Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
Department of Therapeutic Strategy for Heart Failure, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
PLoS One. 2018 May 15;13(5):e0197297. doi: 10.1371/journal.pone.0197297. eCollection 2018.
The 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.
From 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.
During 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.
The 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.
原发性干燥综合征（SS）并发肺动脉高压（PAH）的患病率曾被报道为罕见。然而，最近使用超声心动图作为筛查方法的研究结果存在矛盾，真实患病率仍不清楚。由于原发性 SS 的异质性，其诊断较为困难，因此许多原发性 SS 相关 PAH 患者可能被误诊为特发性 PAH，从而失去接受免疫抑制治疗的机会。因此，我们旨在阐明最初表现为 PAH 的患者中原发性 SS 的患病率。
我们从前瞻性机构性 PAH 数据库中确定了 40 例在 PAH 诊断时无明显 PAH 病因的连续患者。我们回顾性评估了在最初评估 PAH 期间或之后诊断出的原发性 SS 的患病率。
在最初的评估中，有 1 例患者被诊断为原发性 SS 相关的 PAH。在最初诊断为特发性 PAH 的 25 例患者中，有 5 例在疾病过程中被诊断为原发性 SS。这 5 例患者中，有 3 例有提示原发性 SS 的关键体征，可能在最初评估时被漏诊。另外 2 例最终被诊断为原发性 SS 的患者，在最初评估时没有任何提示原发性 SS 的特定体征，但在 PAH 过程中自身抗体呈阳性转化。
在接受 PAH 初始评估的患者中，原发性 SS 相关的 PAH 患病率可能相对较高。此外，在对 PAH 的原发性病因进行常规评估时，可能会漏诊原发性 SS 相关的 PAH。临床医生应特别注意并仔细评估 PAH 患者发生原发性 SS 的可能性。
