What is your approach to the management of shrinking lung syndrome in SLE?

Young woman (20s) with history of active lupus for 3-4 years. Serologies/Labs include +ANA, +dsDNA,+ RNP, + Smith, hypocomplementemia Manifestations include: synovitis, lymphadenopathy, pleural effusion, pericardial effusion, episcleritis/tear duct swelling, cytopenias, and class IV lupus nephritis dx'd about 3 years ago. Treatment has been primarily directed at Class IV LN with HCQ, MMF 1.5g BID, Belimumab. Has reached renal remission on this treatment, with normalization of labs, complement, UPCR <500mg, no active urinary sediment. Other lupus symptoms under control. However, in the past year has had worsening dyspnea. CT Imaging shows no findings of ILD or pleural disease, but PFTs with restrictive defect FEV1/FVC 85, FEV1 48%, TLC 53%. TTE from last year without concern for pHTN. ICS/LABA did not affect symptoms. After discussing with pulmonary, SLS seems to be likely diagnosis.

2 Answers

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Rheumatology · Uniformed Services University of the Health Sciences (USUHS)

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No consensus exists on the best way to diagnose shrinking lung syndrome (SLS). Interestingly, I rarely see this anymore. Possibly due to better treatments and universal use of hydroxychloroquine (HCQ)?

We are not even sure how and why it occurs. Could it be a myopathy of the diaphragms or a result of ongoing pleural damage (and interestingly, she does have a history of serositis).

Your excellent description of a restrictive pattern with normal FEV1/FVC and no interstitial lung disease on imaging ...

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Rheumatology · MUSC Health

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I agree with the approach laid out by Dr. @Dr. First Last. As with Dr. @Dr. First Last, we rarely see it, maybe once a year. The only thing I can add is whether the patient is overweight. I have a young man with lupus and shortness of breath who we evaluated and he has restrictive lung disease with normal hi-res CT. He weighs over 300 pounds. In consultation with pulmonary, we agreed that the plan is for him to lose weight using available pharmaceutical and fitness and diet methods and then reas...

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