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Table 2 Clinical features, treatment, and outcome of reported pediatric cases of SLS associated with SLE

From: Shrinking lung syndrome treated with rituximab in pediatric systemic lupus erythematosus: a case report and review of the literature

Reference Sex Age at SLE diagnosis Age at SLS diagnosis Clinical presentation Imaging findings at SLS diagnosis PFTs at SLS diagnosis Treatment Outcome
[2] F 12 12a Prior diagnosis of mycoplasma pneumonia with recovery. Re-presented 6 months later with dyspnea CXR: Enlarged cardiac silhouette, low lung volumes, elevated diaphragms
HRCT: thoracic lymphadenopathy
FEV1 34%, FVC 27%, TLC 59% CS + CYC (q4weeks × 6 mo) Asymptomatic. Normal PFTs after 1 yr (FEV1 99%, FVC 97%, TLC 92%)
[7] F 11 14 Dyspnea on exertion, chest pain CXR: Low lung volumes, small pleural effusions, small pericardial effusion, mild bibasilar atelectasis FVC 31%, TLC 32%, DLCO 96% CYC monthly × 1 year, then RTX (dose unknown) Clinical improvement,
PFTs 2 yrs. post: FVC 82%, TLC 80%
[10] F 15 15a Pleuritic chest pain, dry cough, dyspnea on exertion Small lung fields, elevated bilateral hemidiaphragms, chest CT normal FEV1 26%, FVC 25%, TLC 31% Beta-agonist Clinical improvement. PFTs after 12d showed FEV1 increase of 58%, FVC increase of 50%, TLC increase of 47%
[17] M 11 14 Fatigue, dyspnea, pleuritic chest pain Enlarged cardiac silhouette, atelectasis, severely reduced diaphragmatic excursion on fluoroscopy FEV1 23%, FVC 20%, TLC 34% CS + AZA Follow-up 23 days later: FEV1 45%, FVC 45%, TLC 57%
[18] F 12 12a Pleuritic chest pain, dyspnea, fever, fatigue, anorexia CXR: Reduced lung volumes, elevated diaphragms, HRCT: pleural thickening Diaphragmatic fluoroscopy: minimal movement FVC 39%, TLC 60%, DLCO normal CS + HCQ 4 yrs. post: ongoing activity limitation, PFTs unchanged
[12] F 12 14 Dyspnea, pleuritic chest pain, orthopnea Elevated right hemidiaphragm FVC 36%, TLC 39%, DLCO 102% CS + RTX (dose unknown) + CYC Active disease
  1. CS corticosteroids, RTX rituximab, CYC cyclophosphamide, AZA azathioprine, HCQ hydroxychloroquine, CXR chest X-ray, HRCT high-resolution computed tomography, PFTs pulmonary function tests, FEV1 forced expiratory volume in 1 s, FVC forced vital capacity, TLC total lung capacity, DLCO diffusing capacity for carbon monoxide. PFT results expressed in % predicted when available
  2. a Diagnosis of SLS made at the time of diagnosis of SLE