In systemic lupus erythematosus (SLE), the respiratory system is frequently compromised. One of its uncommon manifestations is the shrinking lungs syndrome (SLS), characterised by dyspnoea, diaphragmatic elevation and a restrictive pattern in the spirometry. We report two cases affected with this rare entity. They presented with different degrees of respiratory involvement and responses to the therapy. At the same time, clinical, physiopathological, prognostic and therapeutic aspects of this syndrome are reviewed.