Gláucio Ricardo Werner de Castro

Arterite de Takayasu: tratamento com anti-TNF em uma casuística brasileira

The aim of this study was to describe clinical features and response to different therapeutic interventions, including anti-tumor necrosis factor (TNF) agents, in a case series of Takayasu arteritis (TA) from Brazil. A retrospective observational chart-review study was performed including all patients meeting the American College of Rheumatology TA classification criteria followed at the rheumatology outpatient clinic of a Brazilian university hospital. Fifteen patients were included, of which 14 (93.3%) were females, with a mean age of 29.6 years at diagnosis. Systemic hypertension (60.0%) and abolished upper limb pulses (53.3%) were the most common clinical features at the diagnosis. Subclavian and carotid arteries were the most commonly affected vessels. Twelve patients (80.0%) did not achieve sustained remission on therapy with corticosteroids alone and received immunosuppressive agents including methotrexate, azathioprine and cyclophosphamide. Surgical intervention was necessary and performed in 53.3% of cases. Three cases (20.0%) were refractory to corticosteroid plus diverse immunosuppressive therapy and were treated with anti-TNF agents, all of them with disease remission. In conclusion, a significant proportion of TA cases are refractory to traditional therapy. The use of anti-TNF agents may become a possible therapy for these patients.

Resolution of adalimumab-induced psoriasis after vitamin D deficiency treatment

Tumoral necrosis factor alpha blockers are very efficient in the treatment of many inflammatory systemic diseases, including rheumatoid arthritis and psoriasis. However, a paradoxical arouse of psoriasiform lesions may occur in a few patients taking anti-TNFα. The etiology of this rare side effect is still a mystery, and its treatment may be difficult. The authors report the resolution of adalimumab-induced psoriasis in a woman with rheumatoid arthritis after the use of high vitamin D3 doses for the treatment of vitamin D deficiency. This is the first report of resolution of anti-TNFα-induced psoriasiform lesions by high doses of vitamin D3 in a patient with rheumatoid arthritis and vitamin D deficiency. This case raises interesting questions on the role of vitamin D deficiency in the pathogenesis of this side effect and on the possible usefulness of high-dose vitamin D3 in its treatment.

Paget's disease of bone: analysis of 134 cases from an island in Southern Brazil: another cluster of Paget's disease of bone in South America.

Paget’s disease of bone (PDB) exhibits a marked geographic variation. In Brazil, the prevalence of PDB is unknown and only a few clinical data are available. The aim is to determine clinical, laboratory, imaging and response to treatment data in a large PDB case series in the city of Florianopolis, Brazil. We have performed a retrospective study based on charts reviews of all patients with PDB followed at the University Hospital of the Federal University of Santa Catarina and at five different private rheumatology outpatient offices in Florianopolis, between 1995 and 2009. One hundred and thirty-four patients with PDB were identified. Mean age at diagnosis was 63.2xa0±xa010.5xa0years, 67.2% were women, and 91.1% were Caucasian. Positive family history was reported in only 8.2%. Polyostotic disease was found in 75.0% of the cases, bone pain in 77.9%, and bone deformities in 15.9%. Higher levels of AP were significantly associated with polyostotic disease and skull involvement. Pelvic bones were the most frequently affected (53.7%). Complications included deafness in 8.2%, bone fractures in 3.0%, hydrocephalus in 2.2%, and cauda equina syndrome in 0.7% of the cases. Treatment with zoledronic acid achieved the best response with only 2.9% failing to respond adequately. According to literature data, PDB in South America seems to be characterized by an overall low prevalence, but with localized clusters with higher prevalence. The authors have described a cluster of PDB in Florianopolis, in Southern Brazil. Further properly designed studies are necessary to clarify the PDB epidemiology in South America.

