Abstract
The spectrum of pulmonary manifestations associated with mixed connective tissue disease ranges from pulmonary hypertension and interstitial lung disease to pleural effusions, alveolar hemorrhage, and complications from the thromboembolic disease. Interstitial lung disease in mixed connective tissue disease is a frequently occurring entity, although in most cases it tends to be self-limited or slowly progressive. Despite this, a significant percentage of patients may present a progressive fibrosing phenotype, thus posing a great challenge regarding its therapeutic approach, given the scarcity of clinical studies that compare the efficacy of immunosuppressants available to date. Due to this, many recommendations are extrapolated from other diseases with similar characteristics such as systemic sclerosis and systemic lupus erythematosus. That is why it is proposed to carry out an advanced search of the literature in order to clarify its clinical, radiological, and therapeutic characteristics to achieve its evaluation from a holistic point of view.