Thread by @nickmmark

Here's another #Pulmonary teaching case: #FOAMed

A middle aged woman presents with many months of pleuritic chest pain and dyspnea. PFTs and CXR are shown (current CXR on right, prior from 5 yrs ago on left). DLCO is normal. HRCT shows no parenchymal lung disease.
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Which of the following co-morbid conditions might explain the abnormal CXR and PFT findings:

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Shrinking Lung Syndrome (SLS) is a rare complication of SLE or occasionally other #autoimmune/#rheumatologic conditions.

SLS is characterized by a triad of dyspnea, pleuritic chest pain, & progressive decrease in lung volumes w/o interstitial disease.

Here are her serologies
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First described in 1965, SLS is thought to be caused by repeated episodes of pleuritis causing limited muscle engagement and reduced inflation, leading to a progressive decrease in lung volumes and compliance.

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The prognosis of Shrinking Lung Syndrome (SLS) is good. Most patients respond favorably to a course of glucocorticoids (typically 40-60mg prednisone daily), with lung function & imaging often returning (almost) to baseline.

Well illustrated by this case:
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To summarize what we’ve learned about SLS:
* occurs in <1% of pts with SLE (rarely other rheum conditions)
* characterized by pleuritic chest pain, dyspnea, & decreased lung volumes w/ normal parenchyma
* due to repeated episodes of pleuritis
* is usually steroid responsive

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