Evaluation and management of pulmonary disease in Sjogren's syndrome: Consensus Guideline
Written By : Medha Baranwal
Medically Reviewed By : Dr. Kamal Kant Kohli
Published On 2021-09-04 03:30 GMT | Update On 2021-09-04 09:42 GMT
Recommendations for ILD in Sjögren’s Patients
- In a Sjögren’s patient with suspected ILD, an HRCT with expiratory views is recommended.
- In a Sjögren’s patient with suspected ILD, oximetry testing is recommended as part of a patient’s initial evaluation.
- Baseline PFTs must be performed in all Sjögren’s patients with suspected or established ILD and followed initially at 3- to 6-month intervals for at least 1 year. Subsequent testing requires consideration of the type of ILD, the clinical course, and the pace of change noted on the serial PFTs. The baseline PFTs should include lung volumes by body plethysmography, spirometry, diffusing capacity, and oxygen saturations at rest and exercise.
- In a Sjögren’s patient with ILD, a surgical lung biopsy is not routinely recommended. A lung biopsy may be considered following a multidisciplinary review where a biopsy may have significant management implications, such as in:
- Neoplastic and non-neoplastic lymphoproliferative disorder
- Other cancers
- Amyloid
- Progressive deterioration and a suspected infection failing empiric therapies where less invasive testing proved nondiagnostic
- If a Sjögren’s-ILD patient is asymptomatic for lung disease or demonstrates minimal impairment on PFTs or HRCT, serial monitoring by PFTs is recommended every 3-6 months to establish disease trajectory and initiation of pharmacotherapy only if serial studies document a significant decline in lung function.
- Vaccination: All Sjögren’s patients must be immunized against influenza and pneumococcal infection (Prevnar and Pneumovax) in accordance with Centers for Disease Control and Prevention guidelines.
- Pneumothorax and cystic lung disease: Because a Sjögren’s patient with cystic lung disease might have an increased risk of pneumothorax, patients and caregivers/family must be educated about signs and symptoms of pneumothorax and instructed to seek immediate medical attention if they experience signs or symptoms.
- Pulmonary rehabilitation and ILD: In a symptomatic Sjögren’s patient with ILD and impaired pulmonary function, referral for pulmonary rehabilitation is recommended.
- Oxygen and ILD: In a Sjögren’s patient with suspected ILD and clinically significant resting hypoxemia (defined by resting oxygen saturation < 88%, Pao2 < 55 mm Hg or < 60 mm Hg with complication of chronic hypoxemia such as cor pulmonale), long-term oxygen therapy is recommended.
- Air travel and ILD: In a Sjögren’s-ILD patient considering air travel, the need for supplemental oxygen should be evaluated by a physician.
- Air travel and ILD: In a Sjögren’s patient with ILD, discouraging air travel is not recommended unless the patient develops signs and symptoms of pneumothorax or new onset/unexplained chest pain or dyspnea prior to boarding.
- Lung transplant and ILD: In a Sjögren’s patient with ILD whose condition is advanced with resting hypoxia or whose lung function is rapidly deteriorating, lung transplant evaluation is recommended.
- In Sjögren’s patients with symptomatic ILD with moderate to severe impairment on lung function, imaging, or in gas-exchange and especially in organizing pneumonia, systemic steroids should be considered as a first-line treatment at a dosage based on the clinical context and disease severity, with standard dosage being 0.5-1.0 mg/kg.
- Cautions for systemic corticosteroids: In a Sjögren’s patient with ILD or a related disorder, providers must be aware of the following risks/potential harms:
- Glucose intolerance
- Avascular necrosis
- Mineralocorticoid effect, leading to potential fluid retention and/or hypertension
- Myopathy
- Psychological, including hyperactivity, insomnia, psychosis
- Pancreatitis
- Hypertension
- Truncal obesity
- Acne
- Hematopoietic, including leukocytosis
- Ecchymosis
- Acanthosis nigricans
- Symptomatic/moderate-severe ILD—MMF or azathioprine: In a Sjögren’s patient with symptomatic ILD with moderate to severe impairment as determined by lung function testing, imaging, or gas-exchange, MMF or azathioprine should be considered when long-term steroid use is contemplated and steroid-sparing immunosuppressive therapy is required.
- Cautions for azathioprine: In a Sjögren’s patient with ILD or related disorder and considering use of azathioprine, patients and health-care providers must be aware of potential risks for drug-induced pneumonitis, GI upset, hepatotoxicity, bone marrow suppression, rash, and hypersensitivity syndrome. Testing for thiopurine methyltransferase activity or genotype before initiating azathioprine is recommended to reduce the risk of severe, life-threatening leukopenia due to complete lack of thiopurine methyltransferase activity.
- Cautions for MMF: In a Sjögren’s patient with ILD or related disorder and considering use of MMF, patients and health-care providers must be aware of potential side effects, including nausea, diarrhea, hepatotoxicity, and bone marrow suppression.
- Symptomatic/moderate-severe ILD—maintenance therapies: Following initial treatment for Sjögren’s patients with ILD who are symptomatic and in whom PFTs or HRCT demonstrated moderate-severe impairment, first-line maintenance drugs should be either MMF or azathioprine.
- Symptomatic/ moderate-severe ILD—second-line therapies: If initial treatment with MMF or azathioprine is insufficient or not tolerated in Sjögren’s patients with ILD who are symptomatic and in whom PFTs or HRCT demonstrated moderate-severe impairment, subsequent second-line maintenance drugs may include rituximab and calcineurin inhibitors, cyclosporine, or tacrolimus.
Recommendations for Lymphoproliferative Disease in Sjögren’s Patients
- The possibility of lymphoma must be further investigated in a Sjögren’s patient with symptoms such as unexplained weight loss, fevers, night sweats, and/or the presence of head and neck lymphadenopathy and/or parotitis.
- All Sjögren’s patients must be clinically monitored for signs and symptoms of pulmonary lymphoproliferative disorders, including lymphoma and amyloid.
- In Sjögren’s patients suspected of having lymphoproliferative complications, a HRCT chest scan should be considered more appropriate than a baseline CXR at the time of initial diagnosis.
- In a Sjögren’s patient with pulmonary lesions (nodules > 8 mm, consolidations, or lymphadenopathy) in whom a neoplasm is suspected, a PET scan should be considered.
- In Sjögren’s patients with lymphadenopathy, growing lung nodules, and/or progressive cystic lung disease, a biopsy should be recommended. Clinical and radiographic observation may be appropriate in select patients with incidental subcentimeter nodules, stable cysts, and isolated PET-negative subcentimeter lymphadenopathy.
- In a Sjögren’s patient in whom a neoplasm has been confirmed or suspected, multidisciplinary review involving rheumatologist/primary care physician, pulmonologist, pathologist, radiologist, and hematologist/oncologist is recommended.
Reference:
"Consensus Guidelines for Evaluation and Management of Pulmonary Disease in Sjögren’s," is published in the CHEST journal.
DOI: https://journal.chestnet.org/article/S0012-3692(20)34902-3/fulltext
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USA: The Sjogren's Foundation has released clinical practice guidelines for the evaluation and management of pulmonary disease in Sjögren's syndrome. The guideline, published in Chest journal, was developed after identifying a critical need for early diagnosis and improved quality and consistency of care.
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.
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.
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.
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.
Recommendations for Assessment and Management of Upper and Lower Airway Disease in Sjögren's Patients
- 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
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