Aspectos clínicos y factores asociados a compromiso renal en pacientes pediátricos con púrpura de Schönlein Henoch / Clinical aspects and factors related with renal involvement in pediatrics patients with Schönlein Henoch purpura

INTRODUCCIÓN: La púrpura de Schönlein Henoch (PSH) es la vasculitis más frecuente de la infancia. Es generalmente autolimitada, con morbilidad renal a largo plazo. OBJETIVO: Determinar la frecuencia de las manifestaciones clínicas de la PSH y las variables asociadas al compromiso renal en pacientes con PSH del Servicio de Reumatología pediátrica de la Clínica Universitaria Reina Fabiola durante el periodo 2015-2020. MATERIALES Y METODOS: Estudio observacional, retrospectivo, transversal, analítico. Se incluyeron pacientes menores de 15 años con diagnóstico de PSH. Variables: edad, sexo, mes de diagnóstico, compromiso dermatológico, renal, gastrointestinal, articular y recurrencia y/o persistencia y otras manifestaciones. Análisis estadístico: test T de Student, test chi cuadrado y regresión logística multivariada. RESULTADOS: Se analizaron 107 pacientes, 61 (57%) de sexo femenino, con una media (desviación estándar, DE) de edad de 6,49 (3,48) años. El 100% presentó compromiso dérmico, 19 (18%) púrpura persistente o recurrente, 21 (19%) síntomas gastrointestinales, 38 (36%) compromiso articular y 21 (20%) manifestaciones renales. Se asoció con compromiso renal a pacientes con edad mayor a 7 años (p=0.0064), púrpura persistente o recurrente (p=0.0001), compromiso articular (p=0,0135) y dolor abdominal (p=0,0136). En el análisis multivariado, la púrpura persistente o recurrente se asoció con compromiso renal (OR=7,16; IC95%: 1,81-28,25); p=0.005). CONCLUSIONES: La púrpura persistente o recurrente fue considerada factor de riesgo para presentar compromiso renal y además se evidenció una asociación entre pacientes mayores a 7 años, compromiso articular y dolor abdominal con compromiso renal.

INTRODUCTION: Schönlein Henoch purpura (SHP) is the most frequent vasculitis in childhood. Its course is generally self-limited but can lead to long-term renal morbidity OBJECTIVE: To determine the frequency of the clinical manifestations of PSH. To determine the variables associated with renal involvement in patients with SHP, treated by the Pediatric Rheumatology Service of the Reina Fabiola University Clinic during the period 2015-2020. MATERIALS AND METHODS: Observational, retrospective, transversal, analytical study. Patients under 15 years of age with a diagnosis of HSP, were included. The variables were: age, sex, month of diagnosis, dermatological, renal, gastrointestinal, joint involvement, recurrence and/or prevalence and other manifestations. Statistical analysis: Student's t test, chi square test and multivariate logistic regression. RESULTS: 107 patients were analyzed, 57% were female (n = 61), with a mean age of 6.49 SD of 3.48 years. 100% presented dermal involvement, 19 (18%) persistent or recurrent purpura, 21 (19%) gastrointestinal symptoms, 38 (36%) joint involvement and 21 (20%) kidney manifestations. An association with kidney involvement was found in patients older than 7 years (p = 0.0064), persistent or recurrent purpura (p = 0.0001), joint involvement (p = 0.0135) and abdominal pain (p = 0.0136), but only persistent or recurrent purpura was the only risk factor associated with statistically significant renal involvement (OR = 71.17; 95% CI = 1.81 - 28.25; P = 0.005). CONCLUSION: Persistent or recurrent purpura was considered a risk factor for the fact of presenting kidney involvement and also an association was evidenced between patients older than 7 years, joint involvement and abdominal pain with kidney involvement.

Prednisone versus prednisone plus ciclosporin versus prednisone plus methotrexate in new-onset juvenile dermatomyositis: a randomised trial.

