Acute acalculous cholecystitis in juvenile systemic lupus erythematosus.

Small- and medium-vessel vasculitis is a common manifestation in systemic lupus erythematosus (SLE) and may occur in any organ. However, acute acalculous cholecystitis is a rare abdominal manifestation in SLE, especially in children. We report a case of a 12-year-old patient who initially presented with AAC and seizure. Follow-up investigation diagnosed SLE, and brain magnetic resonance imaging had hyperintense white matter lesions in cortico-subcortical regions. The patient was successfully treated with pulse methylprednisolone and cyclophosphamide without surgical intervention.

A comparison of the effect of alendronate and risedronate on bone mineral density in postmenopausal women with osteoporosis: 24-month results from FACTS-International.

OBJECTIVES: To compare alendronate 70 mg once weekly (OW) with risedronate 35 mg OW with respect to change in bone mineral density (BMD), biochemical markers and upper gastrointestinal (UGI) tolerability over 24 months. METHODS: This was a 12-month extension to the Fosamax Actonel Comparison Trial international study (FACTS). Postmenopausal women with osteoporosis randomly assigned to either alendronate 70 mg OW or risedronate 35 mg OW for the 12-month base study continued taking the same double-blind study medication. Efficacy measurements were BMD at the hip trochanter, lumbar spine, total hip, and femoral neck and levels of four bone turnover markers at 24 months. The primary hypothesis was that alendronate would produce a greater mean per cent increase from baseline in hip trochanter BMD at 24 months. RESULTS: Trochanter BMD increased significantly from baseline to month 24 in both groups, with a significantly larger increase with alendronate: adjusted mean treatment difference of 1.50% (95% confidence interval: 0.74%, 2.26%; p < 0.001). Similar results were seen at all BMD sites. Significant geometric mean per cent decreases (p < 0.001) from baseline were seen for all four bone turnover markers in both groups, with significantly larger decreases (p < 0.001) with alendronate: adjusted mean treatment differences ranged from 8.9% to 25.3%. No significant differences were seen in incidence of UGI or other adverse events. CONCLUSIONS: Alendronate 70 mg OW yielded significantly greater BMD gains and larger decreases in bone turnover marker levels than risedronate 35 mg OW over 24 months, with no difference in UGI tolerability.

Prognostic factors of low bone mineral density in systemic sclerosis.

OBJECTIVE: To analyse the results of bone densitometry in patients with systemic sclerosis (SSc), evaluating the prognostic factors of low bone mineral density (BMD) in fertile and postmenopausal patients, and comparing to a control healthy group. METHODS: Cross-sectional study analysing 61 female SSc patients, aged 25 to 51 years, who performed a bone densitometry using dual x-ray absorptiometry. BMD values (lumbar spine, femoral neck, Ward and trochanter) infertile and postmenopausal patients were compared according to SSc clinical variant (limited and diffuse), race, previous use of drugs (corticosteroids and cyclophosphamide) and bone mass index (BMI). These results were compared with 47 fertile and 60 postmenopausal healthy women; multivariate linear regression analysis was used to study the influence of the variables of interest in the BMD results. RESULTS: Twenty-seven SSc patients presented osteopenia and 14 densitometric osteoporosis. No statistical association was found between BMD values and SSc clinical variants, race and previous use of corticosteroids and cyclophosphamide, in the fertile and in the postmenopausal groups. Fertile SSc patients were paired by age and race with the control group, but BMI (p = 0.035) was significantly lower in the SSc group. BMD values of lumbar spine (p = 0.070, statistical trend), femoral neck (p = 0.003), Ward (p < 0.001) and trochanter (p = 0.003) were significantly lower in the SSc group. Postmenopausal SSc patients were paired by age and race with the control group, but BMI (p < 0.001) was also significantly lower in the SSc group. Age at menopause (p = 0.006) was also significantly lower and time from menopause (p < 0.001) was significantly higher in the SSc group. BMD values of femoral neck (p < 0.001), Ward (p < 0.001) and trochanter (p = 0.001) were significantly lower in the SSc group. Multivariate linear regression analysis showed that BMI was the main variable influencing BMD in the fertile and postmenopausal groups. CONCLUSION: In the present study, BMD results in fertile and postmenopausal SSc patients were independent of the SSc clinical variants, race and previous use of corticosteroids and cyclophosphamide. A low BMD in appendicular sites was observed infertile and postmenopausal SSc patients when compared to a control healthy group, associated to a low BMI.

