Post-acute phase and sequelae management of epidermal necrolysis: an international, multidisciplinary DELPHI-based consensus.

BACKGROUND: Long-term sequelae are frequent and often disabling after epidermal necrolysis (Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)). However, consensus on the modalities of management of these sequelae is lacking. OBJECTIVES: We conducted an international multicentric DELPHI exercise to establish a multidisciplinary expert consensus to standardize recommendations regarding management of SJS/TEN sequelae. METHODS: Participants were sent a survey via the online tool "Survey Monkey" consisting of 54 statements organized into 8 topics: general recommendations, professionals involved, skin, oral mucosa and teeth, eyes, genital area, mental health, and allergy workup. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). Results were analyzed according to the RAND/UCLA Appropriateness Method. RESULTS: Fifty-two healthcare professionals participated. After the first round, a consensus was obtained for 100% of 54 initially proposed statements (disagreement index < 1). Among them, 50 statements were agreed upon as 'appropriate'; four statements were considered 'uncertain', and ultimately finally discarded. CONCLUSIONS: Our DELPHI-based expert consensus should help guide physicians in conducting a prolonged multidisciplinary follow-up of sequelae in SJS-TEN.

Supportive care in the acute phase of Stevens-Johnson syndrome and toxic epidermal necrolysis: an international, multidisciplinary Delphi-based consensus.

BACKGROUND: Supportive care is the cornerstone of management of adult and paediatric Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, consensus on the modalities of supportive care is lacking. OBJECTIVES: Our aim in this international multicentric Delphi exercise was to establish a multidisciplinary expert consensus to standardize recommendations regarding supportive care in the acute phase of SJS/TEN. METHODS: Participants were sent a survey via the online tool SurveyMonkey, consisting of 103 statements organized into 11 topics: multidisciplinary team composition, suspect drug management, infection prevention, fluid resuscitation and prevention of hypothermia, nutritional support, pain and psychological distress management, management of acute respiratory failure, local skincare, ophthalmological management, management of other mucosa, and additional measures. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). The results were analysed according to the RAND/UCLA Appropriateness Method. RESULTS: Forty-five participants from 13 countries (on three continents) participated. After the first round, a consensus was obtained for 82.5% of the 103 initially proposed statements. After the second round, a final consensus was obtained for 102 statements. CONCLUSIONS: We have reached an international Delphi-based consensus on best supportive care practice for SJS/TEN. Our expert consensus should help guide physicians in treating patients with SJS/TEN and thereby improve short-term prognosis and the risk of sequelae.

Single-arm, open label prospective trial to assess prediction of the role of ERCC1/XPF complex in the response of advanced NSCLC patients to platinum-based chemotherapy.

BACKGROUND: Platinum-based therapy, combined or not with immune checkpoint inhibitors, represents a front-line choice for patients with non-small-cell lung cancer (NSCLC). Despite the improved outcomes in the last years for this malignancy, only a sub-group of patients have long-term benefit. Excision repair cross-complementation group 1 (ERCC1) has been considered a potential biomarker to predict the outcome of platinum-based chemotherapy in NSCLC. However, the ERCC1 gene is transcribed in four splice variants where the isoform 202 was described as the only one active and able to complex Xeroderma pigmentosum group F-complementing protein (XPF). Here, we prospectively investigated if the active form of ERCC1, as assessed by the ERCC1/XPF complex (ERCC1/XPF), could predict the sensitivity to platinum compounds. PATIENTS AND METHODS: Prospectively enrolled, patients with advanced NSCLC treated with a first-line regimen containing platinum were centrally evaluated for ERCC1/XPF by a proximity ligation assay. Overall survival (OS), progression-free survival (PFS) and objective response rate (ORR) were analyzed. RESULTS: The absence of the ERCC1/XPF in the tumor suggested a trend of worst outcomes in terms of both OS [hazard ratio (HR) 1.41, 95% confidence interval (CI) 0.67-2.94, P = 0.373] and PFS (HR 1.61, 95% CI 0.88-3.03, P = 0.123). ORR was marginally influenced in ERCC1/XPF-negative and -positive groups [odds ratio (stable disease + progressive disease versus complete response + partial response) 0.87, 95% CI 0.25-3.07, P = 0.832]. CONCLUSION: The lack of ERCC1/XPF complex in NSCLC tumor cells might delineate a group of patients with poor outcomes when treated with platinum compounds. ERCC1/XPF absence might well identify patients for whom a different therapeutic approach could be necessary.

