The Shrinking Lungs Syndrome in Systemic Lupus Erythematosus

Abstract

A comprehensive review of the literature on shrinking lungs syndrome (SLS) in systemic lupus erythematosus involved a MEDLINE search (1965-1997) of case reports and clinical series of patients with the diagnosis of SLS. A total of 49 well-documented cases of SLS were reviewed. Shrinking lungs syndrome is characterized by unexplained dyspnea, a restrictive pattern on pulmonary function test results, and an elevated hemldlaphragm. The cause of SLS remains controversial, with several authors attributing the disorder to diaphragmatic weakness and others suggesting that chest wall restriction accounts for the clinical syndrome. No definitive therapy exists. Corticosteroids have been reported to lessen symptoms

Shrinking lungs syndrome, a rare manifestation of systemic lupus erythematosus

“…The previous generalised myophatic compromise, without enzymatic mobility (as in case 2) has been reported by other authors (3).…”

“…In addition, both patients presented hypoventilation on both pulmonary bases and crackling rales. These findings have been attributed to the presence of basal atelectasis (3), confirmed by CXR and HRCT scan.…”

“…Although in 1977 Gibson et al (2) reported a prevalence of 23% of SLS, there are few cases of this entity reported in world literature (3). Recently, the Saint Thomas Hospital of London group stated that only seven patients of a cohort of 2650 followed for 17 years presented findings compatible with SLS, which represents a prevalence of 0.26% (4).…”

“…Physical examination may evidence tachypnea and the use of accessory respiratory muscles (3,5). In addition, both patients presented hypoventilation on both pulmonary bases and crackling rales.…”

Shrinking lung syndrome

“…Exercise intolerance over weeks to months is the common mode of presentation and is seen months to years after SLE is diagnosed. The respiratory dysfunction appears to be independent of the clinical course, duration and lupus antibodies (ANA and anti-DNA) 3. Characteristic diagnostic findings include elevated diaphragms on imaging and restrictive lung volumes on pulmonary function tests 2.…”