Rupture of celiac trunk aneurysm in patient with Behçet Disease.

We report a case of a ruptured aneurysm of the celiac trunk in a 32-year-old, male patient with Behçet Disease (BD). Aneurysm resection was performed and the patient is well during a follow up of 32 months. To our knowledge, this is the first reported case of a ruptured celiac trunk aneurysm successfully treated in a patient with BD. RESUMO Relatamos o caso de um aneurisma roto do tronco celíaco em um paciente de 32 anos, do sexo masculino, portador de Doença de Behçet (DB). A ressecção do aneurisma foi realizada e o paciente está bem, com acompanhamento de 32 meses. Até onde sabemos, este é o primeiro caso relatado de um aneurisma do tronco celíaco roto tratado com sucesso em um paciente com DB.

Rupture of celiac trunk aneurysm in patient with Behçet Disease / Ruptura de aneurisma de tronco celíaco em paciente com Doença de Behçet

ABSTRACT We report a case of a ruptured aneurysm of the celiac trunk in a 32-year-old, male patient with Behçet Disease (BD). Aneurysm resection was performed and the patient is well during a follow up of 32 months. To our knowledge, this is the first reported case of a ruptured celiac trunk aneurysm successfully treated in a patient with BD.

RESUMO Relatamos o caso de um aneurisma roto do tronco celíaco em um paciente de 32 anos, do sexo masculino, portador de Doença de Behçet (DB). A ressecção do aneurisma foi realizada e o paciente está bem, com acompanhamento de 32 meses. Até onde sabemos, este é o primeiro caso relatado de um aneurisma do tronco celíaco roto tratado com sucesso em um paciente com DB.

Tumor-induced osteomalacia: the importance of early diagnosis / Osteomalácia induzida por tumor: a importância do diagnóstico precoce

Tumor-induced osteomalacia is a rare paraneoplasic syndromethat can be completely cured with the removal of the culprittumor. This study described the clinical history of a patientaffected by tumor-induced osteomalacia. The patient was a57-year-old female who sought hospital due to intense andprogressive pain in the lower limbs and muscle weakness, aswell diffuse osteoporosis and a variety of pathologic fracturesat radiographs. Laboratory tests revealed hypophosphatemiawith hyperphosphaturia and raised the hypothesis of tumorinducedosteomalacia. Whole-body technetium-99m octreotidescintigraphy revealed the presence of a focal area of radiotraceruptake in the medial region of the left tarsus. After tumorexcision, there was a rapid correction of serum phosphorus,reduction of musculoskeletal complaints and evidence of bonehealing. Despite the diagnosis and treatment, the patient hadan unfavorable clinical outcome; she developed sepsis frompulmonary focus, evolving into refractory septic shock anddeath. We stress the need for greater recognition of tumorinducedosteomalacia as a cause of clinical bone pain, fractures,osteopenia and muscle weakness, superimposed on thecharacteristic biochemical profile with hypophosphatemia andrelative hyperphosphaturia. Greater awareness of the disease willallow earlier diagnosis and ultimately a greater curative potentialfor patients afflicted with this syndrome.(AU)

Osteomalácia induzida por tumor é uma síndrome paraneoplásicarara que pode ser curada completamente com a ressecção dotumor causador. Este estudo descreveu a história clínica de umapaciente afetada pela osteomalácia induzida por tumor. Paciente,de 57 anos, deu entrada no hospital por dor em membros inferiores,e fraqueza muscular intensa e progressiva, assim comoosteoporose difusa e fraturas patológicas. Exames laboratoriaisevidenciaram hipofosfatemia com hiperfosfatúria e levantarama hipótese de osteomalácia induzida por tumor. Cintilografia detodo corpo com tecnécio-99m revelou a presença de área focalde captação do radiofármaco na região medial do tardo esquerdo.Após a ressecção do tumor, houve rápida correção do fósforosérico, redução das queixas musculoesqueléticas e evidência decalo ósseo. Apesar de diagnóstico e tratamento, a paciente apresentouum desfecho clínico desfavorável, desenvolvendo sepsede foco pulmonar, choque séptico e evoluindo a óbito. Nós enfatizamosa necessidade de maior reconhecimento da osteomaláciainduzida por tumor como causa de dor óssea, fraturas patológicas,osteopenia e fraqueza muscular, superpostos a um perfilbioquímico característico, com hipofosfatemia e hiperfosfatúriarelativa. Maior alerta sobre a doença permitirá um diagnósticomais precoce e maior potencial curativo aos pacientes afetadospor essa síndrome.(AU)

Necrose digital em paciente com lúpus eritematoso sistêmico e esclerose sistêmica tratada com inibidores da fosfodiesterase / Digital necrosis treated with phosphodiesterase inhibitors in a patient with connective tissue disease

Os inibidores da fosfodiesterase têm sido introduzidos, nos últimos anos, como novos agentes farmacológicos no tratamento dos pacientes com fenômeno de Raynaud e isquemia digital. Será descrito o caso de uma paciente com lúpus eritematoso sistêmico e esclerose sistêmica limitada apresentando fenômeno de Raynaud grave e necrose digital refratária à terapia. A paciente obteve excelente resposta à associação de imunossupressão e sildenafil.

