Annals of Thoracic Medicine Official publication of the Saudi Thoracic Society, affiliated to King Saud University
 
Search Ahead of print Current Issue Archives Instructions Subscribe e-Alerts Login 
Home Email this article link Print this article Bookmark this page Decrease font size Default font size Increase font size
ORIGINAL ARTICLE
Year : 2021  |  Volume : 16  |  Issue : 2  |  Page : 156-164

Clinical characteristics and outcomes in patients with primary Sjogren's syndrome-associated interstitial lung disease


1 Department of Medicine, Division of Pulmonary Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia
2 Department of Cardiac Science, College of Medicine, King Saud University, Riyadh, Saudi Arabia
3 Department of Otolaryngology, Head and Neck Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia
4 Department of Medicine, Division of Rheumatology Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia
5 Department of Pathology, College of Medicine, King Saud University, Riyadh, Saudi Arabia
6 Department of Radiology, College of Medicine, King Saud University, Riyadh, Saudi Arabia

Correspondence Address:
Prof. Esam H Alhamad
Department of Medicine (38), Pulmonary Division, P. O. Box 2925, College of Medicine, King Saud University, Riyadh 11461
Saudi Arabia
Login to access the Email id


DOI: 10.4103/atm.atm_632_20

Rights and Permissions

BACKGROUND: Diagnosing primary Sjogren's syndrome (pSS)-associated interstitial lung disease (ILD) is complex and can be very challenging. In addition, information about the prognostic factors is limited. AIMS: We aimed to determine the clinical characteristics and prognostic factors that impact pSS-ILD survival. METHODS: This retrospective review included 84 consecutive patients diagnosed with pSS-ILD. The information analyzed included the clinical characteristics, laboratory findings, and physiological and hemodynamic data. Prognostic factors were identified using a Cox proportional hazards regression model. RESULTS: The mean age was 60.5 years, and 61.9% were females. The mean time between the onset of symptoms and diagnosis was 21 months (range, 1–98 months). Minor salivary gland biopsy (MSGB) was positive for pSS in 92.3% of the cohort. Fifty percent of the patients had negative autoimmune serology related to pSS. Based on the available hemodynamic data, 40% had pulmonary hypertension (PH), and 20% had severe PH. During follow-up, acute exacerbation was noted in 38% of the cohort. The 5-year survival rate for all patients was 56%. Male sex, usual interstitial pneumonia pattern, and a reduced forced vital capacity were independent predictors of mortality in the pSS-ILD patients. CONCLUSIONS: A significant delay between the onset of symptoms and diagnosis was noted in our cohort. Importantly, our study highlights the importance of MSGB and emphasizes that clinicians should not rely solely on serological tests to diagnose pSS in ILD patients. The overall survival was poor, and more efforts are needed to diagnose pSS-ILD at an early stage and refer patients to experienced centers.


[FULL TEXT] [PDF]*
Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 Citation Manager
 Access Statistics
 Reader Comments
 Email Alert *
 Add to My List *
 * Requires registration (Free)
 

 Article Access Statistics
    Viewed648    
    Printed27    
    Emailed0    
    PDF Downloaded129    
    Comments [Add]    

Recommend this journal