Evaluation and management of pulmonary disease in Sjogren's syndrome: Consensus Guideline

Recommendations for Evaluating Patients With Sjögren's

• Serologic biomarkers must not be employed to evaluate for pulmonary involvement in patients with established Sjögren's disease.
• 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.
• 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.
• 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.
• In asymptomatic Sjögren's patients, routine echocardiogram is not recommended.
• In Sjögren's patients with chronic cough and/or dyspnea, complete PFTs and HRCT should be done to evaluate for pulmonary involvement.
• 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.
• 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
• 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 only be considered 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
• In patients who have an uncharacterized ILD, diffuse cystic lung disease, or pulmonary lymphoma, clinical and serologic evaluation for Sjögren's is recommended.
• In a Sjögren's patient with respiratory symptoms, bronchoscopy with BAL must not be performed routinely but determined on a case-by-case basis and limited to special circumstances, such as the need to:
• a)Rule out infectious etiologies, especially in patients on immune suppression
• b)Rule out endobronchial abnormalities such as amyloidosis in patients with chronic cough not otherwise responsive to treatment
• c)Distinguish between other etiologies of sicca symptoms such as sarcoidosis
• In a Sjögren's patient with respiratory symptoms, use of bronchoscopy with endobronchial biopsies and transbronchial lung biopsy are not recommended for routine use.

• In Sjögren's patients with symptomatic vocal cord cystic lesions ("bamboo nodules"), less aggressive interventions, including voice therapy, inhaled corticosteroids, or intra-lesional corticosteroid injection, should be tried first. Surgical resection should be considered if initial measures fail, with consultation by a laryngologist with experience in Sjögren's.
• Sjögren's patients with dry bothersome cough and documented absence of lower airway or parenchymal lung disease must be assessed for treatable or preventable etiologies other than xerotrachea, including gastroesophageal reflux, postnasal drip, and asthma.
• In a Sjögren's patient with dry, nonproductive cough, humidification, secretagogues, and guaifenesin may be empirically initiated after exclusion of other causes.
• The use of humidification for improving positive airway pressure tolerance and compliance may be recommended in Sjögren's patients.
• Smoking cessation is recommended in all Sjögren's patients.
• In Sjögren's patients with symptomatic small airway disease, bronchoscopic biopsy is not recommended as part of routine assessment or evaluation.
• In Sjögren's patients with symptomatic small airway disease, complete pulmonary function testing must be performed to assess severity of small airway disease, and high-resolution CT imaging with additional expiratory views can be helpful in suggesting its presence.
• In Sjögren's patients with small airway disease, time-limited empiric therapy in newly diagnosed and previously untreated disease may include:
• A short course of systemic steroids for 2-4 weeks with a repeat spirometry to determine reversibility, especially if uncontrolled asthma is suspected
• Nebulized or inhaled short or long-acting bronchodilators and/or inhaled corticosteroids if there is physiological obstruction
• Short course (ie, 2-3 months) of empiric macrolide antibiotics (most commonly azithromycin 250 mg 3 days a week) for persistent, nonreversible, symptomatic bronchiolitis
• It is recommended that Sjögren's patients with clinically relevant bronchiectasis be treated similarly to those with primary or secondary bronchiectasis of other etiologies and may include any of the following:
• Mucolytic agents/expectorants
• Nebulized saline or hypertonic saline
• Oscillatory positive expiratory pressure
• Postural drainage
• Mechanical high-frequency chest wall oscillation therapies
• Chronic macrolides in those without non-tuberculous mycobacterium colonization or infection