Neuroimaging findings in scleroderma en coup de sabre.

OBJECTIVES: To describe the neuroimaging and clinical findings in patients with localized scleroderma en coup de sabre (LScs). METHODS: Patients with LScs were evaluated by high-resolution MRI and CT. The authors performed three-dimensional reconstructions of MRI and CT scans to evaluate brain and bone structures. RESULTS: Nine patients with LScs were evaluated (five women), with ages ranging from 6 to 53 years (mean, 30.7 years). Brain CT showed bone deformities with thinning of the skull under the skin lesions in six patients. MRI scans showed focal atrophy and blurring of the gray-white matter interface localized under the skin lesion in all patients. In three patients it was associated with hyperintense signal on fluid-attenuated inversion recovery (FLAIR) and T2-weighted images. Follow-up MRI showed extension of the brain lesion in one patient; in the remaining patients, the lesion did not progress. Four of the nine patients had partial epilepsy. One had surgery for management of refractory seizures, and pathologic findings indicated a focal inflammatory process. CONCLUSION: Localized scleroderma en coup de sabre is associated with focal, and in some progressive, brain lesions underlying the skin atrophy. Epilepsy, when present, is related to these brain lesions. Imaging findings and histopathology indicated that the process, most likely focal inflammatory, may be progressive.

Undifferentiated spondyloarthropathies: a 2-year follow-up study.

The aim of the study was to analyse the 2-year follow-up of a series of patients with the diagnosis of undifferentiated spondyloarthropathy (uSpA). A prospective study was carried out analysing 68 patients with symptomatic uSpA who fulfilled the European Spondylarthropathy Study Group (ESSG) criteria for seronegative spondyloarthropathies (SpA) and were aged between 18 and 50 years. Inclusion criteria included inflammatory low back pain (ILBP) (without radiographic sacroiliitis), asymmetric oligoarthritis (predominantly affecting large joints in the lower limbs) and heel enthesopathies (Achilles tendinitis and/or plantar fasciitis). Imaging methods included pelvic radiography (at study entry and after 2 years) and calcaneal radiography (at study entry). There was a predominance of male gender (78%), caucasoid race (72%) and positive HLA-B27 (54%), with a mean age of 31 years and mean disease duration of 5 years. The first disease manifestations were ILBP (49%), asymmetric oligoarthritis (35%) and heel enthesopathies (16%). A positive family history of a definite SpA was mentioned by 9% of the patients. Seventeen patients (25%) scored 5 points in the Amor set of SpA criteria; logistic regression analysis showed that HLA-B27, heel enthesopathy and asymmetric oligoarthritis were significantly associated with Amor criteria > or = 6, whereas ILBP was associated with Amor criteria <6. Male sex was associated with heel enthesopathies (p = 0.041) and ankle involvement (p = 0.015). Caucasoid race was associated with ILBP (p=0.015) and buttock pain (p = 0.047). Positive HLA-B27 was associated with wrist involvement (p=0.019) and Amor criteria > or = 6 (p=0.001). After a 2-year follow-up the following outcomes were observed: uSpA 75%; disease remission 13%; ankylosing spondylitis 10%; psoriatic arthritis 2%. Logistic regression analysis showed that buttock pain and positive HLA-B27 (trend) were statistically associated with progression to a definite SpA. In conclusion, uSpA can represent a provisional diagnosis in the group of SpA and a systematic follow-up is necessary in order to better establish the different patterns of the disease.

Bone mineral density, vitamin D and anticonvulsant therapy.