BACKGROUND: Most data for treatment of dermatomyositis and juvenile dermatomyositis are from anecdotal, non-randomised case series. We aimed to compare, in a randomised trial, the efficacy and safety of prednisone alone with that of prednisone plus either methotrexate or ciclosporin in children with new-onset juvenile dermatomyositis. METHODS: We did a randomised trial at 54 centres in 22 countries. We enrolled patients aged 18 years or younger with new-onset juvenile dermatomyositis who had received no previous treatment and did not have cutaneous or gastrointestinal ulceration. We randomly allocated 139 patients via a computer-based system to prednisone alone or in combination with either ciclosporin or methotrexate. We did not mask patients or investigators to treatment assignments. Our primary outcomes were the proportion of patients achieving a juvenile dermatomyositis PRINTO 20 level of improvement (20% improvement in three of six core set variables at 6 months), time to clinical remission, and time to treatment failure. We compared the three treatment groups with the Kruskal-Wallis test and Friedman's test, and we analysed survival with Kaplan-Meier curves and the log-rank test. Analysis was by intention to treat. Here, we present results after at least 2 years of treatment (induction and maintenance phases). This trial is registered with ClinicalTrials.gov, number NCT00323960. FINDINGS: Between May 31, 2006, and Nov 12, 2010, 47 patients were randomly assigned prednisone alone, 46 were allocated prednisone plus ciclosporin, and 46 were randomised prednisone plus methotrexate. Median duration of follow-up was 35.5 months. At month 6, 24 (51%) of 47 patients assigned prednisone, 32 (70%) of 46 allocated prednisone plus ciclosporin, and 33 (72%) of 46 administered prednisone plus methotrexate achieved a juvenile dermatomyositis PRINTO 20 improvement (p=0.0228). Median time to clinical remission was 41.9 months in patients assigned prednisone plus methotrexate but was not observable in the other two treatment groups (2.45 fold [95% CI 1.2-5.0] increase with prednisone plus methotrexate; p=0.012). Median time to treatment failure was 16.7 months in patients allocated prednisone, 53.3 months in those assigned prednisone plus ciclosporin, but was not observable in patients randomised to prednisone plus methotrexate (1.95 fold [95% CI 1.20-3.15] increase with prednisone; p=0.009). Median time to prednisone discontinuation was 35.8 months with prednisone alone compared with 29.4-29.7 months in the combination groups (p=0.002). A significantly greater proportion of patients assigned prednisone plus ciclosporin had adverse events, affecting the skin and subcutaneous tissues, gastrointestinal system, and general disorders. Infections and infestations were significantly increased in patients assigned prednisone plus ciclosporin and prednisone plus methotrexate. No patients died during the study. INTERPRETATION: Combined treatment with prednisone and either ciclosporin or methotrexate was more effective than prednisone alone. The safety profile and steroid-sparing effect favoured the combination of prednisone plus methotrexate. FUNDING: Italian Agency of Drug Evaluation, Istituto Giannina Gaslini (Genoa, Italy), Myositis Association (USA).

Performance of current guidelines for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis.

OBJECTIVE: To compare the capacity of the 2004 diagnostic guidelines for hemophagocytic lymphohistiocytosis (HLH-2004) with the capacity of the preliminary diagnostic guidelines for systemic juvenile idiopathic arthritis (JIA)-associated macrophage activation syndrome (MAS) to discriminate MAS complicating systemic JIA from 2 potentially confusable conditions, represented by active systemic JIA without MAS and systemic infection. METHODS: International pediatric rheumatologists and hemato-oncologists were asked to retrospectively collect clinical information from patients with systemic JIA-associated MAS and confusable conditions. The ability of the guidelines to differentiate MAS from the control diseases was evaluated by calculating the sensitivity and specificity of each set of guidelines and the kappa statistics for concordance with the physician's diagnosis. Owing to the fact that not all patients were assessed for hemophagocytosis on bone marrow aspirates and given the lack of data on natural killer cell activity and soluble CD25 levels, the HLH-2004 guidelines were adapted to enable the diagnosis of MAS when 3 of 5 of the remaining items (3/5-adapted) or 4 of 5 of the remaining items (4/5-adapted) were present. RESULTS: The study sample included 362 patients with systemic JIA and MAS, 404 patients with active systemic JIA without MAS, and 345 patients with systemic infection. The best capacity to differentiate MAS from systemic JIA without MAS was found when the preliminary MAS guidelines were applied. The 3/5-adapted HLH-2004 guidelines performed better than the 4/5-adapted guidelines in distinguishing MAS from active systemic JIA without MAS. The 3/5-adapted HLH-2004 guidelines and the preliminary MAS guidelines with the addition of ferritin levels ≥500 ng/ml discriminated best between MAS and systemic infections. CONCLUSION: The preliminary MAS guidelines showed the strongest ability to identify MAS in systemic JIA. The addition of hyperferritinemia enhanced their capacity to differentiate MAS from systemic infections. The HLH-2004 guidelines are likely not appropriate for identification of MAS in children with systemic JIA.

