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