Commentary

Relation between dermatomyositis and polymyositis and cancer

Describir la metodología del Registro de pacientes con miopatía inflamatoria idiopática (MII) de España (Myo-Spain), así como sus fortalezas y limitaciones. El objetivo principal del proyecto es analizar la evolución y el manejo clínico de una cohorte de pacientes con MII.

Estudio observacional, longitudinal, ambispectivo y multicéntrico de una cohorte de pacientes con MII atendidos en servicios de reumatología de España. Se incluirán todos los pacientes con diagnóstico de MII en seguimiento habitual por los centros participantes, sin tener en cuenta la edad de inicio del proceso. Los casos incidentes serán todos los pacientes que al inicio del estudio en cada centro estén diagnosticados desde hace menos de 12 meses y casos prevalentes desde hace más de 12 meses. Se construirá un registro en el que se incluirán los datos de la visita basal, del año y dos años. Se recogerán variables sociodemográficas, clínicas, analíticas, complicaciones, comorbilidad, asociación con otras enfermedades reumáticas, ingresos hospitalarios, mortalidad y tratamientos. Además, se determinarán índices, escalas y cuestionarios de actividad, afectación muscular, daño, discapacidad y calidad de vida. El periodo de reclutamiento será de 23 meses. El propósito es conseguir una cohorte de 400 pacientes con MII.

El estudio Myo-Spain constituye la oportunidad para desarrollar una cohorte de pacientes incidentes y prevalentes con MII en España. Myo-Spain permitirá evaluar en detalle, las características clínicas de la enfermedad en diferentes momentos. Se espera que la información exhaustiva recogida en las visitas suponga una amplia fuente de datos para futuros análisis.

To describe the methods of the Spanish Registry of patients with idiopathic inflammatory myopathy (IIM) (Myo-Spain), as well as its strengths and limitations. The main objective of the project is to analyse the evolution and clinical management of a cohort of patients with IIM.

Observational, longitudinal, ambispective and multicentre study of a cohort of patients with IIM seen in rheumatology units in Spain. All patients with a diagnosis of IMM will be included in the regular follow-up of the participating centres, regardless of age on initiation of the process. Incident cases will be all patients who at the beginning of the study have been diagnosed for less than 12 months and prevalent cases for more than 12 months. The registry will include data from the visit at baseline, one year and two years. Socio-demographic, clinical, analytical variables, complications, comorbidities, association with other rheumatic diseases, hospital admissions, mortality and treatments will be collected. In addition, indices, scales and questionnaires of activity, muscle involvement, damage, disability, and quality of life will be determined. The recruitment period will be 23 months. The purpose is to obtain a cohort of 400 patients with IMM.

Myo-Spain registry provides the opportunity to develop a cohort of incident and prevalent patients with IMM in Spain. Myo-Spain will be able to assess in detail the clinical characteristics of the disease at different times. The comprehensive information collected during the visits is expected to provide a broad source of data for future analysis.

Paraneoplastic neurological disorder (PND) syndromes are disorders that occur physically remote from and commonly develop before the detection of a primary malignancy or metastases. They are thought to be the by-product of systemic immune responses to cancer. PND syndromes may potentially affect any part of the central, peripheral, or autonomic nervous systems individually or in combination. Their diagnosis may be challenging because of the lack of a diagnostic consensus and familiarity among physicians. Patient management centers on the recognition and treatment of the tumor as well as concomitant immunosuppressant and symptomatic therapy.

The histologic findings of scalp involvement in dermatomyositis are not well characterized on account of a lack of large series.

To systematize the histologic features of scalp involvement in dermatomyositis on horizontal and vertical sections.

A descriptive, prospective, cross-sectional study recruited 31 patients with pathologically and serologically confirmed dermatomyositis in Mexico City, Mexico, from June 2014 to June 2015. A total of 36 scalp biopsy specimens from 20 patients with scalp involvement in dermatomyositis were processed as 20 vertical and 16 horizontal sections.

Dilated capillaries and diffuse mucin deposition were detected in all biopsy specimens, followed by interface dermatitis. Partial or segmental thickening of the basement membrane, hyperkeratosis, atrophic epidermis, and acrosyringeal hypergranulosis with hyperkeratosis were other very common findings. Preserved follicular architecture, with intact or slightly atrophic sebaceous glands, was present in most horizontal sections. There was decreased follicular density, with a terminal-to-vellus ratio of 4:1 and telogen count of 10.3%. Eosinophils were present in 15% and 25% of horizontal and vertical sections, respectively.

No special stains performed.

Scalp involvement in dermatomyositis shows nonscarring pattern on horizontal sections that is consistent with chronic telogen effluvium. Telangiectasia and mucin are universal histologic features; eosinophils and acrosyringeal hypergranulosis with hyperkeratosis are new findings.

In patients with juvenile dermatomyositis/polymyositis (JDM/PM), malignancy is a rare phenomenon. An extensive workup for neoplastic disease is not routinely indicated, but should be considered if unusual physical findings are noted at JDM/PM diagnosis or during follow-up period. The objective of this literature review was to assess for any association between, and disease profile of, JDM/PM and lymphoma in childhood. Risk determinants of the possible development of lymphoma in the pediatric population with JDM/PM appear to be the degree and duration of inflammatory activity with chronic B-cell activation and/or antigen stimulation; a paraneoplastic relationship is unlikely.

The association between dermatomyositis (DM) and neoplasm has been widely described, especially in elder patients; this entity can be associated with many different types of tumours, more frequently lung, ovary, and gastric cancers. There is no clear association with central nervous system tumours. We present the case of a 64-year-old woman diagnosed simultaneously of DM with a difficult clinical control and a meningioma, being the course of the two illnesses linked. The activity of the DM was controlled after the meningioma was removed.

La asociación entre la dermatomiositis (DM) y las neoplasias ha sido ampliamente descrita, especialmente en pacientes de edad avanzada; esta entidad puede estar asociada con diferentes tipos de tumores, especialmente con cáncer de pulmón, ovario y estómago. No existe una clara asociación con tumores en el sistema nervioso. Se presenta el caso de una mujer de 64 años a la que se diagnosticó simultáneamente DM de difícil control clínico y un meningioma, estando el curso de ambas enfermedades vinculadas. La actividad de la DM fue controlada tras la extracción del meningioma.