Phagocyte-specific S100 proteins and high-sensitivity C reactive protein as biomarkers for a risk-adapted treatment to maintain remission in juvenile idiopathic arthritis: a comparative study.

OBJECTIVES: Juvenile idiopathic arthritis (JIA) is a chronic inflammatory joint disease affecting children. Even if remission is successfully induced, about half of the patients experience a relapse after stopping anti-inflammatory therapy. The present study investigated whether patients with JIA at risk of relapse can be identified by biomarkers even if clinical signs of disease activity are absent. METHODS: Patients fulfilling the criteria of inactive disease on medication were included at the time when all medication was withdrawn. The phagocyte activation markers S100A12 and myeloid-related proteins 8/14 (MRP8/14) were compared as well as the acute phase reactant high-sensitivity C reactive protein (hsCRP) as predictive biomarkers for the risk of a flare within a time frame of 6 months. RESULTS: 35 of 188 enrolled patients experienced a flare within 6 months. Clinical or standard laboratory parameters could not differentiate between patients at risk of relapse and those not at risk. S100A12 and MRP8/14 levels were significantly higher in patients who subsequently developed flares than in patients with stable remission. The best single biomarker for the prediction of flare was S100A12 (HR 2.81). The predictive performance may be improved if a combination with hsCRP is used. CONCLUSIONS: Subclinical disease activity may result in unstable remission (ie, a status of clinical but not immunological remission). Biomarkers such as S100A12 and MRP8/14 inform about the activation status of innate immunity at the molecular level and thereby identify patients with unstable remission and an increased risk of relapse.

Performance of Birmingham Vasculitis Activity Score and disease extent index in childhood vasculitides.

OBJECTIVES: To evaluate the performance of the Birmingham Vasculitis Activity Score (BVAS) v3 and the Disease Extent Index (DEI) for the assessment of disease activity in 4 primary childhood (c-) systemic vasculitides. METHODS: Patients fulfilling the EULAR/PRINTO/PRES (Ankara) c-vasculitis classification criteria for Henoch-Schönlein purpura (HSP), childhood (c) polyarteritis nodosa (c-PAN), c-Wegener's granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) with disease duration at the time of diagnosis ≤3 months were extracted from the PRINTO database. The performance of the BVAS and DEI were examined by assessing convergent validity, the pattern of disease involvement, and responsiveness. We also evaluated alternative unweighted scoring methods for both tools. RESULTS: The analysis set included 796 patients with 669 HSP, 80 c-PAN, 25 c-WG and 22 c-TA. The median age at diagnosis was 6.9 years (6.6-12) and median delay in making the diagnosis from the onset of signs/symptoms was 0.01 (0.003-0.027) years. A strong correlation was found between the BVAS and DEI (rs=0.78) while correlation with the physician global assessment was moderate (rs=0.48) with BVAS and poor with DEI (rs=0.25). Both the BVAS and DEI sub-scores and total scores were able to descrive the disease involvement in the 4 childhood vasculitides. Responsiveness was large (>1.5) for both tools. The performance characteristics of the BVAS and DEI with the unweighted methods were comparable. CONCLUSIONS: This study demonstrates that both the BVAS and DEI are valid tools for the assessment of the level of disease activity in a large cohort of childhood acute and chronic vasculitides.

The effect of infliximab plus methotrexate on the modulation of inflammatory disease markers in juvenile idiopathic arthritis: analyses from a randomized, placebo-controlled trial.

