Unshrinking the Lung: Successful Improvement of Shrinking Lung Syndrome in Systemic Lupus Erythematosus Through Non-invasive Ventilation and Respiratory Rehabilitation

Shrinking lung syndrome (SLS) is a rare pulmonary manifestation of systemic lupus erythematosus (SLE), characterized by progressive dyspnea and severe restrictive ventilatory impairment. The pathophysiology remains unclear, with proposed mechanisms involving diaphragmatic dysfunction, pleural inflammation, and neuromuscular impairment. We report the case of a 38-year-old non-smoking woman with a history of SLE, presenting with worsening dyspnea and hypoxemic respiratory failure. Pulmonary function tests revealed a severe restrictive pattern, and nocturnal oximetry demonstrated significant desaturation. Imaging showed no interstitial lung disease. A structured respiratory rehabilitation program, combined with nocturnal non-invasive ventilation (NIV) using intelligent volume-assured pressure support, resulted in substantial clinical and functional improvement. Over six months, the patient experienced significant symptom relief, pulmonary function stabilization, and resolution of nocturnal desaturation. This case highlights the potential role of NIV and pulmonary rehabilitation in improving respiratory function and quality of life in patients with SLS. Early diagnosis and a multidisciplinary approach are fundamental in optimizing patient outcomes. Further research is needed to establish standardized treatment protocols for SLS.