Shrinking lung syndrome in systemic lupus erythematosus patients; clinical characteristics, disease activity and damage.

AIM: To detect the prevalence of shrinking lung syndrome (SLS) among systemic lupus erythematosus (SLE) patients and study their clinical, laboratory and radiological characteristics and differences in disease activity and damage.

METHODS: The study included 200 Egyptian SLE patients and SLS was considered in those with exertional dyspnea, restrictive pulmonary function tests (PFTs) and elevated copula of the diaphragm. Full history taking, thorough clinical examination, laboratory and relevant radiological investigations were performed for all the patients. High-resolution computed tomography scans of the chest were performed for patients with radiological findings consistent with SLS and those with pulmonary manifestations.

RESULTS: The mean age of the patients was 29.3 ± 8.4 years, mean disease duration 5.81 ± 4.32 years and female to male ratio was 9 : 1. SLS was present in 27 patients (13.5%) with a female to male ratio of 3.5 : 1.0. The demographic features, clinical and laboratory manifestations, renal biopsy class, disease activity and damage scores, PFTs and radiological findings of the SLE patients are presented.

CONCLUSION: Shrinking lung syndrome is not rare and presents a considerable subset of SLE patients. In SLE patients with dyspnea or chest pain, SLS should be looked for and PFTs are highly suggestive.