Abstract

Shrinking lung syndrome (SLS) is a rare complication of systemic lupus erythematosus (SLE), however it was also reported with other connective tissue diseases (CTDs). It is characterized by its low prevalence with higher incidence in females patients. Although it is much more frequent in advanced stages of the disease, rarely, SLS may be the presenting manifestation of SLE. It was found that it mostly occurs in patients without previous or concomitant major organ involvement, moreover in inactive stages of the disease. Progressive exertional dyspnea accompanied by pleuritic chest pain are considered the dominant symptoms in SLS. Pulmonary function tests (PFTs) with reduced lung volumes and radiographic finding of elevated diaphragmatic copula are the characteristic features in SLS. There are no standardized guidelines for the treatment of SLS in SLE. However, the majority of patients showed good response on medium to high doses of glucocorticoids. Moreover, an immunosuppressive agent should be added if the patient fails to improve on steroids alone.