Secukinumab-associated localized granuloma annulare (SAGA): a case report and review of the literature.

Granuloma annulare (GA) is a benign, usually self-limited inflammatory skin dermatosis characterized clinically by pink-red to brown dermal papules or annular plaques. The main histologic feature is the presence of palisading or interstitial granulomas composed of necrobiotic collagen, elastic fibers, and mucin surrounded by a lymphohistiocytic infiltrate. Granuloma annulare is commonly associated with trauma, infections, diabetes mellitus, dyslipidemia, malignancy, thyroid disease, and a variety of medications. Two cases of GA have been reported in association with the use of secukinumab, a monoclonal antibody directed against interleukin 17A (IL17A), for the treatment of moderate-to-severe plaque psoriasis. We report the third case of secukinumab-associated GA in a 52-year-old woman with a history of diabetes mellitus type II, dyslipidemia, and non-alcoholic steatohepatitis. After four months of therapy with secukinumab, she presented with pink papules coalescing to plaques involving the antecubital fossae. Histology demonstrated a lymphohistiocytic palisading granuloma with central necrobiotic collagen and mucin, consistent with GA. Physicians should be aware of the possibility of GA developing in patients receiving secukinumab, especially in those with predisposing factors for GA. A better understanding of secukinumab-associated GA may lead to discoveries in GA pathogenesis and reveal broader immunomodulatory effects of secukinumab.

Case series on multiple prostate re-irradiation for locally recurrent prostate cancer: something ventured, something gained.

The aim is to present the technical feasibility and efficacy of multiple re-irradiation (re-EBRT) for local recurrence of prostate cancer (PCa) using retrospective analysis of an updated series of patients who received ablative re-EBRT with stereotactic image-guided technique for isolated local recurrence of PCa. Eight patients received three RT courses (2 re-RTs); of those 2 received 4 RT courses (3 re-RTs). Local relapse in the prostate was assessed by multiparametric magnetic resonance and/ or choline positron emission tomography. Before treatment planning, all patients had been evaluated for late toxicity from previous RT according to RTOG/EORTC. Biochemical control was assessed according to Phoenix definition. Mean age at the third RT course was 68 (standard deviation, SD: 7.2); all patients had a good performance status. At diagnosis, four cases were classified as high risk PCa, three as intermediate and one as low per NCCN 2017. Biochemical progression free interval after first and second RT-course were 74 (IQR: 59.3-133.6) months and 33 (IQR: 20.8-53.1) months, respectively. Biochemical and radiological response was registered in all patients. At present, seven out of eight patients are disease free. Overall toxicity profile was good; no severe acute or late genitourinary or gastrointestinal events were recorded. Multiple RT courses with high precision technology and image guidance can be proposed as a possible salvage therapy for locally recurrent, low-burden PCa recurrence in adequately selected patients. Deeper understanding of radiobiological effects of hypofractionation and larger series of patients are warranted to fully evaluate the applicability of multiple RT courses in the setting of locally recurrent PCa.

On the total system error of a robotic radiosurgery system: phantom measurements, clinical evaluation and long-term analysis.

