Acir Rachid Filho

Isolated hypoglossal nerve palsy: an unusual rare presentation in systemic lupus erythematosus

Histologycally tumor revealed bland spindle-shaped cells with indistinct pale eosinophilic cytoplasm and small hyperchromatic oval nuclei, ring cell likes were also observed (Fig 2A and 2B), ploemorphism and mitotic features (Fig 2C and 2D). The tumor cells were embedded in a variably fibrous or myxoid stroma that tended to alternate in different areas of the tumor. Immunohistochemical analysis of the tumor cells revealed diffuse expression of vimentin, CD99 (Fig 2E) and CD117 (Fig 2F). Ewing sarcoma was diagnosed. The tumor was resistant to different kinds of therapy.

Manifestação otológica localizada em paciente com granulomatose de Wegener

Wegeners granulomatosis (WG) is a rare, idiopathic granulomatous vasculitis, affecting small and medium-sized vessels, classically involving upper and lower respiratory tracts and kidneys. In the limited form of the disease, several other sites can be affected. We describe the case of a young woman who presented her disease with dyspnea, laryngeal stridor and hearing loss. Investigation revealed localized glottis involvement and a tympanum biopsy confirmed granulomatous vasculitis. The patient had a partial response with the treatment, with residual deafness. We performed a review concerning the focal manifestations of WG, focusing in the ear involvement.

Infliximabe no tratamento de uveíte posterior refratária em paciente com doença de Behçet

Permanent vision loss resulting from relapsing ocular inflammation occurs frequently in patients with Behcets disease, despite intensive and chronic immunosuppressive therapy. The authors report the case of a 35-year-old man with chronic bilateral posterior uveitis refractory to corticosteroids and immunosuppressive agents (methotrexate and cyclosporine) with a beneficial effect from treatment with infliximab.

c-ANCA-associated vasculitis in patients with ulcerative colitis: a case report ☆

The pulmonary manifestations of ulcerative colitis (UC) are rare and include inflammation of small and large airways, parenchymal disease and serositis among others. A substantial proportion of patients with inflammatory bowel disease, particularly those with ulcerative colitis presents positive ANCA, most p-ANCA pattern. We present a case of patient with ulcerative colitis, with positive c-ANCA, which progressed to hemoptysis associated with radiological findings consistent with pulmonary vasculitis.

Vasculite c-ANCA relacionada em paciente com retocolite ulcerativa: relato de caso*

The pulmonary manifestations of ulcerative colitis (UC) are rare and include inflammation of small and large airways, parenchymal disease and serositis among others. A substantial proportion of patients with inflammatory bowel disease, particularly those with ulcerative colitis presents positive ANCA, most p-ANCA pattern. We present a case of patient with ulcerative colitis, with positive c-ANCA, which progressed to hemoptysis associated with radiological findings consistent with pulmonary vasculitis.

Bullous systemic lupus erythematosus in a pregnant woman: a case report

Systemic lupus erythematosus (SLE) can cause numerous skin lesions. Despite being rare, lupus-specific bullous lesions demonstrate characteristic clinical and immunopathological features and require differential diagnosis among numerous bullous conditions that may overlap with SLE. The present study presents a case of bullous systemic lupus erythematosus (BSLE) in a pregnant woman.

Lúpus eritematoso sistêmico bolhoso em gestante: relato de caso

Systemic lupus erythematosus (SLE) can cause numerous skin lesions. Despite being rare, lupus-specific bullous lesions demonstrate characteristic clinical and immunopathological features and require differential diagnosis among numerous bullous conditions that may overlap with SLE. The present study presents a case of bullous systemic lupus erythematosus (BSLE) in a pregnant woman.

Função ovariana em mulheres lúpicas submetidas ao uso de ciclofosfamida em dois grandes centros de atendimento reumatológico de Curitiba, Estado do Paraná

PURPOSE: To evaluate the ovarian response after cyclophosphamide use (CPM) in patients with systemic lupus erythematosus (SLE) and to correlate the age and cumulative dose findings with changes in menstrual cycle and/or progression to ovarian failure (OF). METHODS: This was a cross-sectional, retrospective study of 50 patients with a diagnosis of SLE who used CFM with a clinical follow-up of at least 1 year. Included were patients aged 12-40 years, who had undergone chemotherapy for SLE control and who had regular menstrual cycles before the beginning of CPM treatment. Patients who discontinued follow-up, who were followed up for less than one year or who had irregular/absent menses before the beginning of CPM treatment were excluded. All women studied were submitted to an interview and a questionnaire containing questions about the pattern of the menstrual cycle before and after therapy, and about the gestational periods and contraception. We asked if the patients had been instructed about the side effects and consequences of CFM. Statistical analysis was performed using the Student t-test and the Mann Whitney, χ2 and nonparametric Kolmogorov-Smirnov tests. RESULTS: The mean age of the patients included in the study was 30.8 years and the mean age at the time of use of CPM was 25.3 years. After CFM, 24% of patients stopped menstruating, 28% returned to regular cycles and 48% continued to have irregular cycles. It was found that the patients who developed OF had longer disease duration (12.3 years) than those who did not develop it (8.9 years). Thirteen patients became spontaneously pregnant after CFM; however, 66% progressed to abortion. The mean age of the patients who used CFM and developed OF was 28.1 years. Amenorrhea occurred in 50% of those aged 31-40 years, in 22.2% of those aged 21-30 years and in 7.7% of those aged 12-20 years. Our study showed no statistical correlation between cumulative dose and OF, although cumulative doses greater than 11grams tended to promote some type of menstrual irregularity. CONCLUSION: SLE disease duration, age at the time of treatment and the highest cumulative doses are important predictors of OF after therapy with CFM. Pregnancy in lupus patients is more likely to evolve with abortion after the use of chemotherapy. It was seen that a small proportion of patients were aware of all the implications of the drug. Therefore, additional studies should be conducted for further knowledge and awareness of the importance of contraception and the preservation of ovarian tissue on the part of the medical community.

Sinovite hipertrófica familiar

Familial hyperthrophic synovitis is a rare condition, of familial origin, that must be remembered in the differential diagnosis of childhood arthritis. The authors report a case of a 7 years old boy with flexion finger deformities and painless joint effusion of the large joints since birth, without systemic involvement. The patient has a cousin with similar manifestations.