IPAF agrupa individuos con EPID y otras características clínicas, serológicas o pulmonares que derivan de una condición autoinmune sistémica subyacente, pero no cumplen con los criterios reumatológicos actuales para una ETC. La EPID, manifestación clínica frecuente de las ETC, puede aparecer en el contexto de una ETC conocida pero no es infrecuente que sea la primera y única manifestación de un ETC oculta. Identificar una ETC subyacente en pacientes que presentan con compromiso intersticial inicial puede ser un desafío; tales evaluaciones pueden optimizarse mediante un enfoque multidisciplinario. Presentamos el caso de tres pacientes, de diferente presentación, evolución y tratamiento, todos caracterizados hasta la fecha como IPAF.
IPAF groups individuals with ILD and other clinical, serologic, or pulmonary manifestations with an underlying systemic autoimmune condition, but do not meet current rheumatologic criteria for a CTD. ILD is a frequent clinical manifestation of CTDs; may appear in the context of a well known CTD but is often the first and only manifestation of an unknown CTD. Identifying an underlying CTD in patients presenting with initial interstitial involvement can be challenging; such evaluations can be optimized using a multidisciplinary approach. We present the case of three patients, of different presentation, evolution and treatment, all characterized to date as IPAF.
Lung Diseases, Interstitial , Patients , Autoimmune Diseases , Therapeutics
Since the discovery of glucocorticoids (GCs), their important anti-inflammatory effect, rapid mechanism of action, low cost, and accessibility have made them one of the mainstays of treatment for Systemic lupus erythematosus (SLE). Although their use has allowed controlling the disease and reducing acute mortality in severe conditions, the implementation of a scheme based on high doses for long periods has inevitably been accompanied by an increase in adverse effects and infections, including long-term damage. The objective of this review is to answer some important questions that may arise from its use in daily clinical practice, and to propose a paradigm based on the use of methylprednisolone pulses followed by medium-low doses and a rapid decrease of prednisone.