The total system error (TSE) of a CyberKnife® system was measured using two phantom-based methods and one patient-based method. The standard radiochromic film (RCF) end-to-end (E2E) test using an anthropomorphic head and neck phantom and isocentric treatment delivery was used with the 6Dskull, Fiducial and Xsight® spine (XST) tracking methods. More than 200 RCF-based E2E results covering the period from installation in 2006 until 2017 were analyzed with respect to tracking method, system hardware and software versions, secondary collimation system, and years since installation. An independent polymer gel E2E method was also applied, involving a 3D printed head phantom and multiple spherical target volumes widely distributed within the brain. Finally, the TSE was assessed by comparing the delineated target in the planning computed tomography images of a patient treated for a thalamic functional target with the radiation-induced lesion defined on the six-month follow-up magnetic resonance (MR) images. Statistical analysis of the RCF-based TSE results showed mean ± standard deviation values of 0.40 ± 0.18 mm, 0.40 ± 0.19 mm, and 0.55 ± 0.20 mm for the 6Dskull, Fiducial, and XST tracking methods, respectively. Polymer gel TSE values smaller than 0.66 mm were found for seven targets distributed within the brain, showing that the targeting accuracy of the system is sustained even for targets situated up to 80 mm away from the center of the skull. An average clinical TSE value of 0.87 ± 0.25 mm was also measured using the FSE T2 and FLAIR post-treatment MR image data. Analysis of the long-term RCF-based E2E tests showed no changes of TSE over time. This study is the first to report long-term (>10 years) analysis of TSE, TSE measurement for targets positioned at large distances from the virtual machine isocenter, or a clinical assessment of TSE for the CyberKnife system. All of these measurements demonstrate TSE consistently < 1 mm.

Evaluation of osteopontin expression in chronic wounds: a potential prognostic and therapeutic biomarker.

OBJECTIVE: Osteopontin (OPN) is abundantly expressed during tissue repair, acting as a powerful chemokine that recruits inflammatory cells such as neutrophils, macrophages, and Langerhans cells. The role of OPN in chronic wounds has not been explored. In this study, we assess the expression levels of OPN in chronic wounds to assess its potential contribution to the exacerbated inflammation seen in chronic ulcers, which is thought to contribute to poor healing. METHODS: This retrospective study included archived biopsies of chronic wounds from several aetiologies. Immunohistochemical staining and blind analysis of OPN expression were carried out. RESULTS: We assessed biopsies from venous leg ulcers (n=5), diabetic foot ulcers (n=5), pyoderma gangrenosum (n=5), squamous cell carcinoma ulcers (n=4), and calciphylaxis ulcers (n=3). The data revealed that all these sets of chronic ulcers expressed high levels of OPN. CONCLUSION: This study provides strong histopathologic evidence that OPN expression is significantly increased in chronic wounds, suggesting that its upregulation could contribute to the exacerbated inflammation. Furthermore, further characterisation of the role of OPN in wound healing could aid the development of specific and efficient anti-OPN therapies for the treatment of chronic wounds.

Predictors of Asymptomatic Radiation-induced Abdominal Atherosclerosis.

