Dolors Grados

Granulomatosis con poliangeítis (Wegener). Descripción de 15 casos

BACKGROUND Granulomatosis with polyangiitis (GP) is a necrotizing vasculitis of unknown etiology that involves small and medium caliber vessels. It is associated with anti neutrophil cytoplasm antibodies (ANCA). It most often affects the respiratory tract and the kidneys and its most important pathologic feature is the presence of necrotizing granulomas. OBJECTIVES To detail the features of 15 patients with GP diagnosed in a university referral center. PATIENTS AND METHODS Retrospective study: between 1984 and 2009, 15 patients with GP were diagnosed in our center. Epidemiological, clinical, laboratory test as well as pathologic studies and treatment were retrospectively analyzed. Biopsy diagnosis of GP was considered as an inclusion criterion. RESULTS Fifteen patients were diagnosed: 12 men and 3 women. Mean age at diagnosis: 52.2 years (14-78). 12 patients had a history of smoking. A biopsy was diagnostic in all patients. ANCA were positive in 11 cases, 6 had a cytoplasmic c-ANCA pattern. All patients had pulmonary involvement and seven (40%) had renal involvement. All patients received intravenous glucocorticoids and cyclophosphamide as induction therapy. During the disease progression 5 patients died. CONCLUSIONS The clinical features of this series do not differ from those described by other authors. However, a history of smoking is more common than expected. Frequently used drugs were glucocorticoids and cyclophosphamide (oral and pulse therapy). The course was usually unfavorable, with outbreaks or complications due to immunosuppression, except for those with limited forms. Immunosuppressive therapy should be maintained indefinitely in most cases.

A comment on “From diagnosis to remission: place of MRI in eosinophilic fasciitis”

We read with interest the letter of Desvignes-Engelbert et al. [1] and we would like to comment on it. We recently reviewed 13 patients with eosinophilic fasciitis (EF). All were diagnosed on the basis of a fullthickness skin to muscle biopsy. We performed magnetic resonance imaging (MRI) in three patients who had a negative histological diagnosis. In all cases, the second guided biopsy was positive. Herein, we present the three cases, in which MRI was very useful.

Asistencia reumatológica en el sector sanitario público de Cataluña: año 2012

OBJECTIVE To determine the current state of Rheumatology in Catalonia (Spain) and to update information regarding previous studies METHODS STUDY DESIGN observational, descriptive and transversal. SAMPLE Physicians practicing rheumatology in the public system of Catalonia. An epidemiological questionnaire was sent to all rheumatologists. The results were compared with previously published studies. RESULTS Information was obtained on 130 rheumatologists (62 men/68 women, mean age 47±9 years). Seventy five (57.7%) physicians worked at a hospital, 5 (3.8%) in primary care and 50 (38.5%) in both. Seven (11.9%) hospitals had no rheumatologist. Eight hospitals were accredited by the National Commission to develop a training program in Rheumatology. The number of residents accredited by each hospital was variable. CONCLUSIONS The number of rheumatologists in the public health sector in Catalonia has increased 4.8% during the last seven years, unlike the 2005 study in which there was an increase of 40% over the previous eight years. There were 7 hospitals without a rheumatologist.

Association Between Vitamin D Status and Schizophrenia: A First Psychotic Episode Study

Abstract Vitamin D deficiency has been linked with schizophrenia. We aimed to determine whether patients with a first episode of psychosis (FEP) had lower vitamin D levels compared with controls considering their final diagnosis. We conducted a cross-sectional study determining 25-hydroxyvitamin D blood levels. 25-Hydroxyvitamin D levels were considered optimum at 20 ng/mL or greater. A group of 45 adult patients with FEP and a group of 22 healthy controls matched for age were recruited. The patient group was subdivided in two final diagnosis groups (schizophrenia versus other psychoses) after a 6-month follow-up. Average vitamin D values were deficient for FEP patients, especially those 22 with a final diagnosis of schizophrenia. These results relating vitamin D and schizophrenia generate interest to further examine this association.

Osteoporosis premenopáusica: ¿cómo tratarla?

