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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.