AIMS: To identify predictors of asymptomatic radiation-induced abdominal atherosclerosis in patients treated with radiotherapy and evaluated by abdominal vascular ultrasonography. MATERIALS AND METHODS: Forty-two testicular classic seminoma patients (median age 34 years, range 16-56) undergoing radical inguinal orchiectomy were analysed. Twenty-six patients underwent post-surgery radiotherapy (median total dose 25 Gy, range 25-43), two of them also received chemotherapy (CHT) and 16 patients were treated with surgery alone or by surgery followed by CHT (control group). The presence of stenosis in an abdominal vessel and renal resistive index (RRI), evaluated by echo-colour Doppler (ECD), were considered as indicators of late vascular damage. Chi-square and Mann-Whitney tests were used to compare groups. For the radiotherapy group, near maximum (D2%) and mean dose (Dmean) metrics of critical structures (abdominal arteries and renal hila) were extracted from retrievable dose maps (18 of 26 radiotherapy patients). To evaluate clinical and dosimetric factors associated with vascular damage, univariate and multivariate analyses were carried out. The impact of dose to arteries, evaluated as separate subvolumes, was analysed comparing the stenotic arteries with normal ones by logistic regression. The area under the receiver operator characteristic curve (AUC) was used to evaluate the test accuracy. RESULTS: In the radiotherapy group there was a significantly different incidence of stenosis (31% versus 0%, P = 0.016) and a higher median average RRI (0.63 versus 0.60, P = 0.032) compared with the control group. The median time intervals between treatment and ECD were 64 months (range 12-120) and 48 months (range 12-168) in the radiotherapy and control groups (P = 0.399), respectively. A younger age at radiotherapy was the only clinical risk factor for stenosis (P = 0.006). Artery Dmean was significantly associated with stenosis (P = 0.008), with an odds ratio of 1.13 (95% confidence interval 1.01-1.26) and an AUC of 0.85 (95% confidence interval 0.77-0.91). Renal hilum D2% was correlated with RRI (Rs = 0.406, P = 0.02). CONCLUSIONS: Late vascular damage represents a potential effect of abdominal radiotherapy, even at a moderate dose. Younger age at irradiation, artery and renal hila dose metrics are associated with increased risk. Ultrasound-based follow-up may allow for non-invasive early detection of asymptomatic radiation-induced damage, helping to prevent severe vascular events.

VERO® radiotherapy for low burden cancer: 789 patients with 957 lesions.

PURPOSE: The aim of this retrospective study is to evaluate patient profile, feasibility, and acute toxicity of RadioTherapy (RT) delivered by VERO® in the first 20 months of clinical activity. METHODS: Inclusion criteria: 1) adult patients; 2) limited volume cancer (M0 or oligometastatic); 3) small extracranial lesions; 4) treatment between April 2012 and December 2013 and 5) written informed consent. Two techniques were employed: intensity modulated radiotherapy (IMRT) and stereotactic body radiotherapy (SBRT). Toxicity was evaluated using Radiation Therapy Oncology Group/European Organisation for Research and Treatment of Cancer (RTOG/EORTC) criteria. RESULTS: Between April 2012 and December 2013, 789 consecutive patients (957 lesions) were treated. In 84% of them one lesion was treated and in 16% more than one lesion were treated synchronously/metachronously; first radiotherapy course in 85%, re-irradiation in 13%, and boost in 2% of cases. The treated region included pelvis 46%, thorax 38%, upper abdomen 15%, and neck 1%. Radiotherapy schedules included <5 and >5 fractions in 75% and 25% respectively. All patients completed the planned treatment and an acceptable acute toxicity was observed. CONCLUSIONS: RT delivered by VERO® was administrated predominantly to thoracic and pelvic lesions (lung and urologic tumours) using hypofractionation. It is a feasible approach for limited burden cancer offering short and well accepted treatment with favourable acute toxicity profile. Further investigation including dose escalation and other available VERO® functionalities such as real-time dynamic tumour tracking is warranted in order to fully evaluate this innovative radiotherapy system.

Linfadenectomía inguinal vídeo endoscópica: resultados quirúrgicos y oncológicos / Video Endoscopic Inguinal Lymphadenectomy: Surgical and oncological results

