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

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From: Annals of Thoracic Medicine(Vol. 16, Issue 2)
Publisher: Medknow Publications and Media Pvt. Ltd.
Document Type: Article
Length: 5,034 words
Lexile Measure: 1650L

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Byline: Esam. Alhamad, Joseph. Cal, Nuha. Alrajhi, Muthurajan. Paramasivam, Waleed. Alharbi, Mohammed. AlEssa, Mohammed. Omair, Ammar. AlRikabi, Ahmad. AlBoukai

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.

Primary Sjogren's syndrome (pSS) is a systemic chronic inflammatory autoimmune disease characterized by lymphocytic infiltration of glandular and extraglandular organs.

The development of interstitial lung disease (ILD) in patients with connective tissue disease (CTD) is well established. It is estimated that approximately 20% of the pSS patients have an underlying ILD.[1] The commonly reported radiological patterns in pSS-ILD are nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), lymphocytic interstitial pneumonia, and organizing pneumonia.[2],[3],[4],[5],[6],[7],[8] While data on the survival rate in patients with pSS-ILD are limited, previous studies have shown that the 5-year survival rate ranges between 84% and 89.9%, depending on the studied population.[3],[9],[10],[11] Among the reported risk factors independently associated with survival in patients with pSS-ILD are the partial pressure of oxygen, microscopic honeycombing, the partial pressure of carbon dioxide, the extent of reticular abnormality based on high-resolution computed tomography (HRCT), the severity of fibroblastic foci, the percent predicted forced vital capacity (FVC), and the serum Krebs von den Lungen-6 level.[3],[9],[10]

In this context, we reviewed a series of consecutive pSS-ILD patients who were evaluated in one center to determine the clinical characteristics and prognostic factors associated with an increased risk of mortality.


The present study is a retrospective review of the ongoing ILD and pulmonary hypertension (PH) registry at the ILD and PH Centre at King Saud University Medical City. Consecutive ILD patients who were diagnosed with...

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Gale Document Number: GALE|A660154801