Fig. 2

Evaluation and management of patients with Sjögren’s disease and symptoms/signs of interstitial lung disease. Recommendations for evaluation and management of patients with Sjögren’s disease and symptoms and/or signs of interstitial lung disease developed by the Sjögren’s Foundation [81]. ^aDose and duration of corticosteroids in Sjögren’s-ILD are not standardized. The panel proposed ≤ 60 mg daily of prednisone with a slow taper over weeks/months. In rapidly progressive ILD or acute respiratory failure, pulse-dose IV corticosteroids or high-dose oral corticosteroids up to 60 mg daily of prednisone should be considered. ^bSteroid-sparing agents should be initiated as maintenance therapy in patients who are not able to taper off corticosteroids or who experience adverse effects or if long-term corticosteroid therapy is predicted. ^cCondition rapidly deteriorates and requires hospitalization. ^dNintedanib is approved by the US Food and Drug Administration for the treatment of progressive fibrotic lung disease. ^eCalcineurin inhibitors can be considered in patients who are intolerant to the initial maintenance therapy; there is no evidence to support superiority in patients who fail first-line therapy. AZA, azathioprine; CYC, cyclophosphamide; CYP, cyclosporine; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; MMF, mycophenolate mofetil; PFTs, pulmonary function tests; PH, pulmonary hypertension; RTX, rituximab. Reprinted from Chest, Vol 159, Lee et al., Consensus guidelines for evaluation and management of pulmonary disease in Sjögren’s, pages no. 16, Copyright 2021, with permission from Elsevier