Shrinking Lungs Syndrome (SLS) | Systemic Lupus Erythematosus

 

Patient selection: SLE

 

Clinical features:

(1) dyspnea

(2) reduced exercise capacity

(3) restrictive pattern on pulmonary function testing (PFT)

 

Imaging studies:

(1) small lung volumes for age and gender

(2) elevated diaphragm (unilateral or bilateral)

(3) basilar atelectasis

 

Additional studies demonstrate:

(1) diaphragmantic dysfunction on electromyogaphy

(2) phrenic nerve palsy on phrenic nerve stimulation

(3) esophageal and gastric pressure measurements

 

Differential diagnosis:

(1) myopathy (especially after high dose corticosteroid therapy)

(2) other syndromes of pulmonary and/or pleural involvement in SLE

 

Therapy may include use of corticosteroids, inhaled beta-agonists, immunosuppression and/or theophylline.