Table 1.

Presentation of shrinking lung syndrome (SLS). Six subjects had active SLS symptoms during the study interval and underwent evaluation of pulmonary mechanics.

CaseRheumatologic DiagnosisSexAge at Rheumatologic DiagnosisAge at SLS DiagnosisSymptoms of SLSChest ImagingSLS TreatmentSLS Outcome
1MCTDF1415Dyspnea, pleuritic CP, orthopneaLow lung volumes, atelectasis, small R effusion, decreased diaphragm excursionMethylpred 1 g× 3, pred 80 mg/day, rituximab 1 g × 2Remission
2MCTDM1515Dyspnea, pleuritic CP, orthopneaLow lung volumes, bilateral atelectasis, small bilateral effusionsMethylpred 1 g × 3, pred 10 mg/day, CYC 750 mg IV Q2 wks, theophylline 300 mg/dayRemission
3SLEF1822DyspneaLow lung volumes, paraspinal atelectasis, small bilateral effusionsPred 10 mg/day, theophylline 600 mg/day, meloxicam 7.5 mg/dayNA
4SLEF1518Dyspnea, pleuritic CPLow lung volumes, bilateral atelectasis, small bilateral effusions, bilateral decreased diaphragm excursionPred 40 mg/day, MMF 1000 mg/dayNA
5SLEM4345Dyspnea, pleuritic CP, orthopneaLow lung volumes, bilateral atelectasis, moderate bilateral effusions, bilateral decreased diaphragm excursionPred 60 mg/day, MMF 3000 mg/dayRemission
6SLEF1214Dyspnea, pleuritic CP, orthopneaElevated R hemi-diaphragmPred 60 mg/day, rituximab with CYC × 2, naproxen 1000 mg/dayActive
  • MCTD: mixed connective tissue disease; SLE: systemic lupus erythematosus; CP: chest pain; methylpred: intravenous methylprednisolone; pred: prednisone; CYC: cyclophosphamide; MMF: mycophenolate mofetil; NA: not available.