Evaluation and Management of Pulmonary Disease in Sjögren’s

Patient Guideline Summary

Publication Date: October 15, 2020

Objective

Objective

This patient summary means to discuss key recommendations from the American College of Rheumatology for Pulmonary Disease in Sjögren’s.

Overview

Overview

  • Sjögren’s syndrome is an immune disorder most commonly causing dry mouth and dry eyes. It can accompany other immune disorders.
    • It can appear at any age and is much more common in women.
    • 10%–20% of patients with Sjögren’s syndrome will have lung involvement.
    • Sjögren’s syndrome increases the risk of lymphoma.
  • Symptoms include dry cough, dry eyes, dental problems, face or jaw swelling, trouble swallowing, joint and muscle pain, and extreme fatigue.
    • Lung symptoms include productive cough, chest pain, and shortness of breath.
  • This patient summary focuses on the diagnosis and management of lung conditions arising from Sjögren’s syndrome.

Diagnosis

Diagnosis

  • Because of the wide range of conditions associated with Sjögren’s syndrome, a variety of medical specialists should be involved in both evaluation and treatment of every patient.
  • Likewise, the evaluation must be comprehensive.
  • Other conditions with similar symptoms like asthma and gastroesophageal reflux should be considered.
  • The initial evaluation should include establishing a baseline for anticipated conditions that have not yet appeared.
  • For lung involvement, testing should include lung function and imaging.
  • Other tests should be selected individually.

Treatment

Treatment

  • Smoking cessation is strongly recommended.
  • Vaccination against respiratory infections like influenza and pneumococcus is strongly recommended.
  • Respiratory therapy, humidification, and medicines that increase or improve fluid production are helpful.
  • Other medication options include:
    • Cortisone-like drugs
    • Asthma drugs
    • Antibiotics for infections
  • If symptoms worsen, further evaluation and treatment will be required. Possible interventions include:
    • Further lung imaging and function testing
    • Oxygen assessment and prescription
    • Pulmonary rehabilitation
    • Lung biopsy
    • Increased medication
  • Throughout the course of the disease, family and caretakers should be involved and educated in the particulars of the patient’s condition.
  • As treatment intensifies, risks increase. Full discussions of each treatment option are required so that the patient, friends and family, and all medical personnel are fully informed.

Source Citation

Lee AS, Scofield RH, Hammitt KM, Gupta N, Thomas DE, Moua T, Ussavarungsi K, St Clair EW, Meehan R, Dunleavy K, Makara M, Carsons SE, Carteron NL; Consensus Expert Panel (CEP) Members. Consensus Guidelines for Evaluation and Management of Pulmonary Disease in Sjögren's. Chest. 2021 Feb;159(2):683-698. doi: 10.1016/j.chest.2020.10.011. Epub 2020 Oct 16. PMID: 33075377; PMCID: PMC8438162.

Disclaimer

The information in this patient summary should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.