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Table 1 Clinical features, treatment and outcome of patients with SLS associated with SLE who received treatment with rituximab

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
[1] F Unknown 61 Chest pain, history of pleurisy, dyspnea Elevated diaphragms, atelectasis, pleural thickening TLC 46%,
DLCO 25%, KCO 59%
CS + Beta-agonists + RTX (dose unknown) + Physiotherapy Improvement
[1] F Unknown 26 Chest pain, history of pleurisy, dyspnea Pleural thickening, reticulations FVC 41%,
TLC 68%,
DLCO 34%
CS + AZA + MMF + RTX (dose unknown) Improvement
[3] F 36 46 Dyspnea on exertion, orthopnea, pleuritic chest pain Elevated diaphragms, atelectasis, pleural thickening FVC 77%, TLC 68% CS + CYC + RTX (375 mg/m2 once weekly × 4 q6mo) Asymptomatic, normal PFTs
[4] F 28 28 (6 mo after diagnosis of SLE) Dyspnea, pleuritic chest pain, dry cough, orthopnea Elevated diaphragms FVC 61%, TLC 45% Beta-agonists + theophylline, RTX (1 g × 2, 2 weeks apart) + CYC Asymptomatic, normal PFTs
[6] F 38 38a Tachypnea, dyspnea Normal HRCT, elevated diaphragms FVC 64%, FEV1 73% CS + CYC without improvement; followed by RTX (1 g × 2, 2 weeks apart) Normal PFTs, normal CXR 6 months after treatment
[7] F 11 14 Dyspnea on exertion, chest pain Low lung volumes, small bilateral 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%
[14] F 22 27 Pleuritic chest pain, exertional dyspnea Elevated diaphragms, normal HRCT FVC 1.45 L (predicted value 4.20), TLC 2.35 (predicted value 5.76), DLCO 16.3 (predicted value 26.5) CS + RTX (375 mg/m2 × 2 doses 6 weeks apart) Initial clinical improvement, followed by re-presentation requiring second course of RTX. Improvement reported 2 yrs. later
[15] F 22 57 Dyspnea, dry cough, pleuritic chest pain Elevated diaphragms, bibasilar atelectasis FVC 43%, TLC 56%, DLCO 55% CS + beta-agonists + AZA + MMF, then 6 mo later RTX (1 g × 2 doses, 2 weeks apart, repeated q6mo) Clinical improvement. PFTs 5 years post: FVC 76%, TLC 79%, DLCO 53%
[16] F Unknown 28 Exercise intolerance, pleuritic chest pain Unknown FVC 0.99 L CS + MMF + RTX (2800 mg) Unlimited exercise tolerance, FVC 2.23 L
[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
[8] F 36 37 Dyspnea, pleuritic chest pain CXR: Bilateral diaphragmatic elevation with mild pleural effusion
HRCT: Mild pleural effusion
Restrictive pattern CS + MTX + beta-agonists + RTX (dose unknown) Restrictive defect improvement
[8] F 36 39 Dyspnea, pleuritic chest pain, fever CXR: Unilateral diaphragmatic elevation, left atelectasia
HRCT: Basal atelectasis
Restrictive pattern CS + MMF + beta-agonists + RTX (dose unknown) Restrictive defect stabilization. Developed ILD 4 yrs. later
[8] F 27 31 Dyspnea, pleuritic chest pain CXR: Unilateral diaphragmatic elevation, right atelectasia
HRCT: Basal atelectasis, mild pleural effusion
Restrictive pattern CS + theophylline + beta-agonists + RTX (dose unknown) Restrictive defect stabilization
[8] F 23 30 Dyspnea, pleuritic chest pain CXR: Unilateral diaphragmatic elevation
HRCT: Basal atelectasis
Restrictive pattern CS + MMF + beta-agonists + RTX (dose unknown) Restrictive defect improvement
[8] F 34 59 Dyspnea, pleuritic chest pain CXR: Bilateral diaphragmatic elevation, atelectasia
HRCT: Basal atelectasis
Restrictive pattern CS + MMF + RTX (dose unknown) + IVIG Restrictive defect stabilization
  1. CS corticosteroids, RTX rituximab, CYC cyclophosphamide, AZA azathioprine, MMF mycophenolate mofetil, IVIG intravenous immunoglobulin, 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, ILD interstitial lung disease. PFT results expressed in % predicted when available
  2. a Diagnosis of SLS made at the time of diagnosis of SLE