Evaluation and Management of Pulmonary Disease in Sjögren’s
Publication Date: October 14, 2020
Last Updated: October 3, 2022
Recommendations
Evaluating Patients With Sjögren’s
Evaluating asymptomatic Sjögren’s patients for pulmonary complications
1. Serologic biomarkers must not be employed to evaluate for pulmonary involvement in patients with established Sjögren’s disease. (Intermediate, Strong)
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2. Due to the prevalence of respiratory involvement in Sjögren’s, clinicians must obtain a detailed medical history inquiring about respiratory symptoms in all Sjögren’s patients at the initial and every subsequent visit. (High, Strong)
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3. In Sjögren’s patients without respiratory symptoms, a baseline two-view chest radiograph may be performed. The baseline chest radiograph can:
(1) help identify pulmonary involvement despite the absence of symptoms,
(2) identify alternate etiologies of sicca symptoms such as sarcoidosis, vasculitis, and lymphoma, and
(3) serve as a baseline for future comparisons.
(Intermediate, Weak)(2) identify alternate etiologies of sicca symptoms such as sarcoidosis, vasculitis, and lymphoma, and
(3) serve as a baseline for future comparisons.
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4. In Sjögren’s patients who have no respiratory symptoms, baseline complete PFTs may be considered to evaluate for the presence of underlying pulmonary manifestations. PFTs should include pre- and post-bronchodilator spirometry, lung volumes, and diffusing capacity of the lung for carbon monoxide. Abnormalities identified may require further corroboration with advanced testing. (Intermediate, Weak)
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5. In asymptomatic Sjögren’s patients, routine echocardiogram is not recommended. (Intermediate, Strong)
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Evaluating Sjögren’s patients with pulmonary symptoms
1A. In Sjögren’s patients with chronic cough and/or dyspnea, complete PFTs and HRCT should be done to evaluate for pulmonary involvement. (Intermediate, Moderate)
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1B. In a Sjögren’s patient with respiratory symptoms, the interval for repeat HRCT and PFTs must be determined on a case-by-case basis and individualized according to the nature and severity of the underlying pulmonary abnormality and the degree of symptoms and functional impairment. (Insufficient, Strong)
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2. In a Sjögren’s patient with dyspnea, an echocardiogram is recommended in the following circumstances:
- a) In patients with suspected pulmonary hypertension
- b) In patients with unexplained dyspnea after pulmonary etiologies (asthma, small airway disease, bronchiectasis, ILD) have been excluded
- c) In patients with suspected cardiac involvement.
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3. In a Sjögren’s patient with respiratory symptoms, a CTPA to look for pulmonary embolism must not be performed routinely in all patients but rather dictated by clinical suspicion for pulmonary embolism in individual circumstances. If clinically concerned about a pulmonary embolism, CTPA is the confirmatory test of choice.
Ventilation-perfusion scan should be considered only in the following circumstances:
Ventilation-perfusion scan should be considered only in the following circumstances:
a) To rule out chronic thromboembolic pulmonary hypertension in patients with pulmonary hypertension
b) When clinical concern for pulmonary embolism exists, and a physician is unable to do a CTPA because of patient allergy to contrast or renal insufficiency.
(Low, Strong)b) When clinical concern for pulmonary embolism exists, and a physician is unable to do a CTPA because of patient allergy to contrast or renal insufficiency.
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Overview
Title
Evaluation and Management of Pulmonary Disease in Sjögren’s
Authoring Organization
American College of Chest Physicians