Presentation of shrinking lung syndrome (SLS) in 3 female subjects who did not undergo evaluation of pulmonary mechanics. Subjects A and B presented before esophageal manometry was routine at our institutions; Subject C had comorbidities that rendered study impractical. The results demonstrate that these subjects were phenotypically similar to the 6 patients tested.
| Rheumatologic Diagnosis | Age at SLS Diagnosis | Symptoms of SLS | Imaging Results | FVC, % Predicted | TLC, % Predicted | Corrected DLCO, % Predicted | |
|---|---|---|---|---|---|---|---|
| Normal | > 80 | > 80 | > 80 | ||||
| Subject A | UCTD | 14 | Dyspnea, pleuritic CP, orthopnea | CXR: NA CT: No ILD | 61 | 81 | Normal |
| Subject B | SLE | 56 | Dyspnea, pleuritic CP, orthopnea | CXR: Elevated R diaphragm CT: No ILD, bilateral atelectasis | 84 | 77 | 86 |
| Subject C | SLE | 12 | Dyspnea, pleuritic CP | CXR: LLV, elevated R diaphragm, small bilateral effusions CT: No ILD | 33 | 48 | 81 |
CP: chest pain; CT: computed tomography; CXR: chest radiograph; DLCO: diffusing capacity of lung for carbon monoxide; FVC: forced vital capacity; ILD: interstitial lung disease; LLV: low lung volumes; NA: not available; SLE: systemic lupus erythematosus; TLC: total lung capacity; UCTD: undifferentiated connective tissue disorder.