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