There is no agreement in defining osteoporosis in premenopausal women and diagnosis must be done carefully and not based on densitometric parameters. One must take into account the presence of other risk factors and history of fragility fractures, diseases or drugs that cause bone loss. Over 50% of premenopausal women with osteoporosis will have a secondary cause, with the remainder diagnosed with idiopathic osteoporosis. Therapeutic considerations are limited by a few studies in this group of patients, especially in regard to the risk of fractures. On the other hand, the FRAX index cannot be applied to premenopausal women. This article will review the measures to apply depending on the type of premenopausal osteoporosis, based on current scientific evidence.

AB0769 Assessment of Sequels of poliomyelitis in Hips by 3D-DXA

Background Polio is a devastating infectious disease that causes paralysis and severe muscular atrophy. When any of the legs is affected, a low aBMD at the corresponding hip is a common finding in DXA measurement. Objectives to study the influence of polio consequences on volumetric bone density and cortical thickness using the 3D-DXA technology. Methods 22 patients of both sexes suffering post-polio syndromes (mean age 57±uffering pos were scanned using a GE iDXA system and both proximal femurs were reconstructed using the 3D-DXA technology. 3D-DXA is based on the registration of a 3D statistical model of the femoral shape and density onto the 2D DXA image and provides measurements of volumetric BMD (vBMD) and BMCof the trabecular and cortical bone as well as a quantification of the cortical thickness. The measurements at the leg affected by polio were compared by t-test with those at the non-affected leg. Results Trabecular BMC was 22% lower (-1.8 g, p<0.01) and cortical BMC 19% lower (-2.9 g, p<0.01) at the polio leg (total hip region). Similar findings were observed for the vBMD: 18% decrease (-0.020 g/cm3, p<0.01) at the trabecular region and a 2.3% decrease for the cortical bone (-0.024 g/cm3, p<0.01). The proximal femur volume was also inferior (-12%, -9 cm3, p<0.01). The cortical thickness was thinner at the polio leg (-12%, -0.2 mm, p<0.01). Conclusions A long period of decreased of mechanical charges, low muscle strength, postural imbalance has dramatic consequences on bone development. The analyses performed using the 3D-DXA technology indicates an overall impairment of cortical and trabecular BMD and a significant decrease of the cortical thickness. Disclosure of Interest None declared

AB0882 BMD and TBS use in Adult Patients Suffering Polio

Objectives Assess TBS to categorize skeletal status in adult subject which history of Poliomyelitis sequels in limbs. Methods We scan total body, lumbar spine (L1-L4) and both hips (Total femur and Femoral Neck ROIs) in 58 patients with history of poliomyelitis infection and limb paralysis (men: 14 -57.5 years; women: 48 -57±8.5 years). A DXA device GE-iDXA model was used. The difference between affected extremity and the opposite in BMD T-score and trabecular microarchitecture assessed by TBS (TBS® Insight; Medimaps) were used. The results were stratified as normal, low bone density or osteoporosis taking the lower T-score of the scanned regions of interest. TBS results were categorized as normal (TBS N) ≥1.350; partially deteriorated-(TBS-PD) between: 1.250 -1.349, and significantly deteriorated (TBS-SD): <1.250. Results Three patients had hip fracture history. The left limb was the extremity most affected in this group (32 vs 20). Five patients had both extremities affected, with more severe affectation on the right side (4/5). Only 7 patients had normal BMD of the hip, which contrasts with the 43.1% of patients with normal lumbar BMD. The TBS was normal in 16 patients (27.6%). Stratification was more similar in patients who had a low bone density (48.3% in the hip and 50% in lumbar spine) and TBS-PD (50%). In patients categorized as osteoporotic the lowest T-score of BMD was found in proximal femur (39.7%) of the most affected limb, the T-score BMD of the lumbar spine in 6.9% of the patients, while the TBS-SD was found at 22.4%. The DXA spine images showed a spondyloarthritis and scoliosis signs in 25 patients. 16.7% of patients had a osteoporotic T-score in affected femur and had normal BMD at the lumbar spine and opposite femur, but interestingly had a deteriorated TBS (TBS-PPD and TB SSD). Conclusions A degradation TBS allow identify a greater number of patients who had polio with low bone status than assess BMD of lumbar spine and femur. Postural instability could increase irregular mechanical charges to lumbar spine and femur unaffected that may decrease the sensitivity of DXA when only the BMD is used to classify patients. Disclosure of Interest None declared