BACKGROUND: We evaluated the effect of infliximab on markers of inflammation in patients with juvenile idiopathic arthritis (JIA). METHODS: In this randomized, placebo-controlled substudy, 122 patients with JIA received infliximab 3 mg/kg + methotrexate (MTX)(n = 60) or placebo + MTX (n = 62) at weeks 0, 2, and 6. At week 14, patients receiving placebo + MTX crossed over to infliximab 6 mg/kg + MTX; patients receiving infliximab 3 mg/kg + MTX continued treatment through week 44. Sera and plasma from eligible patients receiving infliximab 3 mg/kg + MTX (n = 34) and receiving placebo→infliximab 6 mg/kg +MTX (n = 38) were collected at weeks 0, 2, 14, 16, 28, and 52 and analyzed for inflammatory markers (IL-6, IL-12p40, ICAM-1, MMP-3, VEGF, TNF-α, and CRP). RESULTS: At week 2, decreases from baseline in IL-6, ICAM-1, MMP-3, TNF-α, and CRP were greater with infliximab versus placebo treatment, and with the exception of CRP, these differences were generally maintained through week 14. The decreases from baseline to week 52 in IL-6, ICAM-1, VEGF, MMP-3, and CRP and increases in IL-12p40 levels were larger in patients receiving placebo→infliximab 6 mg/kg +MTX versus infliximab 3 mg/kg + MTX treatment. Patients receiving infliximab 3 mg/kg+MTX who achieved an American College of Rheumatology Pediatric 30 (ACR-Pedi-30) response had significantly larger decreases from baseline in ICAM-1 (p = 0.0105) and MMP-3 (p = 0.0253) at week 2 and in ICAM-1 (p = 0.0304), MMP-3 (p = 0.0091), and CRP (p = 0.0011) at week 14 versus ACR-Pedi-30 nonresponders. CONCLUSION: Infliximab + MTX attenuated several inflammatory markers in patients with JIA; larger decreases in ICAM-1, MMP-3, and CRP levels were observed in ACR-Pedi-30 responders versus nonresponders. TRIAL REGISTRATION: NCT00036374.

Health-related quality of life of patients with juvenile dermatomyositis: results from the Pediatric Rheumatology International Trials Organisation multinational quality of life cohort study.

OBJECTIVE: To investigate the health-related quality of life (HRQOL) change over time, as measured by the Child Health Questionnaire (CHQ), and its determinants in patients with active juvenile dermatomyositis (DM). METHODS: We assessed patients with juvenile DM at both baseline and 6 months of followup, and healthy children age < or =18 years. Potential determinants of poor HRQOL included demographic data, physician's and parent's global assessments, muscle strength, functional ability as measured by the Childhood Health Assessment Questionnaire (C-HAQ), global disease activity assessments, and laboratory markers. RESULTS: A total of 272 children with juvenile DM and 2,288 healthy children were enrolled from 37 countries. The mean +/- SD CHQ physical and psychosocial summary scores were significantly lower in children with juvenile DM (33.7 +/- 11.7 versus 54.6 +/- 4.1) than in healthy children (45.1 +/- 9.0 versus 52 +/- 7.2), with physical well-being domains being the most impaired. HRQOL improved over time in responders to treatment and remained unchanged or worsened in nonresponders. Both physical and psychosocial summary scores decreased with increasing levels of disease activity, muscle strength, and parent's evaluation of the child's overall well-being. A C-HAQ score >1.6 (odds ratio [OR] 5.06, 95% confidence interval [95% CI] 2.03-12.59), child's overall well-being score >6.2 (OR 5.24, 95% CI 2.27-12.10), and to a lesser extent muscle strength and alanine aminotransferase level were the strongest determinants of poor physical well-being at baseline. Baseline disability and longer disease duration were the major determinants for poor physical well-being at followup. CONCLUSION: We found that patients with juvenile DM have a significant impairment in their HRQOL compared with healthy peers, particularly in the physical domain. Physical well-being was mostly affected by the level of functional impairment.

A randomized, placebo-controlled trial of infliximab plus methotrexate for the treatment of polyarticular-course juvenile rheumatoid arthritis.

OBJECTIVE: To evaluate the safety and efficacy of infliximab in the treatment of juvenile rheumatoid arthritis (JRA). METHODS: This was an international, multicenter, randomized, placebo-controlled, double-blind study. One hundred twenty-two children with persistent polyarticular JRA despite prior methotrexate (MTX) therapy were randomized to receive infliximab or placebo for 14 weeks, after which all children received infliximab through week 44. Patients received MTX plus infliximab 3 mg/kg through week 44, or MTX plus placebo for 14 weeks followed by MTX plus infliximab 6 mg/kg through week 44. RESULTS: Although a higher proportion of patients in the 3 mg/kg infliximab group than in the placebo group had achieved responses according to the American College of Rheumatology (ACR) Pediatric 30 (Pedi 30) criteria for improvement at week 14 (63.8% and 49.2%, respectively), the between-group difference in this primary efficacy end point was not statistically significant (P = 0.12). By week 16, after the crossover from placebo to infliximab 6 mg/kg when all patients were receiving infliximab, an ACR Pedi 30 response was achieved in 73.2% of all patients. By week 52, ACR Pedi 50 and ACR Pedi 70 responses had been reached in 69.6% and 51.8%, respectively, of patients. Infliximab was generally well tolerated, but the safety profile of infliximab 3 mg/kg appeared less favorable than that of infliximab 6 mg/kg, with more frequent occurrences of serious adverse events, infusion reactions, antibodies to infliximab, and newly induced antinuclear antibodies and antibodies to double-stranded DNA observed with the 3 mg/kg dose. CONCLUSION: While infliximab at 3 mg/kg and 6 mg/kg showed durable efficacy at 1 year, achievement of the primary efficacy end point at 3 months did not differ significantly between infliximab-treated and placebo-treated patients. Safety data indicated that the 6-mg/kg dose may provide a more favorable risk/benefit profile. These results warrant further investigation in children with JRA.

