Poster Presentations

DETERMINANTS OF EXERCISE CAPACITY AND PERCEIVED EXERTION IN PATIENTS WITH PRESERVED LEFT VENTRICULAR SYSTOLIC FUNCTION

To review the epidemiology, general clinical aspects and diagnosis, impact on morbidity and mortality, and general treatment approaches for myositis-associated ILD.

The relevant literature was reviewed.

The clinical, radiographic, and histopathological features of interstitial lung disease (ILD) in idiopathic inflammatory myopathies (IIM) are similar to idiopathic ILD. Patients with a known diagnosis of myositis require prompt clinical evaluation including the determination of myositis-associated autoantibodies. Patients possessing autoantibodies associated with ILD or those with any pulmonary symptoms should undergo a pulmonary function test and high-resolution CT (HRCT) scanning of their lungs.

Despite the lack of placebo-controlled trials, systemic glucocorticoids are considered the mainstay of initial treatment of myositis-associated ILD. Glucocorticoid-sparing agents are often concomitantly administered, particularly in patients with severe disease. The first-line conventional immunosuppressive drugs include either mycophenolate mofetil or azathioprine. If these agents fail or if the pulmonary features are severe or rapidly progressive, then more aggressive immunosuppressive or immunomodulatory therapy including cyclophosphamide, tacrolimus or cyclosporine, rituximab, IVIg, or tofacitinib can be considered. Further investigations are required to assess the role of novel therapies in the treatment of myositis-associated ILD.

Revisar la epidemiología, los aspectos clínicos generales, el diagnóstico, el impacto en la morbilidad y la mortalidad y los enfoques generales de tratamiento para la enfermedad pulmonar intersticial (EPI) asociada a miositis.

Se revisó la literatura relevante.

Las características clínicas, radiográficas e histopatológicas de la EPI en las miopatías inflamatorias idiopáticas (MII) son similares a las de la EPI idiopática. Los pacientes con un diagnóstico conocido de miositis requieren una evaluación clínica inmediata que incluya la determinación de autoanticuerpos asociados a la miositis. Los pacientes que poseen autoanticuerpos asociados con EPI o aquellos con cualquier síntoma pulmonar deben someterse a una prueba de función pulmonar y una tomografía computarizada de alta resolución (TCAR) de sus pulmones.

A pesar de la falta de ensayos controlados con placebo, los glucocorticoides sistémicos se consideran el pilar del tratamiento inicial de la EPI asociada a miositis. Los agentes ahorradores de glucocorticoides a menudo se administran de forma concomitante, particularmente en pacientes con enfermedad grave. Los fármacos inmunosupresores convencionales de primera línea incluyen micofenolato mofetilo o azatioprina. Si estos agentes fallan o si las características pulmonares son graves o rápidamente progresivas, se puede considerar una terapia inmunosupresora o inmunomoduladora más agresiva que incluya ciclofosfamida, tacrolimus o ciclosporina, rituximab, IgIV o tofacitinib. Se requieren más investigaciones para evaluar el papel de las nuevas terapias en el tratamiento de la EPI asociada a la miositis.

La enfermedad pulmonar intersticial en el lupus es una entidad que se presenta con poca frecuencia y en la mayoría de los casos tiende a ser de progresión lenta. A pesar de esto, se desconoce en gran medida el enfoque terapéutico de los casos moderados a severos, debido a que la mayor parte de la evidencia proviene de reportes de caso y muchos de ellos fueron anteriores al advenimiento de los nuevos tratamientos para el lupus que se conocen hoy en día. Adicionalmente, se ha avanzado poco en entender su fisiopatología, los conceptos actuales provienen de otras enfermedades del tejido conectivo como la esclerosis sistémica que en ocasiones son agrupadas dentro del grupo de neumonías intersticiales con características autoinmunes. Esto de cierta forma ha sido un obstáculo para la investigación en este campo, sin que se haya logrado un enfoque diagnóstico y terapéutico unificado. Por ello, se realiza una búsqueda avanzada con el objetivo de tener la mayor evidencia disponible hasta la fecha en cuanto a los métodos diagnósticos y las terapias emergentes, ofreciendo al clínico una visión práctica para lograr su abordaje integral.

Interstitial lung disease in lupus is an entity that occurs infrequently and tends to progress slowly in most cases. Despite this, the therapeutic approach for moderate to severe cases is largely unknown because most of the evidence comes from case reports, many of which predate the advent of today's known treatments for lupus. Additionally, little progress has been made in understanding its pathophysiology and current concepts come from other connective tissue diseases such as systemic sclerosis or are grouped within the group of interstitial pneumonias with autoimmune characteristics. This, to an extent, has been an obstacle for research in this field, and to date there is no unified diagnostic and therapeutic approach. Therefore we conducted a state of the art search of the best evidence available to date, in terms of diagnostic methods and emerging therapies, to offer the clinician a practical vision for a comprehensive approach.

Although interstitial lung disease (ILD) is a leading cause of morbidity and mortality in patients with inflammatory myopathies, the current definition and diagnostic criteria of autoimmune myositis remain inadequate to capture the large proportion of patients with lung-dominant disease. As a result, these patients present unique diagnostic and treatment challenges for even the most experienced clinicians. This article highlights the emerging role of autoantibodies in the diagnosis, classification, and management of patients with ILD. We propose alternative nomenclature to facilitate research on this unique patient population. Additionally, evidence supporting the various therapies used in the treatment of myositis-associated ILD is reviewed. The classification and treatment of patients with myositis-associated ILD remains challenging. A standardized therapeutic approach to these patients is lacking, and prospective studies in the field are needed to determine optimal treatment regimens.