AB0890 Is There a Relationship Between Vitamin D and Schizophrenia

Background Studies suggest a neuroprotective role for vitamin D. Its deficit has been linked with various psychiatric disorders such as schizophrenia (1). Increase in the prevalence of schizophrenia has been associated with high latitudes, winter/spring births, migration, urbanicity and cold climates (2,3). Vitamin D deficiency could be an important environmental risk factor, linking all of the previously exposed. Objectives We aimed to determine whether patients with a first psychotic episode had low levels of vitamin D compared to controls, considering their final diagnoses (schizophrenia vs other psychoses). Methods Cross-sectional study in an acute inpatient psychiatric unit. Vitamin D (25-hydroxyvitamin D) was determined by direct competitive chemiluminescence immunoassay. Vitamin D levels (ng/ml) were considered optimum >30, insufficient 20–30 and deficient <20 (4). Final diagnosis was obtained from the outpatient mental health service after a 6 month follow-up. Results We analyzed 27 patients with first-episode psychosis (13 with final diagnosis of schizophrenia and 14 of other psychoses) and 17 healthy controls, with no differences in mean age between the three groups (χ2 (2)=5.20, p>0.05). Schizophrenic patients showed deficient average values (ng/ml) of vitamin D (mean 12.87, range 6-24.9). Vitamin D levels in the other psychoses patient group were higher but also deficient (mean 15.74, range 6-34.6). Controls presented higher levels of vitamin D, but still insufficient (mean 21.45, range 11.20-33.5). Vitamin D levels in the schizophrenia group were significantly lower compared to controls (CI 1.8 – 15.4; p<0.009). No other significant differences were found between groups. Conclusions We observed deficient vitamin D levels in all the patients studied, being significantly lower for schizophrenic patients. Future studies are required to further examine this association in schizophrenia because vitamin D deficiency may be an easily modifiable risk factor by means of a cheap and save public health intervention. References Belvederi Murri, M., Respino, M., et al. 2013 Vitamin D and psychosis: mini meta-analysis. Schizophr Res 150, 235-239. McGrath, J. J., Eyles, D.W., et al. 2010 Neonatal vitamin D status and risk of schizophrenia: a populationbased case-control study. Arch Gen Psychiatry 67, 889-94. McGrath, J. J., Burne, T.H., et al. 2010 Developmental vitamin D deficiency and risk of schizophrenia: a 10-year update. Schizophr Bull 36, 1073-8. Holick, M.F. 2007 Vitamin D deficiency. N Engl J Med 357, 266-81. Disclosure of Interest None declared