Calidad de vida en niños con artritis idiopática juvenil / Quality of life in children with juvenile idiopathic arthritis

El pronóstico de los pacientes con Artritis Idiopática Juvenil (AIJ) ha mejorado considerablemente en las últimas décadas probablemente debido al diagnóstico temprano, al reconocimiento de las formas leves, los avances en el conocimiento de las posibilidades terapéuticas de la enfermedad y sus complicaciones. A pesar de esto la AIJ está aún asociada con importante morbilidad y discapacidad funcional a largo plazo. La evaluación clínica de los niños con AIJ requiere no solamente las medidas de la actividad de la enfermedad sino también el entendimiento de los efectos de la enfermedad y la terapia prescripta sobre el bienestar físico y psicosocial. OBJETIVOS: 1. Investigar la Calidad de Vida Relacionada con la Salud (CVRS) y sus determinantes en pacientes con AIJ. 2. Evaluar la CVRS en los diferentes subtipos de AIJ. 3. Compara la CVRS entre los pacientes con AIJ y niños sanos de edades similares y provenientes de la misma área geográfica

Calidad de vida en niños con artritis idiopática juvenil / Quality of life in children with juvenile idiopathic arthritis

El pronóstico de los pacientes con Artritis Idiopática Juvenil (AIJ) ha mejorado considerablemente en las últimas décadas probablemente debido al diagnóstico temprano, al reconocimiento de las formas leves, los avances en el conocimiento de las posibilidades terapéuticas de la enfermedad y sus complicaciones. A pesar de esto la AIJ está aún asociada con importante morbilidad y discapacidad funcional a largo plazo. La evaluación clínica de los niños con AIJ requiere no solamente las medidas de la actividad de la enfermedad sino también el entendimiento de los efectos de la enfermedad y la terapia prescripta sobre el bienestar físico y psicosocial. OBJETIVOS: 1. Investigar la Calidad de Vida Relacionada con la Salud (CVRS) y sus determinantes en pacientes con AIJ. 2. Evaluar la CVRS en los diferentes subtipos de AIJ. 3. Compara la CVRS entre los pacientes con AIJ y niños sanos de edades similares y provenientes de la misma área geográfica

The Pediatric Rheumatology International Trials Organization/American College of Rheumatology provisional criteria for the evaluation of response to therapy in juvenile systemic lupus erythematosus: prospective validation of the definition of improvement.

OBJECTIVE: To use the Pediatric Rheumatology International Trials Organization (PRINTO) core set of outcome measures to develop a validated definition of improvement for the evaluation of response to therapy in juvenile systemic lupus erythematosus (SLE). METHODS: Thirty-seven experienced pediatric rheumatologists from 27 countries, each of whom had specific experience in the assessment of juvenile SLE patients, achieved consensus on 128 patient profiles as being clinically improved or not improved. Using the physicians' consensus ratings as the gold standard measure, the chi-square, sensitivity, specificity, false-positive and false-negative rates, area under the receiver operating characteristic curve, and kappa level of agreement for 597 candidate definitions of improvement were calculated. Only definitions with a kappa value greater than 0.7 were retained. The top definitions were selected based on the product of the content validity score multiplied by its kappa statistic. RESULTS: The definition of improvement with the highest final score was at least 50% improvement from baseline in any 2 of the 5 core set measures, with no more than 1 of the remaining worsening by more than 30%. CONCLUSION: PRINTO proposes a valid and reproducible definition of improvement that reflects well the consensus rating of experienced clinicians and that incorporates clinically meaningful change in core set measures in a composite end point for the evaluation of global response to therapy in patients with juvenile SLE. The definition is now proposed for use in juvenile SLE clinical trials and may help physicians to decide whether a child with SLE responded adequately to therapy.

