Consensus Guidelines for Evaluation and Management of Pulmonary Disease in Sjögren's

Abstract

Background: Pulmonary disease is a potentially serious yet underdiagnosed complication of Sjögren's syndrome, the second most common autoimmune rheumatic disease. Approximately 16% of patients with Sjögren's demonstrate pulmonary involvement with higher mortality and lower quality of life.

Research question: Clinical practice guidelines for pulmonary manifestations of Sjögren's were developed by the Sjögren's Foundation after identifying a critical need for early diagnosis and improved quality and consistency of care.

Study design and methods: A rigorous and transparent methodology was followed according to American College of Rheumatology guidelines. The Pulmonary Topic Review Group (TRG) developed clinical questions in the PICO (Patient, Intervention, Comparison, Outcome) format and selected literature search parameters. Each article was reviewed by a minimum of two TRG members for eligibility and assessment of quality of evidence and strength of recommendation. Guidelines were then drafted based on available evidence, expert opinion, and clinical importance. Draft recommendations with a clinical rationale and data extraction tables were submitted to a Consensus Expert Panel for consideration and approval, with at least 75% agreement required for individual recommendations to be included in the final version.

Results: The literature search revealed 1,192 articles, of which 150 qualified for consideration in guideline development. Of the original 85 PICO questions posed by the TRG, 52 recommendations were generated. These were then reviewed by the Consensus Expert Panel and 52 recommendations were finalized, with a mean agreement of 97.71% (range, 79%-100%). The recommendations span topics of evaluating Sjögren's patients for pulmonary manifestations and assessing, managing, and treating upper and lower airway disease, interstitial lung disease, and lymphoproliferative disease.

Interpretation: Clinical practice guidelines for pulmonary manifestations in Sjögren's will improve early identification, evaluation, and uniformity of care by primary care physicians, rheumatologists, and pulmonologists. Additionally, opportunities for future research are identified.

Keywords: Sjögren; Sjögren's; guideline; lung; pulmonary.

Figure 1

Respiratory evaluation for Sjögren’s patients. ^aThe benefit of obtaining baseline PFTs in asymptomatic Sjögren’s patients regarding long-term outcomes is not clear. This paucity of evidence and the potential costs of the test should be taken into account and discussed with individual patients prior to proceeding with PFTs. Complete PFTs includes spirometry, Dlco, and lung volumes, ideally measured by body plethysmography. CXR = chest radiograph; Dlco = diffusing capacity of the lung for carbon monoxide; HRCT = high-resolution CT; PFTs = pulmonary function tests.

Figure 2

Evaluation and management of patients with Sjögren’s who exhibit symptoms and/or physical examination signs of airway disorders.^, Details regarding PFTs and HRCT examination are given in Figure 1. HRCT = high-resolution CT; pulmonary function tests.

Figure 3

Evaluation and management of patients with Sjögren’s who exhibit symptoms and/or physical examination signs of interstitial lung disease. Details regarding PFTs and HRCT examinations are given in Figure 1. ^aThe dose and duration of corticosteroids in Sjögren’s-ILD is not standardized. The panel proposes a dose not to exceed 60 mg daily of prednisone with a slow taper over weeks-months. In rapidly progressive ILD, or acute respiratory failure, consider pulse dose IV corticosteroids or high-dose oral corticosteroids up to 60 mg daily of prednisone. ^bIn patients who are not able to successfully taper off corticosteroids, or experience unfavorable adverse effects, or in patients where the length of corticosteroid therapy is predicted to be long-term, steroid-sparing agents should be initiated as maintenance therapy. ^cCondition rapidly deteriorates and requires hospitalization. ^dNintedanib is approved by the US Food and Drug Administration for progressive fibrotic lung disease phenotype. ^eCalcineurin inhibitors can be considered in patients who are intolerant to the initial maintenance therapy; no evidence to support the superiority in patients who fail the first-line therapy. AZA = azathioprine; CYP = cyclophosphamide; HRCT = high-resolution CT; ILD = interstitial lung disease; MMF = mycophenolate mofetil; PFTs = pulmonary function tests; PH = pulmonary hypertension; RTX = rituximab.