AB0883 Denosumab: Patterns of Prescription and Side Effects in 68 Patients

Background Denosumab is a human monoclonal antibody (IgG2) that targets and binds with high affinity and specificity to RANKL. It is indicated for the treatment of osteoporosis in postmenopausal women and in men with high risk of fractures. Objectives To describe the clinical characteristics, indications, side effects and densitometric parameters in patients treated with denosumab. Methods Retrospective study (2012-2014). We reviewed the medical records of patients treated with denosumab. We collected the epidemiological and clinical features and side effects. Results Sixty-eight patients received denosumab, all of them women with an average age of 74.8 years (60-91 years). All of them had osteoporosis: postmenopausal in 48 patients (70.5%) and secondary in 20 patients (29.5%): 9 early menopause, 6 primary hyperparathyroidism, 2 glucocorticoid treatment and 2 epilepsy. Fifty-two patients (76.4%) had received other treatments previously (70.5% oral or intravenous bisphosphonates, 16.2% teriparatide or PTH1-84, 19.1% strontium ranelate, 5.9% SERMS). Sixteen patients (23.5%) had no prior treatment performed mostly by gastric intolerance. Forty patients (58.8%) had fractures before treatment: vertebral (29.4%), wrist (17.6%), femur (8.8%) and other (19.1%): pubic rami, sacrum and humerus. The average of bone mineral density before treatment was: T score -3.2 in the lumbar spine (L2-L4) and -2.6 in the femur, and after treatment (36 patients) was: T score -2.6 in the lumbar spine and -2.1 in the femur. Before injecting each dose of denosumab, all patients underwent an analytic with calcium and phosphorus metabolism in which we checked that calcium levels were correct. All patients received calcium supplements and vitamin D. We observed that in the analysis prior to the second dose of denosumab, 14 patients had elevated PTH values (average of 88pg/ml), vitamin D average of 26.8ng/ml, with previous normal values (10-65pg/ml). Out of all patients, 4 had received a single injection, 30 two, 12 three, 12 four, 7 five and 3 six injections of denosumab. Twelve patients suffered side effects (17.6%): urine infections and otitis (3 patients), symptomatic hypocalcemia (3 patients), arthralgia (2 patients), toothache (1 patient), tooth loss (1 patient), leukopenia (1 patient) and abnormal liver tests (1 patient). Two patients (3%) had fractures after treatment: a patient with polio presented a vertebral fracture during a treatment break and a patient who had previously undergone 10 years of oral bisphosphonate presented bilateral femoral shaft fractures. Conclusions The treatment was well tolerated and only a small percentage of patients was suspended by mild side effects. Densitometric values showed an improvement in both spine and femoral neck. Two fractures were observed during follow-up of patients, one in a patient with spinal polio and bilateral femoral shaft fractures four months after administration of denosumab in a patient previously treated over 10 years with bisphosphonate. Disclosure of Interest None declared

Afectación del sistema nervioso central y periférico en la fascitis eosinofílica

Eosinophilic fasciitis (EF) is a rare sclerodermiform syndrome of unknown cause described by Shulman in 1974.1 It features2: induration, peripheral eosinophilia, increased erythrocyte sedimentation rate (ESR) and hypergammaglobulinemia. Various systemic manifestations associated with EF have been described, such as renal, cardiac, respiratory, joint and peripheral nervous system manifestations. Below we present the case of a patient with FE of the central and peripheral nervous system. The patient was a 71-year-old male with a medical history of hypertension and tuberculosis. He came to the hospital with induration of the arms, legs, chest and neck, which had started 5 months prior. He referred dysphagia to solids and loss of 10 kg in the past year. Physical examination revealed skin induration in the arms, legs, trunk and neck, respecting the hands and feet. He had a positive sulcus sign and orange skin. Neurological examination was normal. Laboratory tests showed peripheral eosinophilia (8.1 × 109/l leukocytes with 2% eosinophils) and an ESR of 40 mm in the first hour. Blood biochemistry was normal. Protein, tumor markers, chest X-ray, l capillaroscopy and immunological tests were normal. The upper GI series revealed esophageal motor incoordination with passage of contrast between the larynx and esophagus. An MRI showed changes consistent with eosinophilic fasciitis. A muscle biopsy was performed which revealed deep and reactive inflammatory changes in adipose tissue and fascia, all compatible with EF. Glucocorticoid therapy was initiated at doses of 1 mg/kg/day. At 2 months he showed a left facial central paralysis and hypoglossal nerve palsy. Cranial MRI was normal. A lumbar puncture was performed which was also normal. Subsequently, paralysis of the external popliteal nerve was observed. Physical examination showed persistent induration of the arms, legs and trunk. The neurological examination revealed a central left facial nerve palsy, left hypoglossal nerve palsy, paresis of the external cyatic popliteal nerve, but sensitivity and tendon reflexes were present and symmetrical as was the bilateral flexor plantar cutaneous reflex. The blood count was normal and ESR was 22 mm in the first hour. Blood biochemistry showed mild hypoproteinemia. Tumor markers and a CT scan were normal. An electromyogram was performed which revealed an asymmetric