Localized scleroderma in childhood is not just a skin disease.

OBJECTIVE: Juvenile localized scleroderma is usually considered a disease that is confined to the skin and subcutaneous tissue. We studied the prevalence and clinical features of extracutaneous manifestations in a large cohort of children with juvenile localized scleroderma. METHODS: Data from a multinational study on juvenile scleroderma was used for this in-depth study. Clinical features of patients with extracutaneous manifestations were compared with those of patients who had exclusively skin involvement. RESULTS: Seven hundred fifty patients entered the study. One hundred sixty-eight patients (22.4%) presented with a total of 193 extracutaneous manifestations, as follows: articular (47.2%), neurologic (17.1%), vascular (9.3%), ocular (8.3%), gastrointestinal (6.2%), respiratory (2.6%), cardiac (1%), and renal (1%). Other autoimmune conditions were present in 7.3% of patients. Neurologic involvement consisted of epilepsy, central nervous system vasculitis, peripheral neuropathy, vascular malformations, headache, and neuroimaging abnormalities. Ocular manifestations were episcleritis, uveitis, xerophthalmia, glaucoma, and papilledema. In more than one-fourth of these children, articular, neurologic, and ocular involvements were unrelated to the site of skin lesions. Raynaud's phenomenon was reported in 16 patients. Respiratory involvement consisted essentially of restrictive lung disease. Gastrointestinal involvement was reported in 12 patients and consisted exclusively of gastroesophageal reflux. Thirty patients (4%) had multiple extracutaneous features, but systemic sclerosis (SSc) developed in only 1 patient. In patients with extracutaneous involvement, the prevalence of antinuclear antibodies and rheumatoid factor was significantly higher than that among patients with only skin involvement. However, Scl-70 and anticentromere, markers of SSc, were not significantly increased. CONCLUSION: Extracutaneous manifestations of juvenile localized scleroderma developed in almost one-fourth of the children in this study. These extracutaneous manifestations often were unrelated to the site of the skin lesions and sometimes were associated with multiple organ involvement. The risk of developing SSc was very low. This subgroup of patients with juvenile localized scleroderma should be evaluated extensively, treated more aggressively, and monitored carefully.

Granulomatosis de Wegener en la infancia / Wegener's granulomatosis in childhood

La Granulomatosis de Wegener (GW) es una enfermedad de etiología desconocida, poco frecuente en adultos, excepcional en la infancia, que se caracteriza por un cuadro clínico-patológico de vasculitis necrozante granulomatosa de vasos pequeños. Se presentaba el caso de una niña de 14 años de edad, quien evidencia la tríada clásica de sinutsitis, afectación pulmonar con hemosiderosis e infiltrados alveolares bilaterales difusos y glomerulonefritis. Además presentaba proptosis, queratoconjuntivitis, dacrioadenitis, retinitis miliar, otitis, cefalea, neuritis, artralgias, mialgias, tos, hemoptisis, fiebre y anemia. El cuadro, compatible con Granulomatosis de Wegener, fue tratado con Trimetoprima-Sufametoxazol (TMP-SMZ), prednisona y ciclofosfamida, con buena evolución hasta el momento de esta presentación

Granulomatosis de Wegener en la infancia / Wegeners granulomatosis in childhood

La Granulomatosis de Wegener (GW) es una enfermedad de etiología desconocida, poco frecuente en adultos, excepcional en la infancia, que se caracteriza por un cuadro clínico-patológico de vasculitis necrozante granulomatosa de vasos pequeños. Se presentaba el caso de una niña de 14 años de edad, quien evidencia la tríada clásica de sinutsitis, afectación pulmonar con hemosiderosis e infiltrados alveolares bilaterales difusos y glomerulonefritis. Además presentaba proptosis, queratoconjuntivitis, dacrioadenitis, retinitis miliar, otitis, cefalea, neuritis, artralgias, mialgias, tos, hemoptisis, fiebre y anemia. El cuadro, compatible con Granulomatosis de Wegener, fue tratado con Trimetoprima-Sufametoxazol (TMP-SMZ), prednisona y ciclofosfamida, con buena evolución hasta el momento de esta presentación (AU)