Introducción: Evaluamos la reproducibilidad de la linfadenectomía inguinal vídeo endoscópica (VEIL) y relatamos nuestra experiencia inicial en el tratamiento del cáncer de pene con ganglios palpables .Material y métodos: De julio de 2006 a noviembre de 2010 fueron realizadas 33 linfadenectomías inguinales vídeo endoscópicas en 20 pacientes, como tratamiento complementario del cáncer de pene, en 2 hospitales de referencia en Latinoamérica. Fueron analizadas las características epidemiológicas y clínicas de los pacientes, así como los resultados quirúrgicos y oncológicos. Resultados: De los pacientes incluidos el 55% fueron de estadio clínico N0 y el 45% fueron N+. La VEIL se llevó a cabo de forma bilateral en 13 pacientes y en 7 casos se realizó VEIL unilateral, asociada con la cirugía convencional contralateral. El tiempo operatorio medio de la VEIL fue de 119 min, y la media de adenopatías resecadas fue de 8 por cada linfadenectomía. La tasa global de complicaciones fue del 33,2%. Ningún paciente presentó necrosis cutánea. La tasa de complicaciones linfáticas fue del 27,2%. En los 6 casos en que se preservó la vena safena interna (18,2%) no hubo complicaciones linfáticas (p = 0,2). La tasa de sobrevida global fue del80% y la sobrevida cáncer específica fue del 90%. El seguimiento medio fue de 20 meses. Conclusiones: La VEIL en el tratamiento complementario del cáncer de pene es un procedimiento seguro y reproducible, siendo una alternativa a la linfadenectomía convencional. Los pacientes con adenopatías palpables también se pueden beneficiar con esta técnica. Los resultados oncológicos son adecuados, aunque se necesita más tiempo de seguimiento (AU)

Introduction: We evaluated the reproducibility of video endoscopic inguinal lymphadenectomy (VEIL) and we report our initial experience in the treatment of penile cancer with palpable inguinal lymph nodes. Material and methods: From July 2006 to November 2010 were conducted 33 VEIL in 20 patients as complementary treatment for penile cancer in two referral hospitals in Latin America. We analyzed the epidemiological and clinical characteristics of patients and surgical and oncologic outcomes. Results: Fifty-five percent of the patients included were clinical stage N0 and 45% were N +.Thirteen patients underwent bilateral VEIL and the remaining seven underwent VEIL unilateral and conventional open surgery in the contralateral limb. The average operative time for VEIL was 119 minutes and mean resected lymph nodes was 8 per lymphadenectomy. The overall complication rate was 33.2%. No patient had skin necrosis. The lymphatic complication rate was 27.2%. Of the 6 cases in which the saphenous vein was preserved (18.2%) there were no lymphatic complications (P = 0,2). The overall survival rate was 80% and cancer-specific survival was 90%. Mean follow-up was 20 months. Conclusions: VEIL in the adjunctive treatment of penile cancer is safe, reproducible and maybe an alternative to conventional lymphadenectomy. Patients with palpable lymphadenopathy also may benefit from this technique. Oncological results seem to be adequate however require longer follow-up to be confirmed (AU)

Video endoscopic inguinal lymphadenectomy: surgical and oncological results.

INTRODUCTION: We evaluated the reproducibility of video endoscopic inguinal lymphadenectomy (VEIL) and we report our initial experience in the treatment of penile cancer with palpable inguinal lymph nodes. MATERIAL AND METHODS: From July 2006 to November 2010 were conducted 33 VEIL in 20 patients as complementary treatment for penile cancer in two referral hospitals in Latin America. We analyzed the epidemiological and clinical characteristics of patients and surgical and oncologic outcomes. RESULTS: Fifty-five percent of the patients included were clinical stage N0 and 45% were N +. Thirteen patients underwent bilateral VEIL and the remaining seven underwent VEIL unilateral and conventional open surgery in the contralateral limb. The average operative time for VEIL was 119 minutes and mean resected lymph nodes was 8 per lymphadenectomy. The overall complication rate was 33.2%. No patient had skin necrosis. The lymphatic complication rate was 27.2%. Of the 6 cases in which the saphenous vein was preserved (18.2%) there were no lymphatic complications (P=,2). The overall survival rate was 80% and cancer-specific survival was 90%. Mean follow-up was 20 months. CONCLUSIONS: VEIL in the adjunctive treatment of penile cancer is safe, reproducible and may be an alternative to conventional lymphadenectomy. Patients with palpable lymphadenopathy also may benefit from this technique. Oncological results seem to be adequate however require longer follow-up to be confirmed.