Lesões destrutivas da linha média induzidas por cocaína com ANCA positivo mimetizando a granulomatose de Wegener

Chronic use of cocaine by inhalation may induce midline destructive lesions (CIMDL), which can sometimes be difficult to distinguish from the ear, nose and throat lesions of Wegeners Granulomatosis (WG). We describe the case of a 43-year-old female patient admitted with a two-year history of nasal obstruction and rhinorrhea. She had been diagnosed with WG for five months, being on prednisone and cyclophosphamide. On her physical examination, perforation of her nasal septum and palate was observed. Laboratory tests showed elevated acute phase proteins and a positive p-ANCA test. ELISA assays anti-proteinase 3 and myeloperoxidase were negative. The paranasal sinus computed tomography (CT) showed destruction of the nasal septum and palate, in addition to bilateral maxillary sinusitis. Chest CT was normal. Nasal mucosal biopsy revealed an inflammatory infiltrate, with neither granuloma nor vasculitis. When questioned, she admitted being a cocaine user for five years. Medical therapy and cocaine use were withdrawn. She has been followed up for six months and no other lesion or other organ symptoms occurred. Differential diagnosis in patients with midline destructive lesions can be very challenging. Evaluation should include enquiry about intranasal use of cocaine. Although ANCA testing does not clearly differentiate the ANCA found in some patients with CIMDL from those found in WG patients, the localized involvement and the biopsy findings non-characteristic of small vessel granulomatous vasculitis should be recognized as features for cocaine-induced lesions.

Avaliação do sistema musculoesquelético na unidade de emergência

OBJECTIVEnMusculoskeletal conditions have an enormous and growing impact worldwide. In spite of that, some clinicians are not confident in their own musculoskeletal examination skills. This study aimed to evaluate the prevalence of musculoskeletal symptoms in an emergency room, and the frequency of musculoskeletal physical examination description on those cases.nnnMETHODSnThis was a cross-sectional study. We performed a systematic analysis of medical files at the emergency room of the University Hospital of the Federal University of Santa Catarina, Brazil, from April 24th to 30th, 2009.nnnRESULTSnWe analyzed 392 files, where 41.5% of patients were male and mean age was 38.7 ± 17.2 years-old. Sixty nine out of 392 patients (17.6%) presented with a musculoskeletal complaint. The most common musculoskeletal complaint was low back pain (33/69). Only 49.2% of patients with a musculoskeletal chief complaint had a specific physical examination registered on the files. Patients with musculoskeletal complaints had lower registrations of abdominal examination (46% versus 62%, P = 0.01) and vital signs (46% versus 66%, P = 0.002), but a higher frequency of musculoskeletal examination registration (49% versus 0.6%, P = 0.00).nnnCONCLUSIONSnOur study confirms other observations worldwide. Musculoskeletal complaints are frequent in a emergency room setting and in spite of that it is suggested that musculoskeletal symptoms are poorly evaluated, which is probably related to an insufficient musculoskeletal education. It is essential that medical schools place more emphasis on these conditions so that young physicians will be more prepared to deal with these common diseases.

Reinforcing a medical hypothesis with a new question: is there a subgroup of shrinking lungs syndrome that is induced by pleurisy in systemic lupus erythematosus and is this subgroup marked by anti-Ro/SSA?

Shrinking lungs syndrome (SLS) is a rare entity associated with autoimmune diseases and its underlying pathogenesis is still unclear. We describe a series of seven consecutive cases of SLS in systemic lupus erythematosus, all of them with serositis and six (85.7%) with anti-Ro/SSA antibodies. Our findings reinforce the hypothesis that SLS may be, in some cases, a consequence of diaphragmatic restriction due to pleuritic pain, and we suggest anti-Ro/SSA as a marker of this subgroup of SLS.

Tumor markers are elevated in patients with rheumatoid arthritis and do not indicate presence of cancer

Aim:u2002 To investigate serum levels of tumor markers in patients with rheumatoid arthritis (RA) and their association with disease activity or the presence of cancer.

Subclinical atherosclerosis in ankylosing spondylitis: is there a role for inflammation?