UNLABELLED: The aim of this study was to assess bone mineral density and vitamin D metabolism in patients on chronic anticonvulsant therapy. METHODS: Sixty-nine men, outpatients on chronic anticonvulsant therapy, who had been treated for at least 5 years, were studied, comparing them to thirty healthy controls. Bone mineral density was measured as well as serum levels of calcium, ionized calcium, alkaline phosphatase, PTH, 25-hydroxycholecalciferol and 1, 25-dihydroxycholecalciferol. RESULTS: No differences in bone mineral density, serum levels of vitamin D and intact-PTH were observed between patients and controls. Bone mineral density was not associated with chronic anticonvulsant therapy. CONCLUSION: Those adult patients who were on chronic anticonvulsant therapy and who lived in low latitude regions had normal bone mineral density as well as vitamin D serum levels.

Gynaecologic history in systemic sclerosis.

The aim of the study was to analyse the gynaecologic history of 150 Brazilian patients with systemic sclerosis (SSc) by comparing the outcome of the pregnancies before and after disease onset and in the two clinical variants of SSc, as well as to assess the effects of the pregnancy on the progress of the disease. A retrospective analysis was carried out of 150 female SSc patients, more than 18 years old, who attended the outpatient clinic of the Unit of Rheumatology of the State University of Campinas. The patients were questioned about the number of pregnancies, deliveries (full-term infants, premature births and twins) and fetal deaths (spontaneous abortions and perinatal deaths). These data were subdivided into pregnancies before and after SSc onset. In those gestations started after disease onset the patients were questioned about the evolution of SSc during the pregnancy. The patients were also asked about dyspareunia and the age at menopause. Thirty-two patients (21 %) had never been pregnant, and only five of them were considered infertile. One hundred and eighteen patients (79%) had a total of 406 pregnancies, with an average of 3.4 per patient; there were 364 pregnancies before and 42 after SSc onset. There were 58 fetal deaths (14% of the pregnancies), 50 of these occurring before and eight after disease onset; 55 were spontaneous abortions and the other three were perinatal deaths. The fertility rate was higher in the limited SSc (3.6) than in the diffuse SSc patients (3.1), although the percentage of fetal deaths and the evolution of SSc during the pregnancy were similar in the two clinical variants. In the pregnancies that occurred after the onset of SSc, the clinical course remained stable in 72% of the cases, worsened in 14% and improved in 14%. Dyspareunia was mentioned by 49 patients (37% of those with an active sexual life). Menopause was reported by 72 patients, predominantly with limited SSc (61 patients). The fertility rate in the postmenopausal SSc patients was 3.9, similar to that observed in general postmenopausal population in Brazil. The analysis of the gynaecologic history in this series of SSc patients showed no increased risk in infertility or spontaneous abortions. The fertility rate in the two SSc clinical variants was higher than that observed in the local global population. Most of the patients who became pregnant after the onset of SSc showed no signs of worsening during the course of the disease.

Weismann-Netter-Stuhl syndrome: first Brazilian case reports.

Weismann-Netter-Stuhl syndrome was first described in 1954 and is defined by an anterior curvature of the bones of the lower limbs, usually bilateral and symmetrical. Since its initial description, 82 cases were reported, including only 14 pediatric patients. The authors report two cases of this syndrome. One patient was an adult who presented with almost all the characteristic features of the disease. The second case was a 12-year-old girl who also presented with severe bone deformities of the upper limbs. Weismann-Netter-Stuhl syndrome is probably more common than previously reported and must be included in the differential diagnosis of rickets/osteomalacia, congenital syphilis and some cases of Paget's disease.

Rhythmic external compression of the limbs: a method for healing cutaneous ulcers in systemic sclerosis.