Deep brain stimulation of subthalamic nuclei affects arm response inhibition in Parkinson's patients.

The precise localizations of the neural substrates of voluntary inhibition are still debated. It has been hypothesized that, in humans, this executive function relies upon a right-lateralized pathway comprising the inferior frontal gyrus and the presupplementary motor area, which would control the neural processes for movement inhibition acting through the right subthalamic nucleus (STN). We assessed the role of the right STN, via a countermanding reaching task, in 10 Parkinson's patients receiving high-frequency electrical stimulation of the STN of both hemispheres (deep brain stimulation, DBS) and in 13 healthy subjects. We compared the performance of Parkinson's patients in 4 experimental conditions: DBS-ON, DBS-OFF, DBS-OFF right, and DBS-OFF left. We found that 1) inhibitory control is improved only when both DBS are active, that is, the reaction time to the stop signal is significantly shorter in the DBS-ON condition than in all the others, 2) bilateral stimulation of STN restores the inhibitory control to a near-normal level, and 3) DBS does not cause a general improvement in task-related motor function as it does not affect the length of the reaction times of arm movements, that is, in our experimental context, STN seems to play a selective role in response inhibition.

[Locally recurrent rectal cancer. Our experience]. / Recidiva locoregionale da cancro del retto operato. Esperienza personale.

The aim of this study was to describe the personal experience in the treatment of 31 patients (19 males, 12 females) with local recurrence following surgery for rectal adenocarcinoma. The exeresis of recurrence was possible in 17 patients (6 Miles, 9 Hartmann, 1 re-resection, 1 transanal resection); of these, only 6 patients reported an improvement of the quality of life. Patients surgically treated and patients with recurrence inoperable and submitted to palliative treatment (colostomy, pain control, radio- and/or chemotherapy) showed similar survival rates.

D2-40 highlights lymphatic vessel proliferation of angiolymphoid hyperplasia with eosinophilia.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign, uncommon idiopathic condition, characterized by cutaneous papules or nodules, whose etiopathogenesis is still unclear. It has been considered an angioproliferating lesion (epithelioid hemangioma) since histologically it is marked by a proliferation of blood vessels, accompanied by an inflammatory infiltrate, consisting mainly of lymphocytes and eosinophils. We present a case of ALHE assessed immunohistochemically for D2-40-a new marker for lymphatic endothelial cells. A biopsy specimen obtained from the same anatomical area of a healthy individual served as a normal control. The ALHE specimen showed increased number of lymphatic vessels when stained for D2-40, whereas the endothelial cells lining blood vessels were negative. The specificity of D2-40 for lymphatic vessels was further substantiated by studying Factor VIII-related antigen expression in consecutive sections of both ALHE and the control specimen. A reverse pattern was appreciated-blood vessels showed Factor VIII positive labeling, whereas lymphatic endothelial cells remained unlabeled. We therefore assume that apart from the lymphocytic infiltrate in the lesion, the recognized lymphoid component in ALHE is due to lymphatic vessel proliferation as well. Hence, this condition may be considered as possibly derived from lymphatic endothelium.

Atlas-based functional radiosurgery: early results.

