Erectile dysfunction in systemic sclerosis

The aim of this report is to analyze the incidence of erectile dysfunction (ED) and the usefulness of the International Index of Erectile Function (IIEF) on the investigation of ED in men with systemic sclerosis (SSc). Fifteen male SSc patients were asked about the presence of ED symptoms and were submitted to the IIEF questionnaire, after obtaining informed consent. Their medical data were reviewed in the search of other possible causes of ED. The studied patients were predominantly Caucasian and had diffuse SSc. ED was referred by 67% of them, and it was first noted after an average of 29 months of SSc onset; four patients referred concomitant onset of ED and SSc. All men with ED detected by IIEF questionnaire also reported ED when directly asked, and all men who affirmed having ED were also identified by IIEF. There were no clear association between ED and extent of cutaneous involvement, visceral involvement, drugs, total skin score and disease duration. Five patients with ED also had other causes of impotence. ED affects a high percentage of male SSc patients, and the IIEF may be a valid instrument in its evaluation. The high incidence of ED in young SSc patients and the occasional concomitance of the onset of SSc and ED warrants further evaluation of a causal association.O objetivo deste trabalho é analisar a prevalência da disfunção erétil (DE) e a utilidade do Índice Internacional de Função Erétil (IIFE) na investigação da DE em pacientes com esclerose sistêmica (ES). Quinze homens com ES foram questionados acerca dos sintomas da DE e submetidos ao questionário de IIFE. Seus prontuários foram revisados para identificar outras possíveis causas da DE. Os pacientes estudados eram predominantemente caucasóides e tinham ES difusa. DE foi referida por 67% dos pacientes, iniciando-se, em média, 29 meses após o início da ES; quatro pacientes relataram início concomitante da DE e da ES. O diagnóstico da DE pelo IIFE e por interrogação direta foi concordante em todos os pacientes. Não foi encontrada associação nítida entre DE e extensão do envolvimento cutâneo, envolvimento visceral, drogas, escore cutâneo total e duração da doença. Cinco pacientes com DE também tinham outras causas de impotência. A DE afeta um grande porcentual de homens acometidos por esclerose sistêmica e o IIFE pode ser um instrumento válido em sua avaliação. A alta incidência da DE em jovens com ES e a eventual concomitância do início da ES e da DE enfatizam a necessidade de estudos posteriores sobre uma provável relação causal.949

Coexistence of hypothyroidism and polymyositis

Patients with hypothyroidism frequently present with musculoskeletal complaints and elevated serum levels of muscle enzymes, like creatine kinase (CK). With adequate hormonal treatment, the maintenance of high values of CK and muscle symptoms are uncommon. This work reports a 47-year-old woman with hypothyroidism, proximal muscle weakness and increased CK, who didn't improved with levothyroxine treatment. The diagnosis of polymyositis was based on electromyography and muscle biopsy. After treatment with prednisone plus methotrexate and increase of the levothyroxine dose, there were a clinical improvement and a normalization of CK values.Pacientes com hipotiroidismo freqüentemente apresentam sintomas músculo-esqueléticos e aumento de enzimas musculares, como a creatinaquinase (CK). Com o tratamento hormonal adequado é incomum ocorrer grandes aumentos de CK e manutenção de queixas musculares. Este trabalho relata o caso de uma paciente com hipotiroidismo, queixa de fraqueza muscular proximal e aumento de CK, sem melhora clínica com uso de levotiroxina. O diagnóstico de polimiosite foi confirmado por eletroneuromiografia e biópsia muscular. Após tratamento com prednisona e metotrexato, além de adequação da dose de levotiroxina, houve melhora clínica significativa e normalização da CK.33033

Renal crisis as the initial clinical manifestation in systemic sclerosis sine scleroderma

Systemic sclerosis sine scleroderma (ssSSc) is a rare disorder, characterised by visceral involvement (usually peripheral vascular, gastrointestinal and pulmonary) in the absence of the characteristic skin fibrosis. The authors describe in this case report the rare occurrence of sclerodermic renal crisis (SRC) as the initial clinical manifestation in a patient with ssSSc.A esclerose sistêmica (ES) sine scleroderma é uma doença rara, caracterizada pelo acometimento visceral (geralmente gastrintestinal, pulmonar ou vascular periférico) da ES, na ausência da característica fibrose cutânea. Os autores descrevem um caso da rara ocorrência da crise renal esclerodérmica como manifestação inicial em uma paciente com ES sine scleroderma.878

Characterization And Outcome Of Uveitis In 350 Patients With Spondyloarthropathies.

This retrospective study analyzed 350 patients with the diagnosis of spondyloarthropathies (SPA) (207 with ankylosing spondylitis (AS), 80 with undifferentiated spondyloarthropathies (USPA) and 63 with psoriatic arthritis (PsA)) attended at a tertiary referral hospital for a minimum period of 5 years. All the patients presented complete clinical (axial and peripheral involvement, heel enthesopathies, extra-articular manifestations) and radiologic (sacroiliac, lumbar, dorsal and cervical spine) evaluation. HLA-B27 and respective alleles were searched. These data were compared with the occurrence of uveitis during the follow-up of the SPA patients. Thirty AS patients (14.5%) presented 55 episodes of acute anterior uveitis; there was statistical association between uveitis and juvenile-onset AS (P = 0.0094) and achillean (P = 0.0003) and plantar (P = 0.0067) enthesopathies; one AS patient presented a single episode of posterior uveitis, associated to tuberculosis. Seven USPA patients (8.8%) presented 13 episodes of acute anterior uveitis; it was not observed statistical association with any variable; one patient presented a single episode of posterior uveitis, associated to toxoplasmosis. Five HLA-B27 positive PsA patients (8%) presented 13 episodes of acute anterior uveitis. All the 26 positive HLA-B27 SPA patients with anterior uveitis tested for the HLA-B27 alleles were HLA-B*2705. No patient presented ophthalmologic severe sequelae of the anterior uveitis. Concluding, anterior uveitis was associated to the juvenile onset of the disease and to the enthesophatic involvement of the lower limbs in AS patients. The HLA-B*2705 allele was predominant in the anterior uveitis patients, whilst posterior uveitis was rare and associated to infectious disease.261143-

Hipoparatireoidismo primário manifestando-se com aumento de enzimas musculares

Os autores relatam o caso de um jovem de 18 anos de idade com fraqueza muscular generalizada, cãibras e crises convulsivas, tratado com corticosteróides por oito anos em decorrência de um diagnóstico de polimiosite, sem melhora clínica. Ao exame físico apresentava força muscular normal, ausência de atrofias musculares, diminuição de reflexos tendíneos profundos e presença do sinal de Chvostek e Trousseau. A hipótese diagnóstica inicial foi de miopatia metabólica e a investigação laboratorial revelou cálcio sérico e urinário diminuídos e dosagem de paratormônio (PTH) indetectável. O diagnóstico final foi de hipoparatireoidismo, tendo sido o paciente tratado com cálcio endovenoso. A tomografia de crânio demonstrou calcificações difusas, características da síndrome de Fahr. O paciente recebeu alta assintomático com carbonato de cálcio e colecalciferol