OBJECTIVE: To evaluate the usefulness of rhythmic external compression (REC) of the limbs on the healing of ischemic cutaneous ulcers in systemic sclerosis (SSc). METHODS: A prospective study analyzing 17 patients with SSc with symptomatic ischemic cutaneous ulcers in the limbs of more than 4 weeks' duration, who submitted to 20 sessions of REC, each session of one hour duration, 3 times a week. Patients were assessed at study entry, at the end of REC sessions, and at 30, 60, and 90 days after treatment. We also conducted a retrospective analysis of the outcome of ischemic vascular ulcers in a group of 20 patients with SSc who did not undergo REC. RESULTS: Twenty-eight ischemic vascular ulcers were submitted to REC. There was complete healing of 20 ulcers (71%) at the end of REC sessions. The healing was statistically more frequent in the distal ulcers (fingers and toes) (p = 0.0289), independent of SSc variant. There was a statistically significant correlation between pain resolution until the 15th session of REC and future ulcer healing (p < 0.0001). At followup, there were relapses in 2 ulcers 30 days after treatment. In the 20 patients with SSc who did not undergo REC, at followup, after 90 days of conventional treatment, there was healing of 7 ulcers (35%). CONCLUSION: REC could represent a therapeutic option in the treatment of ischemic cutaneous ulcers in SSc.

Tamponamento cardíaco como manifestaçäo predominante no lúpus eritematoso sistêmico / Cardiac tamponade as the predominant manifestation in systemic lupus erythematosus

Os autores relatam as características clínicas, o diagnóstico e o seguimento de uma paciente com lúpus eritematoso sistêmico (LES), em que o tamponamento cardíaco foi a principal manifestaçäo. Discute-se a ausência de alguns sinais de tamponamento cardíaco, com hipofonese de bulhas cardíacas e taquidispnéia, bem como a baixa freqüência de tamponamento cardíaco no LES

Doenca mista do tecido conjuntivo.Aspectos clinicos e laboratoriais. / Mixed connective tissue disease. Clinical and laboratorial aspects

Os autores apresentam 19 casos de doenca mista do tecido conjuntivo (DMTC) relatando dados clinicos e laboratoriais mais frequentemente observados.Artralgia (100%) artrite (57,6%), fenomeno de Raynaud (84, 3%), edema em "luva" de maos (57,6%) foram as principais manifestacoes clinicas.A associacao de lupus eritematoso sistemico (LES) e esclerose sistemica progressiva (ESP) ocorreu em oito casos, LES e dermatopolimiosite (DMP) em cinco. LES e artrite reumatoide (AR) em quatro, ESP e DPM em um e AR e DPM em outro.A presenca de FAN e hipergamaglobulinemia foram as alteracoes laboratoriais mais frequentes, 15 e 12 casos respectivamente. Discutem as variacoes das manifestacoes clinicas, que apresentaram comportamento heterogeno, como tambem as oscilacoes dos titulos e padroes do anti-ENA observados em alguns casos durante o periodo de seguimento

Acromegalia e condrocalcinose articular difusa. / Acromegaly and diffuse articular chondrocalcinosis

Os autores apresentam um caso de acromegalia com condrocalcinose e comentam sobre a revisao feita nos outros cinco casos acompanhados pelo servico de Endocrinologia; a associacao e raramente descrita e tem algumas implicacoes clinicas, como a possibilidade de crises de pseudogota no stress e pos-cirurgico, ou a continuacao das artralgias, tipo pseudoartrose, mesmo apos o tratamento adequado da patologia

Alteracoes osseas na angiomatose congenita. Relato de um caso de angiomatose osteo-hipotrofica. / Bone changes in congenital angiomatosis. Report of a case of osteohypotrophic angiomatosis

Os autores apresentam as principais alteracoes osseas na angiomatose congenita relatando sua frequencia, a proposito de um caso que evoluiu para atrofia ossea. Conceitua-se a angiomatose osteo-hipotrofica como fistula arteriovenosa que envolve os ossos e tecidos moles, resultando em encurtamento do mebro envolvido. Discutem-se as hipoteses propostas para o diagnostico diferencial

Determinacao do fenotipo de acetilacao da isoniazida em lupus eritematoso sistemico. / Determination of the acetylator phenotype of isoniazid in systemic lupus erythematosus

