Prevalence of primary Sjögren’s syndrome in patients undergoing evaluation for pulmonary arterial hypertension

Abstract

BackgroundThe prevalence of pulmonary arterial hypertension (PAH) in primary Sjögren’s syndrome (SS) had been reported to be rare. However, recent studies using echocardiography as a screening method showed conflicting results, and the true prevalence is still unclear. Since diagnosing primary SS is difficult because of its heterogeneous nature, a number of patients with primary-SS-associated PAH may be misdiagnosed with idiopathic PAH, losing their chance to undergo immunosuppressive therapy. Therefore, we sought to elucidate the prevalence of primary SS among patients who initially present with PAH.MethodsFrom our prospective institutional PAH database, 40 consecutive patients without any obvious cause of PAH at the time of PAH diagnosis were identified. We retrospectively evaluated the prevalence of primary SS diagnosed during or after the initial assessment of PAH.ResultsDuring the initial assessment, one patient was diagnosed with primary-SS-associated PAH. Among the 25 patients who were initially diagnosed with idiopathic PAH, five were diagnosed with primary SS during their course of the disease. Of the five patients, three had key signs suggesting primary SS and were probably underdiagnosed at the time of initial evaluation. The remaining two patients, who were finally diagnosed with primary SS, did not have any specific signs suggesting primary SS at the time of initial evaluation but showed positive conversion of their autoantibodies during the course of PAH.ConclusionThe prevalence of primary-SS-associated PAH may be relatively high among patients who undergo initial evaluation for PAH. Furthermore, primary-SS-associated PAH may be underdiagnosed with routine evaluation for the primary cause of PAH. Clinicians should pay specific attention and carefully evaluate the possibility of primary SS in patients with PAH.

Autoantibody clustering of lupus-associated pulmonary hypertension

“…Cluster 3 was defined by anti-Ro and anti-La, possibly representing an SLE phenotype with features of Sjogren’s, which can be associated with pulmonary hypertension 36–38. This is consistent with work by Hachulla et al 5 showing significantly higher incidences of anti-Ro and anti-La in patients with SLE with pulmonary hypertension compared with those without, suggesting these autoantibodies may be risk factors for pulmonary hypertension in SLE.…”

A case of pulmonary arterial hypertension complicated by anti-neutrophil cytoplasmic antibody-associated vasculitis and systemic sclerosis

“…The present case also had SS. However, it seemed that the PAH was not associated with SS in the present case, because PAH associated with SS often responds to GC [16].…”

“…Connective tissue disease-associated PAH (CTD-PAH) can be grouped into two types [15]: PAH complicated by SSc (SSc-PAH) [8,9] and PAH complicated by other diseases except SSc (non-SSc-PAH), such as SLE [9,10], SS, and mixed connective tissue disease. Although non-SSc-PAH usually responds to GC, SSc-PAH is refractory to GC [9,16]. In the present case, whether PAH was associated with AAV, SS, or SSc was considered important because their responses to GC could be different.…”

Endostatin and Vascular Endothelial Growth Factor-A₁₆₅b May Contribute to Classification of Pulmonary Hypertension

“…Furthermore, primary Sjogren's syndrome is known to be underdiagnosed with routine evaluation as a primary cause of PH. 21 Combined evaluation of PlGF for CTD-PAH and VEGF-A165b for LD-PH may help with a proper primary diagnosis. sVEGF-A165b was significantly higher in the LD-PAH (Group 3) group than in the other groups.…”