The phosphodiesterase inhibitors have been used recently for the treatment of Raynaud's phenomenon and digital ischaemia. We report the case of a patient affected by systemic lupus erythematosus and limited systemic sclerosis who presented severe Raynaud's phenomenon with digital necrosis despite treatment. The patient presented an excellent response to the association of immunosuppressant therapy and sildenafil.

Physical activity prevents bone loss in premenopausal women with rheumatoid arthritis: a cohort study.

The aim of the study was to compare the bone loss and the influence of physical activity between premenopausal women with rheumatoid arthritis (RA) and healthy women. A total of 71 patients with RA and 29 healthy premenopausal women with the criteria of the American College of Rheumatology for RA were followed for 2 years. Of these 85% were Caucasian, aged 38 +/- 6.6 years and with a duration of disease of 88 +/- 50 months and 48 (71.8%) used GC, mean daily dose, 7.3 +/- 3.5 mg. There was a reduction in the T-score of the femoral neck (P = 0.04) and in the Ward region (P = 0.05) in RA. Through logistic regression, it was found that sedentarism was a risk factor for osteopenia in RA, with relative risk of 1.6 (IC = 1.238-1.734). Moderate physical activity reduced the risk of osteopenia by 50%. Sedentarism and low weight are the main factors associated with bone loss. Physical activity reduces bone loss. Early preventive and therapeutic measures must be encouraged.

Rheumatoid arthritis: evidence for bone loss in premenopausal women.

OBJECTIVE: To assess bone mineral density (BMD) status in patients with rheumatoid arthritis (RA). METHODS: Rheumatoid factor-positive premenopausal women with RA meeting the American College of Rheumatology criteria were enrolled. Exclusion criteria included diseases or drugs that affect BMD, except for glucocorticoids, and smoking. Evaluation consisted of a questionnaire, physical examination, and laboratory tests. Lumbar spine and right proximal femur BMD were measured with a DPX-Lunar DEXA scanner. Data were analyzed by Student t test, chi-square, and multivariate analysis. RESULTS: We studied 78 patients with RA and 39 controls; 82% were Caucasian, with mean age 35.5 +/- 6.7 years, and mean disease duration 48 +/- 51 months. Among patients, 74.4% had been treated with glucocorticoids, with a mean daily dose of 9.7 +/- 5.9 mg. Mean lumbar spine BMD was 1.157 +/- 0.124 g/cm2 in the RA patients, and 1.223 +/- 0.147 g/cm2 in controls (p < 0.01). Mean right proximal femur BMD did not differ significantly. Lumbar spine osteopenia correlated with "no physical activity at work" status, low body weight, and duration of glucocorticoid therapy. Femoral neck osteopenia correlated with "no physical activity at work" status, Steinbrocker class III, erosions of the hands, and high erythrocyte sedimentation rate (ESR). Trochanteric osteopenia correlated with "no physical activity at work" status, erosions on hand radiographs, low body weight, high ESR, and anemia. CONCLUSION: Patients with RA of relatively short disease duration already exhibited significantly lower lumbar spine BMD. The identification of prognostic markers for bone loss in patients with RA should not only prompt early therapeutic intervention, but also facilitate early preventive measures.

Association between systemic lupus erythematosus, rheumatoid arthritis, hyperprolactinemia and thyroid autoantibodies.

