Table 1. Presentation of Shrinking Lung Syndrome.

	Rheum Dx	Sex	Age at Rheum Dx	Age at SLS Dx	Symptoms of SLS	Chest Imaging	SLS Treatment	SLS Outcome
Case 1	MCTD	F	14	15	Dyspnea, Pleuritic CP, Orthopnea	Low lung volumes, atelectasis, small R effusion, decreased diaphragm excursion	Methylpred 1gm × 3, Pred 80mg/day, Rituximab 1 gm × 2	Remission
Case 2	MCTD	M	15	15	Dyspnea, Pleuritic CP, Orthopnea	Low lung volumes, bilat atelectasis, small bilat effusions	Methylpred 1gm × 3, Pred 10mg/ day, Cy 750mg IV Q2 wks, Theophylline 300mg/day	Remission
Case 3	SLE	F	18	22	Dyspnea	Low lung volumes, paraspinal atelectasis, small bilat effusions	Pred 10mg/day, Theophylline 600mg/day, Meloxicam 7.5mg/day	N/A
Case 4	SLE	F	15	18	Dyspnea, Pleuritic CP	Low lung volumes, bilat atelectasis, small bilat effusions, bilat decreased diaphragm excursion	Pred 40mg/day, MMF 1000mg/day	N/A
Case 5	SLE	M	43	45	Dyspnea, Pleuritic CP, Orthopnea	Low lung volumes, bilat, atelectasis, moderate bilat effusions, bilat decreased diaphragm excursion	Pred 60mg/day, MMF 3000mg/day	Remission
Case 6	SLE	F	12	14	Dyspnea, Pleuritic CP, Orthopnea	Elevated R hemi-diaphragm	Pred 60mg/day, Rituximab with Cytoxan × 2, Naproxen 1000mg/day,	Active

Six subjects, who had active SLS symptoms during the study interval and underwent pulmonary mechanics evaluation, are presented. Rheum Dx = rheumatologic diagnosis; SLS = shrinking lung syndrome; Dx= diagnosis; MCTD = mixed connective tissue disease; SLE = systemic lupus erythematosus; F = female; M = male; CP = chest pain; R = right; bilat = bilateral; methylpred = intravenous methylprednisolone; pred = prednisone; Cy = cyclophosphamide; wks = weeks; MMF = mycophenolatemofetil; N/A = not available