OBJECTIVESnTo evaluate the prevalence of subclinical atherosclerosis in patients with ankylosing spondylitis (AS) in comparison to controls with similar cardiovascular risk factors.nnnMETHODSnForty-two consecutive patients with AS and 42 controls matched for age (43.3 ± 11.7 vs. 43.7 ± 11.3, P = 0.89), gender, smoking, diabetes mellitus and arterial hypertension were enrolled. Participants were excluded if a personal cardiovascular disease (CV) history was present. A questionnaire recording demographic data, medical and medication history was fulfilled. Blood pressure, abdominal circumference, height and weight were measured. Lipid profile was determined in a 12-hour fastened blood sample. Ultrasound analysis of the common carotid artery was performed by one blind observer. The distance between the lumen-intima interface and the leading edge of the media-adventitia interface (IMT) was measured and participants were also evaluated for the presence of plaques.nnnRESULTSnThe comparative analysis of demographic and cardiovascular risk factors between AS patients and controls did not reveal statistically significant differences. Also, no significant differences between groups were observed for TC, HDL-C, T-C/HDL-C, LDL-C, triglycerides, or dyslipidemia frequency. IMT measures were not different in AS and controls (0.62 ± 0.09 vs. 0.61 ± 0.09, P = 0.39) as well as plaques frequencies (19% vs. 17%, P = 0.78).nnnCONCLUSIONSnSubclinical atherosclerosis assessed through carotid ultrasound imaging was not more prevalent in the AS group when compared to controls with similar cardiovascular risks. Our observations may imply that CV risk factors may have more influence on the CV system than AS itself. These findings should be confirmed in a larger population with a prospective study design.

Aterosclerose subclínica em pacientes com espondilite anquilosante: há um papel para a inflamação?

OBJECTIVES: To evaluate the prevalence of subclinical atherosclerosis in patients with ankylosing spondylitis (AS) in comparison to controls with similar cardiovascular risk factors. METHODS: Forty-two consecutive patients with AS and 42 controls matched for age (43.3 ± 11.7 vs. 43.7 ± 11.3, P = 0.89), gender, smoking, diabetes mellitus and arterial hypertension were enrolled. Participants were excluded if a personal cardiovascular disease (CV) history was present. A questionnaire recording demographic data, medical and medication history was fulfilled. Blood pressure, abdominal circumference, height and weight were measured. Lipid profile was determined in a 12-hour fastened blood sample. Ultrasound analysis of the common carotid artery was performed by one blind observer. The distance between the lumen-intima interface and the leading edge of the media-adventitia interface (IMT) was measured and participants were also evaluated for the presence of plaques. RESULTS: The comparative analysis of demographic and cardiovascular risk factors between AS patients and controls did not reveal statistically significant differences. Also, no significant differences between groups were observed for TC, HDL-C, T-C/HDL-C, LDL-C, triglycerides, or dyslipidemia frequency. IMT measures were not different in AS and controls (0.62 ± 0.09 vs. 0.61 ± 0.09, P = 0.39) as well as plaques frequencies (19% vs. 17%, P = 0.78). CONCLUSIONS: Subclinical atherosclerosis assessed through carotid ultrasound imaging was not more prevalent in the AS group when compared to controls with similar cardiovascular risks. Our observations may imply that CV risk factors may have more influence on the CV system than AS itself. These findings should be confirmed in a larger population with a prospective study design.

Micofenolato mofetil na síndrome de Sjögren primária: uma opção para o tratamento da agranulocitose

The Sjögrens syndrome (SS) is an autoimmune disease characterized by a lymphocytic infiltration of salivary and lacrimal glands. Hematological manifestations of primary SS (pSS) usually consist of mild anemia, thrombocytopenia, moderate neutropenia, and lymphopenia. Agranulocytosis is rarely reported and usually responds to immunosuppression. We report the case of a pSS patient who presented with refractory agranulocytosis. Bone marrow biopsy disclosed a hypocellular bone marrow with normal maturation of the granulocytic series. The patient was successively treated with high-dose prednisone, granulocyte-macrophage colony stimulation factor, and cyclosporine, with no hematological response. Mycophenolate mofetil (MMF) was initiated and after two months there was a rise on the white blood cell count. After one year of follow-up, she had neither further neutropenia episodes, nor infectious complications. We conclude that, in pSS refractory agranulocytosis, MMF can be an effective and well-tolerated treatment option.