Background/Purpose:

Shrinking lung syndrome (SLS) is a rare a little known complication associated with systemic lupus erythematosus (SLE) and other connective tissue diseases (CTDs). This study describes the clinical features, investigations, management, and outcome of a series of patients with CTDs and SLS.

Methods:

Ambispective study of 9 patients with SLS and CTDs

Results:

All nine patients were women, with a mean age at SLS diagnosis of 42 years (SD: 11; range 30-64). Eight of these patients had SLE and 1 had mixed connective tissue disease (MTCD). The prevalence of SLS in our cohort of SLE patients was 1,8% (8/429). The median time to onset of SLS after SLE/MCTD diagnosis was 7 years (SD: 6.8; range 1.6-25 yrs). Most patients with SLE had a severe disease with concomitant or previous major organ involvement, especially lupus nephritis (75% of cases). All presented progressive exertional dyspnea of variable severity accompanied by pleuritic chest pain in 89% (8/9) of cases. Dry cough or fever was more rarely seen (33%). In 56% (5/9) of patients, the syndrome was bilateral. Imaging techniques (chest X-Ray and HRCT) at the evolution showed elevated hemidiaphragms without evidence of interstitial lung disease (ILD) in all cases. Pleural thickening/effusions or basal atelectasis were observed in 56% and 89% of patients, respectively. Pulmonary function tests (PFT) were consistent with a restrictive defect (mean baseline FVC: 59%), while the DLCO was decreased in 89% of cases (mean baseline DLCO: 44.95%). In those to whom diaphragmatic function tests were made, a decrease of the minimum inspiratory pressures (MIP) was observed. Treatment included 0.5 to 1 mg/kg/day of oral prednisone (associated with short-term pulses of intravenous methylprednisolone in 2 cases), associated with beta-agonist. Concomitant immunosuppressive agents (azathioprine, mycophenolate, or methotrexate) were used in 5 patients and rituximab in 4. At the end of the follow-up period (median 31.5 months; range, 3-192), 1 patient cured without functional sequelae. The remaining 8 patients had subjective improvement with stabilization or mild to moderate improvement on PFT. One of these patients finally developed ILD (nonspecific interstitial pneumonitis) several years after SLS onset.

Conclusion:

SLS is a rare complication in CTDs which must be suspected in patients with dyspnea and/or pleuritic chest pain, lung volume reduction with no parenchymal abnormalities and a restrictive ventilatory defect on PTF. The frequent presence of pleuritic chest pain and pleural thickening/effusions at the time of evaluation, suggests that pleuritic inflammation may have an important role in the pathogenesis of this complication.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/shrinking-lung-syndrome-in-connective-tissue-disease/