Las enfermedades pulmonares intersticiales difusas asociadas a enfermedades autoinmunes sistémicas (EPID-EAS) pueden presentar una progresión fibrótica. El objetivo principal del estudio es describir una serie de casos de pacientes con EPID-EAS que cursan con fibrosis pulmonar progresiva e inician tratamiento con fármacos antifibróticos.

Estudio observacional retrospectivo unicéntrico de un hospital de tercer nivel sobre una serie de casos de pacientes con EPID-EAS con fibrosis pulmonar progresiva valorados en una consulta conjunta de neumología y reumatología, que iniciaron tratamiento con fármacos antifibróticos entre el 01/01/2019 y el 01/12/2021. Se analizaron las características epidemiológicas, clínicas, funcionales, radiológicas y terapéuticas al inicio del tratamiento, y la evolución funcional durante el tratamiento, así como los efectos adversos.

Se incluyeron 18 pacientes. La edad media observada fue de 66,7 ± 12,7 años, con mayor frecuencia de sexo femenino (66,7%), siendo la esclerosis sistémica la enfermedad autoinmune sistémica más frecuente (36,8%). La mayoría de los pacientes se encontraban en tratamiento con glucocorticoides sistémicos (88,9%), un 72,2% de pacientes con fármacos modificadores de la enfermedad, siendo el más frecuente el micofenolato mofetilo (38,9%), y un 22,2% con rituximab. Se observó una estabilidad funcional tras el inicio del tratamiento antifibrótico. Fallecieron 2 pacientes durante el seguimiento, uno de ellos como consecuencia de la progresión de la enfermedad intersticial pulmonar.

Nuestro estudio sugiere un efecto beneficioso del tratamiento antifibrótico añadido al tratamiento inmunomodulador en pacientes con EPID-EAS fibrótica en vida real. En nuestra serie de casos, los pacientes con EPID-EAS con afectación fibrosante progresiva muestran una estabilidad funcional tras el inicio del tratamiento antifibrótico. La tolerancia al tratamiento fue relativamente buena, con un perfil de efectos secundarios similar al descrito en la literatura médica.

Interstitial lung diseases associated with systemic autoimmune diseases (ILD-SAD) can progress to a fibrotic form that can benefit from antifibrotic treatment. The aim of the study is to describe a cohort of patients with ILD-SAD who manifest progressive pulmonary fibrosis treated with antifibrotics.

Single-centre retrospective observational study from a tertiary care hospital on a cohort of patients with ILD-SAD with progressive pulmonary fibrosis evaluated in a joint pulmonology and rheumatology clinic that initiated treatment with antifibrotic drugs between 01/01/2019 and 01/12/2021. Clinical characteristics were analysed. The evolution of pulmonary function test and adverse effects during treatment were described.

18 patients were included. The mean age was 66.7 ± 12.7 years, with a higher frequency of females (66.7%). Systemic sclerosis (SS) was the most frequent systemic autoimmune disease (36.8%). The majority of patients were receiving systemic glucocorticoid treatment (88.9%), 72.2% of patients were receiving treatment with disease-modifying drugs, the most frequent being mycophenolate mofetil (38.9%), and 22.2% with rituximab. Functional stability was observed after the start of antifibrotic treatment. Two patients died during follow-up, one due to progression of ILD.

Our study suggests a beneficial effect of antifibrotic treatment added to immunomodulatory treatment in patients with fibrotic ILD-SAD in real life. In our cohort, patients with ILD-SAD with progressive fibrosing involvement show functional stability after starting antifibrotic treatment. Treatment tolerance was relatively good with a side effect profile similar to that described in the medical literature.

Idiopathic inflammatory myopathies (IIMs) represent a heterogeneous group of systemic autoimmune diseases characterized by immune-mediated muscle injury. As insights into pathogenesis of IIM evolve, novel therapeutic strategies have become available to optimize outcomes. Herein, we summarize novel and emerging strategies in the management of dermatomyositis (DM), immunemediated necrotizing myopathy (IMNM), and inclusion body myositis (IBM).

Many therapies have been investigated for systemic sclerosis-associated interstitial lung disease (SSc-ILD), including immunosuppressive therapies, antifibrotic agents, immunomodulators and monoclonal antibodies. There is a high unmet medical need to better understand the current evidence for treatment efficacy and safety. This systematic review aims to present the existing literature on different drug treatments investigated for SSc-ILD and to critically assess the level of evidence for these drugs.

A systematic review was performed in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A structured literature search was performed for clinical trials and observational studies on the treatment of SSc-ILD with pharmaceutical interventions from 1 January 1990 to 15 December 2020. The quality of each reference was assessed using the Grades of Recommendation, Assessment, Development, and Evaluation (GRADE) criteria.

A total of 77 references were reviewed and 13 different treatments were identified. We found high-quality evidence for the use of cyclophosphamide, nintedanib, mycophenolate and tocilizumab. Therefore, we would posit that the clinical community has four valid options for treatment of SSc-ILD. Further research is mandatory to provide more evidence for the optimal treatment strategy in SSc-ILD, including the optimal time to initiate treatment, selection of patients for treatment and upfront combination therapy.