Primary Sjögren's syndrome 1976–2005 and associated interstitial lung disease: a population-based study of incidence and mortality

Abstract

ObjectiveA very few studies describe the epidemiology of primary Sjögren's syndrome (pSS). The reported frequency of pulmonary involvement in pSS varies widely depending on the detection method employed, and consists mainly of various forms of airways disease. We aimed to evaluate the incidence and mortality of pSS and of lung disease in pSS, focusing on interstitial lung disease (ILD).MethodsA population-based incidence cohort of patients diagnosed with pSS in 1976–2005 was assembled. Diagnosis was based on the 2002 American-European Consensus Group criteria for pSS. Cumulative incidence adjusted for the competing risk of death was estimated. A Cox model with a time-dependent covariate was used to determine the incidence and the standardised mortality HR of pSS.Results85 patients with pSS were identified (mean age 59.9 years; 91% women). The annual incidence of pSS was 4.2, 95% CI (3.3 to 5.1)/100 000 population and it increased with higher age at pSS diagnosis (18–44 years: 2.1/100 000 vs ≥75 years: 12.3/100 000). Standardised mortality ratio in pSS compared with the general population was 0.92, 95% CI (0.57 to 1.41). A total of 105 patients with pSS and ILD were identified (mean age 58.1 years; 91% women). Among patients with pSS without prior ILD, the cumulative incidence of ILD in patients with pSS was 10% (±3%) at 1 year after diagnosis of pSS and increased to 20% (±4%) by 5 years after pSS. The development of lung disease in pSS was associated with poor survival (HR 2.16; 95% CI 0.99 to 4.74).ConclusionspSS incidence seems to be almost the same as was reported in a previous study conducted among Olmsted County Minnesota population. Survival among patients with pSS and general population does not differ substantially. However, patients with pSS who have ILD likely have increased premature mortality.

Risk factors for primary Sjögren syndrome-associated interstitial lung disease

“…pSS is a chronic autoimmune disease, and genetic predisposition may play a role in its pathogenesis (13). A population-based study showed that the annual incidence of pSS was 5.1/100,000 population, and increased with age at pSS diagnosis (18-44 years: 1.8/100,000 vs. ≥75 years: 10.7/100,000) (14). Female patients were more affected (8.7/100,000) than male patients (1.1/100,000) (14).…”

Chest HRCT findings in patients with primary Sjögren’s syndrome

“…The development of lung disease in pSS was associated with poor survival. 19 Thus, repeated pulmonary function tests and diagnostic radiology are necessary and should be the preferred methods to monitor pSS-specific organ involvement.…”

Pulmonary manifestations of Sjögren's syndrome

“…Computed tomography (CT) scan abnormalities are found in 34-50% of patients [38]. The annual incidence of respiratory manifestations is estimated at 10% (±3%) 1 year after diagnosis of Sjögren's syndrome and increases to 20% (±4%) by 5 years [6].…”

“…Clubbing is rare [36]. ILD is associated with premature mortality [6]. The presence of anti-SSA is a predisposing factor of ILD [90].…”

“…The presence of ILD is associated with impaired respiratory function. Importantly, pulmonary involvement leads to increased risk of mortality [6]. Herein, we summarise the literature about Sjögren's syndrome pulmonary manifestations.…”