Functional disorders of the brain, such as dystonia and neuropathic pain, may respond poorly to medical therapy. Deep brain stimulation (DBS) of the globus pallidus pars interna (GPi) and the centromedian nucleus of the thalamus (CMN) may alleviate dystonia and neuropathic pain, respectively. A noninvasive alternative to DBS is radiosurgical ablation [internal pallidotomy (IP) and medial thalamotomy (MT)]. The main technical limitation of radiosurgery is that targets are selected only on the basis of MRI anatomy, without electrophysiological confirmation. This means that, to be feasible, image-based targeting must be highly accurate and reproducible. Here, we report on the feasibility of an atlas-based approach to targeting for functional radiosurgery. In this method, masks of the GPi, CMN, and medio-dorsal nucleus were nonrigidly registered to patients' T1-weighted MRI (T1w-MRI) and superimposed on patients' T2-weighted MRI (T2w-MRI). Radiosurgical targets were identified on the T2w-MRI registered to the planning CT by an expert functional neurosurgeon. To assess its feasibility, two patients were treated with the CyberKnife using this method of targeting; a patient with dystonia received an IP (120 Gy prescribed to the 65% isodose) and a patient with neuropathic pain received a MT (120 Gy to the 77% isodose). Six months after treatment, T2w-MRIs and contrast-enhanced T1w-MRIs showed edematous regions around the lesions; target placements were reevaluated by DW-MRIs. At 12 months post-treatment steroids for radiation-induced edema and medications for dystonia and neuropathic pain were suppressed. Both patients experienced significant relief from pain and dystonia-related problems. Fifteen months after treatment edema had disappeared. Thus, this work shows promising feasibility of atlas-based functional radiosurgery to improve patient condition. Further investigations are indicated for optimizing treatment dose.

Spinal radiosurgery: technology and clinical outcomes.

The development of computer-based image guidance has allowed stereotactic radiosurgery and radiotherapy to be freed from the constraints imposed by the stereotactic frames once required for intracranial radiosurgery. This freedom has led to the application of radiosurgery to targets outside the brain. In this paper, we briefly review the technologies, treatment parameters, and clinical outcomes of radiosurgical treatment for spinal pathology, including metastatic tumors and rare but challenging lesions such as arteriovenous malformations and benign tumors. A special emphasis is put on the newest development, fiducial-less robotic radiosurgery. Spinal radiosurgery is associated with excellent rates of tumor control and pain relief with a good dose sparing of the highly sensitive spinal cord. Further research is required to optimize treatment strategies and to assess clinical benefits and toxicity in the long term.

A Brazilian registry of juvenile dermatomyositis: onset features and classification of 189 cases.

OBJECTIVE: To describe onset features, classification and treatment of juvenile dermatomyositis (JDM) and juvenile polymyositis (JPM) from a multicentre registry. METHODS: Inclusion criteria were onset age lower than 18 years and a diagnosis of any idiopathic inflammatory myopathy (IIM) by attending physician. Bohan & Peter (1975) criteria categorisation was established by a scoring algorithm to define JDM and JPM based on clinical protocol data. RESULTS: Of the 189 cases included, 178 were classified as JDM, 9 as JPM (19.8: 1) and 2 did not fit the criteria; 6.9% had features of chronic arthritis and connective tissue disease overlap. Diagnosis classification agreement occurred in 66.1%. Median onset age was 7 years, median follow-up duration was 3.6 years. Malignancy was described in 2 (1.1%) cases. Muscle weakness occurred in 95.8%; heliotrope rash 83.5%; Gottron plaques 83.1%; 92% had at least one abnormal muscle enzyme result. Muscle biopsy performed in 74.6% was abnormal in 91.5% and electromyogram performed in 39.2% resulted abnormal in 93.2%. Logistic regression analysis was done in 66 cases with all parameters assessed and only aldolase resulted significant, as independent variable for definite JDM (OR=5.4, 95%CI 1.2-24.4, p=0.03). Regarding treatment, 97.9% received steroids; 72% had in addition at least one: methotrexate (75.7%), hydroxychloroquine (64.7%), cyclosporine A (20.6%), IV immunoglobulin (20.6%), azathioprine (10.3%) or cyclophosphamide (9.6%). In this series 24.3% developed calcinosis and mortality rate was 4.2%. CONCLUSION: Evaluation of predefined criteria set for a valid diagnosis indicated aldolase as the most important parameter associated with definite JDM category. In practice, prednisone-methotrexate combination was the most indicated treatment.