Tobal Rachid, Potjewijd Judith, van Doorn Daan, van Empel Vanessa, Damoiseaux Jan, van Paassen Pieter
Department of Internal Medicine, Division of Nephrology and Clinical and Experimental Immunology, Maastricht University Medical Center, 6229 HX Maastricht, The Netherlands.
Department of Cardiology, Maastricht University Medical Center, 6229 HX Maastricht, The Netherlands.
Diagnostics (Basel). 2024 Jul 9;14(14):1471. doi: 10.3390/diagnostics14141471.
Pulmonary hypertension (PH) is a serious condition linked to immune-system dysfunction. Myositis-specific/associated antibodies (MSAs/MAAs) play a role in idiopathic inflammatory myopathy (IIM) and interstitial lung disease (ILD), but their significance in PH remains unclear. We believe the presence of these antibodies may be underestimated. This study analyzed adult PH patients without pre-existing IIM for MSA/MAA prevalence using a line-blot assay. We compared PH patients with and without ILD signs to a cohort clinically suspected of IIM/ILD ( = 558). Our PH cohort ( = 121) showed a significantly higher prevalence of overall weak positive MSAs/MAAs and positive overlap syndrome-associated MAAs than the suspected IIM/ILD group ( < 0.001). Notably, MSAs/MAAs were found in PH patients both with and without ILD, though more prevalent in those with ILD. Anti-synthetase and anti-overlap syndrome antibodies were the most common. Our study is the first to systematically show a high MSA/MAA prevalence in PH without IIM presentation. This highlights the need to consider PH when diagnosing MSA/MAA-associated conditions. We recommend MSA/MAA screening for newly diagnosed PH, especially in those with ILD, for early detection and potential immunomodulatory treatment. Further research should explore the link between MSAs/MAAs and PH, and the value of monitoring patients with weak MSA/MAA positivity over time.
肺动脉高压（PH）是一种与免疫系统功能障碍相关的严重疾病。肌炎特异性/相关抗体（MSAs/MAAs）在特发性炎性肌病（IIM）和间质性肺疾病（ILD）中起作用，但其在PH中的意义仍不清楚。我们认为这些抗体的存在可能被低估了。本研究使用线性免疫印迹法分析了无既往IIM的成年PH患者中MSA/MAA的患病率。我们将有和无ILD体征的PH患者与临床怀疑患有IIM/ILD的队列（n = 558）进行了比较。我们的PH队列（n = 121）显示，总体弱阳性MSAs/MAAs和阳性重叠综合征相关MAAs的患病率显著高于疑似IIM/ILD组（P < 0.001）。值得注意的是，在有和无ILD的PH患者中均发现了MSAs/MAAs，尽管在有ILD的患者中更为普遍。抗合成酶抗体和抗重叠综合征抗体最为常见。我们的研究首次系统地显示了无IIM表现的PH患者中MSA/MAA的高患病率。这突出了在诊断与MSA/MAA相关疾病时考虑PH的必要性。我们建议对新诊断的PH患者进行MSA/MAA筛查，尤其是对那些有ILD的患者，以便早期发现并进行潜在的免疫调节治疗。进一步的研究应探索MSAs/MAAs与PH之间的联系，以及长期监测MSA/MAA弱阳性患者的价值。
