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
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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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