicaçoes terapêuticas de ordem prática

El ecocardiograma en la evaluacion del compromiso cardíaco en las espondiloartropatias seronegativas / Echocardiography in the evaluation of cardiac involvement in seronegative spondylo-arthropathies

Se estudió compromiso cardíaco en pacientes con espondiloartropatías seronegativas utilizando la ecodopplercardiografía. De los 35 pacientes, 20 eran portadores de espondilitis anquilosantes, 10 de síndrome de Reiter y 5 presentaban artritis psoriásica. Se estudiaron 21 hombres y 14 mujeres de entre 17 y 68 años. La mayoría (65,71 por ceinto) no presentaba sintomatología cardíaca y 12 (34, 29 por ciento) eran oligosintomáticos. El sintoma predominante era palpitaciones. Todos fueron sometidos a un estudo ecodoppler y electrocardiográfico. El ecocardiograma mostró alteraciones en 19 pacientes (54, 29 por ciento), de ellos, el 28,50 por ciento era asintomáticos y el 25,71 por ciento presentaba síntomas cardiovasculares. El examen bidimensional fue el que detectó mayor número de alteraciones (17/19-84,47 por ciento), en tanto que el Doppler pulsado detectó 10 de los 19 (52,63 por ceinto) y unidimensional (9/19-47,36 por ciento). La válvula aórtica estaba comprometida en 10 pacientes (28,58 por ciento) de los que 7 tenían engrosamiento y 3 calcificación; la válvula mitral mostraba alteraciones en 11 enfermos (57,89 por ciento), engrosamientos en 9, calcificación en 1 y prolapso en 2. No se observaron alteraciones en las válvulas tricúspide y pulmonar. El Doppler pulsado detectó 4 insuficiencias aórticas y 3 mitrales. El compromiso valvular aórtico estaba presente en 8 (40 por ciento) de los pacientes con espondilitis anquilosante. El examen electrocardiográfico presentó una bradicardia sinusal, un bloqueo aurículo-ventricular de 1er grado, un bloqueo de rama derecha y 19 casos con ATc prolongado. El ecocardiograma demostró ser un método no invasivo de gran utilidad para revelar alteraciones cardíacas en los pacientes con espondiloartropatías seropnegativas

Aneurismas das artérias pulmonares na doenças de Behçet: regressäo após tratamento imunossupressor / Pulmonary artery aneurysms in Behçet's disease: regression after immunosuppressive treatment

A doença de Behçet é uma enfermidade sistémica com múltiplas manifestaçöes clínicas, cuja lesäo histopatológica subjacente é uma vasculite näo-específica. Aneurismas da artéria pulmonar e, mais raramente, manifestaçöes pleuropulmonares podem ser encontradas. Vários relatos estäo disponíveis na literatura sobre o uso de terapia imunossupressora nessas condiçöes. Apresenta-se um caso em que remissäo clínica e resoluçäo radiológica foram observadas após tratamento com corticosteróide e ciclofosfamida

Vasculite de Churg-Strauss / Churg-Strauss`s vasculitis

Tema livre apresentado no Simpósio Multidisciplinar sobre vasculites, na II Jornada Anual da Sociedade de Reumatologia do Rio de Janeiro, em 22-23 de agosto de 1997, no Colégio Brasileiro de Cirurgiöes, sobre o tema. Universidade do Estado do Rio de Janeiro - Hospital Universitário Pedro Ernesto - Serviço de Dermatologia e Sifilografia