Symptoms of shrinking lung syndrome reveal systemic lupus erythematosus in a 12-year-old girl

Abstract

While pleuropulmonary involvement in systemic lupus erythematosus (SLE) is a common occurrence, shrinking lung syndrome (SLS) is a rare complication of SLE, particularly in children. We report on a teenager girl with a primary SLE diagnosis, which was based upon clinical, imaging, lung-function and histological findings ascertained to be compatible with SLS. Following a pneumonia, the patient developed inflammatory residues in the lower lobes, an event that probably caused diaphragmatic immobility and subsequently led to SLS. Treatment response to steroids, cyclophosphamide and hydroxychloroquine in this case was excellent, and efficacy was more profound than previously has been reported in the literature with respect to pediatric patients. This case report argues that prognosis of SLS in SLE is likely to be favorable when the diagnosis is made early and the disease is treated appropriately.

Organ involvement other than lupus nephritis in childhood-onset systemic lupus erythematosus

“…The etiology of SLS remains unclear, but evidence of diaphragmatic weakness/dysfunction has been demonstrated. 13,14 Prognosis is generally good with treatment, which includes corticosteroids, with addition of azathioprine or other immunosuppressive agents, if needed. 13…”

“…The etiology of SLS remains unclear, but evidence of diaphragmatic weakness/ dysfunction has been demonstrated. 13,14 Prognosis is generally good with treatment, which includes Organ involvement other than lupus nephritis in childhood-onset SLE JL Huggins et al…”

“…8,12 Shrinking lung syndrome Shrinking lung syndrome (SLS) is extremely rare in cSLE, with a recent report citing only six published cases in the literature. 13 Typical clinical features of SLS include progressive, sometimes episodic dyspnea and pleurisy, with a marked restrictive pattern on PFT, and in some cases elevated diaphragms. 13,14 The DLCO may be decreased, but usually normalizes with correction for reduced lung volumes.…”

“…13 Typical clinical features of SLS include progressive, sometimes episodic dyspnea and pleurisy, with a marked restrictive pattern on PFT, and in some cases elevated diaphragms. 13,14 The DLCO may be decreased, but usually normalizes with correction for reduced lung volumes. In the setting of isolated SLS, lung parenchymal imaging abnormalities are typically absent, 13,14 though atelectasis may be seen.…”

“…13,14 The DLCO may be decreased, but usually normalizes with correction for reduced lung volumes. In the setting of isolated SLS, lung parenchymal imaging abnormalities are typically absent, 13,14 though atelectasis may be seen. The etiology of SLS remains unclear, but evidence of diaphragmatic weakness/ dysfunction has been demonstrated.…”