BACKGROUND: Hyperprolactinemia (hyperPRL) has been associated with autoimmune rheumatic disorders and the presence of thyroid autoantibodies (tAb). The interrelation between these variables was the focus of this prospective study. METHODS: The study assessed six groups of individuals: 26 with systemic lupus erythematosus (SLE), 20 with rheumatoid arthritis (RA), 28 with tAb (tAb+), 14 with untreated hyperprolactinemia (hyperPRL), 10 with treated hyperPRL, and a control group (n = 28). Prolactin (PRL), free thyroxin, TSH, antibodies against thyroglobulin (TgAb), thyroid microsomal antigen (MsAb) and/or thyroid peroxidase (TPOAb) were determined in all patients. Those with hyperPRL had macroprolactin investigated by the polyethylene glycol (PEG) precipitation method. RESULTS: PRL (ng/mL) levels in the SLE, RA, and tAb+ groups were, respectively, 21.3 +/- 12.6, 11.5 +/- 7.4, and 12.5 +/- 8.6, and were significantly greater in the SLE group (p = 0.006) than in the controls (12.5 +/- 6.5) and in the other groups. Five patients had hyperPRL: three with SLE, one with RA, and one with tAb+. Macroprolactinemia was detected in three of the untreated hyperprolactinemic patients and in the hyperprolactinemic patient of the tAb+ group. Positivity for any of the tAb was 15% in the SLE, 15% in the RA, 57.1% in the untreated hyperPRL, 10% in the hyperPRL on treatment, and 3.6% in the control group. The presence of antibodies was significantly more frequent in the untreated hyperPRL group than in the control group (p = 0.001). CONCLUSIONS: The results indicate that the PRL level is higher in SLE patients and that in the presence of hyperPRL there is increased prevalence of antithyroid antibodies, evidencing the association of PRL and autoimmunity and pointing to the appropriateness of assessing and monitoring the progress of these markers in patients affected by these disorders.

Artrite reumatóide e depressão / Rheumatoid arthritis and depression

A depressão é uma doença freqüentemente subdiagnosticada e dificilmente bem manejada em pacientes com artrite reumatóide(AR). É possível verificar que alterações psicopatológicas podem ser decorrentes da AR, ou, pelo menos, de uma estado de dor crônica. Quanto à etiologia, não há base científica consistente que permita afirmar que distúrbios psiquiátricos participem da fisiopatologia dessa doença e vice-versa, sendo necessária a realização de estudos mais criteriosos nesse sentido. Todavia, as repercussões que a depressão pode provocar na qualidade de vida e prognóstico dos pacientes com AR já justificam por si o dever dos clínicos em atentar para o diagnóstico dessa co-morbidade e para o adequado manejo dessa situação. Nesta revisão bibliográfica são abordados fatores etiológicos, diagnóstico e o manejo de pacientes portadores de Ar e transtornos do humor

Associaçäo de mielite transversa e lúpus eritematoso sistêmico: relato de dois casos / Association of transverse myelitis and systemic lupus erythematosus: report of two cases

Säo relatados dois casos de lúpus eritematoso sistêmico (LES) com mielite transversa (MT) e revisados 66 casos descritos previamente na literatura, destacando apresentaçäo clínica, técnicas diagnósticas e terapia empregada. A maioria dos pacientes era do sexo feminino, com idade entre 10 e 62 anos, como os dois casos apresentados. Houve maior incidência de MT no primeiro ano de evoluçäo e os achados clínicos de LES mais comumente observados foram artrite, nefrite e leucopenia. Näo foi possível estabelecer relaçäo entre MT e anticorpo antifosfolipídio. O tratamento com metilprednisolona e ciclofosfamida proporcionou os melhores resultados e o tempo de início do tratamento mostrou-se um fator determinante para o prognóstico neurológico

Alteraçöes oculares em pacientes com lúpus eritematoso sistêmico / Ocular changes in patients with systemic lupus erythematosus

Para determinar a prevalência de alteraçöes oculares em pacientes com LES, os autores estudaram 60 pacientes do HCPA através de questionário objetivo e exame oftalmológico completo. Cinqüenta e cinco pacientes (91,6%) apresentavam algum tipo de sintoma ocular, sendo ardência (53,3%) e incômodo com a luz (40%) os mais freqüentes. Somente nove casos tiveram exame oftalmológico normal. Em 23 casos (38,3%) foi identificada ceratoconjuntivite sicca (CCS). Näo se identificaram exsudatos algodonosos. CCS foi mais prevalente nos casos com maior tempo de doença (LES) e menos prevalente nos casos com idade menor que 27 anos

Alteraçöes músculo-esqueléticas em pacientes com SIDA / Musculoskeletal changes in patients with AIDS

O envolvimento músculo-esquelético em pacientes com SIDA tem sido descrito como freqüente na literatura. Para determinar o tipo de acometimento e sua prevalência nos pacientes do HCPA, os autores realizaram um estudo transversal com 42 pacientes. Em 45,2% dos casos havia alguma alteraçäo músculo-esquelética (artralgias, mialgias, fraqueza), que em todos os casos podiam ser justificadas por neoplasia avançada ou infecçäo secundária. O único caso de monoartrite apresentava sarcoma de Kaposi, sendo identificadas células tumorais no líquido sinovial

Artrite séptica por "Streptococcus faecalis" / Septic arthritis by \"Streptococcus faecalis\"

Os autores relatam o nono caso de artrite séptica por Streptococcus faecalis na literatura, com acometimento de punho, isolamento precoce do germe e boa resposta à terapêutica