Manifestations and management of Sjögren's disease

Abstract

Sjögren's disease is a heterogeneous autoimmune disorder that may be associated with systemic manifestations such as pulmonary or articular involvement. Systemic complications have prognostic implications and need to be identified and managed in a timely manner. Treatment should be tailored to the type and severity of organ involvement, ideally based on multidisciplinary evaluation.

Keywords: Pulmonary fibrosis; Sjögren’s syndrome; Systemic manifestations; Treatment.

Fig. 1

Associations between immunological markers and disease phenotype based on ESSDAI domains. Heat map of the main associations between immunological markers and disease phenotype based on ESSDAI domains in patients with Sjögren’s disease in the Big Data Sjögren Project [Adapted from 22]. ESSDAI, EULAR Sjögren’s Syndrome Disease Activity Index; C3, complement component C3; C4, complement component C4; RF, rheumatoid factor; ANA, antinuclear antibodies

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