循環器ジャーナル71巻4号

2023年10月発行

特集 変革期を迎えた肺高血圧症—次世代の病態理解，診断，治療とは？ Ⅱ.各論：各種肺高血圧症の診断と治療

膠原病に合併する肺高血圧症の鑑別と治療

著者： 加藤将¹ 久田諒¹

所属機関： ¹北海道大学大学院医学院・医学研究院免疫・代謝内科学教室

ページ範囲：P.530 - P.537

POINT
●膠原病には，疾患に直接関連して肺動脈性肺高血圧症/第1群肺高血圧症が合併する．
●加えて，全身性強皮症には左心性疾患（第2群肺高血圧症），間質性肺疾患（第3群肺高血圧症）がしばしば合併する．
●全身性強皮症の肺血管障害は，肺動脈のみならず肺毛細血管，肺静脈と広範囲に及ぶことが多い．

参考文献

1）Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022 ; 43 : 3618-731.

2）Kato M, Sugimoto A, Atsumi T. Diagnostic and prognostic markers and treatment of connective tissue disease-associated pulmonary arterial hypertension : current recommendations and recent advances. Expert Rev Clin Immunol 2020 ; 16 : 993-1004.

3）Sasaki N, Kamataki A, Sawai T. A histopathological study of pulmonary hypertension in connective tissue disease. Allergol Int 2011 ; 60 : 411-7.

4）Overbeek MJ, Vonk MC, Boonstra A, et al. Pulmonary arterial hypertension in limited cutaneous systemic sclerosis : a distinctive vasculopathy. Eur Respir J 2009 ; 34 : 371-9.

5）Good RB, Gilbane AJ, Trinder SL, et al. Endothelial to Mesenchymal Transition Contributes to Endothelial Dysfunction in Pulmonary Arterial Hypertension. Am J Pathol 2015 ; 185 : 1850-8.

6）Jais X, Launay D, Yaici A, et al. Immunosuppressive therapy in lupus- and mixed connective tissue disease-associated pulmonary arterial hypertension : a retrospective analysis of twenty-three cases. Arthritis Rheum 2008 ; 58 : 521-31.

7）Humbert M, Yaici A, de Groote P, et al. Screening for pulmonary arterial hypertension in patients with systemic sclerosis : clinical characteristics at diagnosis and long-term survival. Arthritis Rheum 2011 ; 63 : 3522-30.

8）Coghlan JG, Denton CP, Grunig E, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis : the DETECT study. Ann Rheum Dis 2014 ; 73 : 1340-9.

9）Todoroki Y, Kubo S, Nakano K, et al. Nailfold microvascular abnormalities are associated with a higher prevalence of pulmonary arterial hypertension in patients with MCTD. Rheumatology（Oxford）2022 ; 61 : 4875-84．

10）Ninagawa K, Kato M, Nakamura H, et al. Reduced diffusing capacity for carbon monoxide predicts borderline pulmonary arterial pressure in patients with systemic sclerosis. Rheumatol Int 2019 ; 39 : 1883-7.

11）Ghigna MR, Guignabert C, Montani D, et al. BMPR2 mutation status influences bronchial vascular changes in pulmonary arterial hypertension. Eur Respir J 2016 ; 48 : 1668-81.

12）Nossent EJ, Antigny F, Montani D, et al. Pulmonary vascular remodeling patterns and expression of general control nonderepressible 2（GCN2）in pulmonary veno-occlusive disease. J Heart Lung Transplant 2018 ; 37 : 647-55.

13）Montani D, Lau EM, Dorfmuller P, et al. Pulmonary veno-occlusive disease. Eur Respir J 2016 ; 47 : 1518-34.

14）Lammi MR, Saketkoo LA, Gordon JK, et al. Changes in hemodynamic classification over time are common in systemic sclerosis-associated pulmonary hypertension : insights from the PHAROS cohort. Pulm Circ 2018 ; 8 : 2045893218757404.

15）Ninagawa K, Kato M, Ohira H, et al. The assessment of left heart disease in patients with systemic sclerosis and pulmonary hypertension. Clin Exp Rheumatol 2021 ; 39 Suppl 131 : 103-10.

16）Kuwana M, Blair C, Takahashi T, et al. Initial combination therapy of ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension（CTD-PAH）in the modified intention-to-treat population of the AMBITION study : post hoc analysis. Ann Rheum Dis 2020 ; 79 : 626-34.

17）Ninagawa K, Kato M, Kikuchi Y, et al. Predicting the response to pulmonary vasodilator therapy in systemic sclerosis with pulmonary hypertension by using quantitative chest CT. Mod Rheumatol 2023 ; 33 : 758-67.

18）Waxman A, Restrepo-Jaramillo R, Thenappan T, et al. Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease. N Engl J Med 2021 ; 384 : 325-34.

19）Volkmann ER, Andreasson K, Smith V. Systemic sclerosis. Lancet 2023 ; 401 : 304-18.

20）Li M, Tian Z, Qian J, et al. Impact of pregnancy in patients with systemic lupus erythematosus-associated pulmonary arterial hypertension : case series and literature review. Lupus Sci Med 2022 ; 9 : e000636.