O fenotipo de acetilacao da isoniazida foi determinado em 20 pacientes com lupus eritematoso sistemico (LES). Dez pacientes (50%) eram acetiladores lentos de dez outros acetiladores rapidos. Nao houve correlacao do fenotipo acetilador e manifestacoes clinicas, padrao de fator antinucleo ou alteracoes laboratoriais. O fenotipo acetilador lento nao parece ser suficiente para faciliar a expressao clinica do LES

Evolucao clinica e conduta terapeutica na tuberculose osteoarticular. / Clinical course and therapeutic management in osteoarticular tuberculosis

Os autores apresentam dez casos de tuberculose osteoarticular, dos quais a coluna foi o local mais frequentemente acometido (sete), sendo o segmento lombar em quatro e segmento dorsal em tres. Em tres pacientes manifestou-se como monoartrite, dois de punho e um de joelho. Um dos casos revelou acometimento concomitante de coluna e punho. Tuberculose pulmonar ativa estava presente em quatro casos. Em quatro casos evidenciou-se envolvimento neurologico atraves de manifestacoes sensitivo-motoras e alteracoes de esfincteres.Os autores comentam a boa evolucao observada com tratamento conservador, mesmo nos casos que evidenciavam envolvimento neurologico

Sacroileite por condrocalcinose. / Sacroileitis due to chondrocalcinosis

Os autores apresentaram o seguimento clinico de um paciente acometido de condrocalcinose, cuja manifestacao inicial foi uma coxalgia a esquerda de ritmo inflamatorio e dor lombar na area de projecao da sacroiliaca esquerda. A avaliacao radiologica permitiu um diagnostico de condrocalcinose, tendo o inventario osteoarticular e os parametros laboratoriais descartado outras condicoes morbidas associadas. Trata-se de manifestacoes bastante peculiar e inusitada, devendo integrar, contudo, o conjunto de situacoes relacionadas ao diagnostico diferencial nas lombalgias

Forma espinal da neurofibromatose de von Recklinghausen. A proposito de um caso. / Spinal form of von Recklinghausen neurofibromatosis. Report of a case

Os autores apresentam e discutem as manifestacoes clinicas e radiologicas de uma paciente acometida por doenca de von Recklinghausen. Sintomas e sinais sugestivos de compressao mielorradicular e evidencias radiologicas de erosao ossea vertebral permitiram o diagnostico da forma de tumor espinal

Acometimento pulmonar nas dermatopolimiosites. / Pulmonary involument in dermatopolymyositis

Os autores apresentam as manifestacoes pulmonares em 18 pacientes acometidos por dermatopolimiosite, comentando os aspectos clinicos radiologicos e funcionais. Quatro pacientes eram do sexo mesculino e 14 do sexo feminino, faixa etaria, em media, de 29 anos, tempo de doenca variando entre 6 e 24 meses. Todos apresentavam queixa de astenia, angustia respiratoria ou dispneia conquanto apenas 11 apresentassem evidencias radiologicas de pneumofibrose intersticial. O estudo da funcao pulmonar mediante provas espirometricas revelou alteracoes ventilatorias do tipo restritivo em 10 pacientes, nao se observando, entretanto, uma correlacao direta entre estes achados e as imagens radiologicas. O seguimento de nove necropsias evidenciou alteracoes pulmonares, caracterizando diferentes graus de pneumonite intersticial e pleurite eventualmente associadas a manifestacoes de carater infeccioso.Os autores apresentam, nesses casos, correlacao entre os parametros clinicos diagnosticos e evolutivos e os achados anatomopatologicos

Hipogamaglobulinemia em dermatopolimiosite. / Hypogammaglobulinaemia in dermatomyositis

Os autores relatam um caso de dermatomiosite que, apos dezessete meses de evolucao desenvolveu hipogamaglobulinemia.Apesar da terapeutica com prednisona (60 a 12 mg/ dia) e metotrexato (25mg/semana), evoluiu ao obito com quadro de insuficiencia respiratoria grave associado com infeccao pulmonar. O estudo histoplatologico revelou foliculos linfoides hipotrofiados tanto da cortical dos nodulos linfoides como da polpa branca do baco, ou seja, areas relativas aos linfocitos B, justificando a presenca de hipogamaglobulinemia