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.
Highlights
Pulmonary arterial hypertension (PAH) is one of the complications of connective tissue diseases (CTDs), such as systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease
We retrospectively evaluated the prevalence of primary s syndrome (SS) diagnosed during or after the initial assessment of pulmonary arterial hypertension (PAH)
Among the 25 patients who were initially diagnosed with idiopathic PAH, five were diagnosed with primary SS during their course of the disease
Summary
Pulmonary arterial hypertension (PAH) is one of the complications of connective tissue diseases (CTDs), such as systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease. PAH had been reported as a rare complication of primary SS [3], but recent studies using echocardiography as a screening method suggest that a substantial proportion of patients with primary SS may develop PAH [4,5], and the true prevalence of PAH among patients with primary SS is still undetermined. Because specific attention is often required for accurate primary SS diagnosis due to the existence of patients with primary SS without sicca symptoms [6], a proportion of patients with primary-SS-associated PAH may be misdiagnosed with idiopathic PAH, losing their chance to undergo immunosuppressive therapies. 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. We sought to elucidate the prevalence of primary SS among patients who initially present with PAH
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