The Association of Systolic Pulmonary Artery Pressure with Pulmonary Artery Diameter, Age, Disease Duration, Stage of the Disease, and Extrapulmonary Involvement in Patients with Sarcoidosis

Year 2021, Volume: 6 Issue: 1, 22 - 30, 05.03.2021

Celalettin Korkmaz , Soner Demirbaş Hülya Vatansev

https://doi.org/10.26453/otjhs.845321

Abstract

Objective: We aimed to investigate the association of systolic pulmonary artery pressure (sPAP), with pulmonary artery diameter (PAD), age, gender, disease duration, disease stages, extrapulmonary involvement, and spontaneous remission in patients with sarcoidosis.
Materials and Methods: Diagnosed and followed-up in the chest diseases clinic between 2010-2020, 60 sarcoidosis patients were included. Patients’ demographic data, clinical course, information over treatment, radiological and echocardiographic examinations were recorded. sPAB was utilized to calculate mean PAP based on ECHO. PAD was measured by an experienced radiologist in thoracic CT. Associatons of PAP and PAD with other parameters were investigated.
Results: sPAP and PAD were found as 29.68±5.73 mmHg and 28.20±5.75 mm. A significant correlation was detected between them (r=0.46 p<0.001). PADs were 39.7±4.6 and 27±4.9 in patients with and without PH (p<0.001). sPAPs were found as 27.52±3.77 and 31.03±6.35 among those with and without extrapulmonary involvement (p=0.01). No association was found between these parameters and others.
Conclusion: A moderately strong positive correlation was detected between sPAP and PAD in sarcoidosis patients. PAD was significantly found higher in patients with PH than those without. This also shows that PAD, which can be easily measured during thoracic CT examination in sarcoidosis patients, can be a guide in predicting sarcoidosis-associated pulmonary hypertension (SAPH).

Keywords

Pulmonary artery diameter, pulmonary hypertension, sarcoidosis, systolic pulmonary artery pressure

Thanks

Authors thank Cengiz Kadıyoran for the measurements of pulmonary artery diameter and Numan Duran for language editing.

Sarkoidozlu Hastalarda Sistolik Pulmoner Arter Basıncının Pulmoner Arter Çapı, Yaş, Hastalık Süresi, Hastalığın Evresi ve Akciğer Dışı Tutulumla İlişkisi

Abstract

Amaç: Sarkoidozlu hastalarda sistolik pulmoner arter basıncının (sPAB) pulmoner arter çapı (PAÇ), yaş, cinsiyet, hastalık süresi, hastalığın evresi, akciğer dışı tutulum ve spontan remisyon ile olan ilişkilerini araştırmayı amaçladık.
Materyal ve Metot: Çalışmaya göğüs hastalıkları kliniğinde 2010-2020 yılları arasında tanı almış ve takip edilmiş 60 sarkoidozlu hasta alındı. Hastaların demografik verileri, klinik seyri, tedavi bilgileri radyolojik ve ekokardiyografik incelemeleri kaydedildi. EKO’ya dayalı ortalama PAB’nın hesaplanmasında sPAB kullanıldı. PAÇ toraks BT konusunda yetkin radyolog tarafından ölçüldü. PAB ile PAÇ ve diğer parametreler arasındaki ilişki araştırıldı.
Bulgular: Hastaların sPAB: 29.68±5.73 mmHg, PAÇ:28.20±5.75 mm olarak bulundu. Aralarında anlamlı korelasyon saptandı (r=0.46 p<0.001). PAÇ, PH’u olan hastalarda: 39.7±4.6, olmayanlarda: 27±4.9 (p<0.001) idi. sPAB, ekstrapulmoner tutulumu olmayanlarda: 31.03±6.35, olanlarda: 27.52±3.77 (p=0.01) bulundu. Araştırılan diğer parametrelerle anlamlı ilişki saptanmadı.
Sonuç: Sarkoidozlu hastalarda sPAB ve PAÇ arasında orta derecede kuvvetli pozitif yönlü korelasyon saptandı. Aynı zamanda PAÇ, PH olan hastalarda olmayanlara göre anlamlı olarak daha yüksek bulundu. Bu da sarkoidozlu hastalarda toraks BT incelemesi esnasında kolaylıkla ölçülebilen PAÇ’nın sarkoidozla ilişkili pulmoner hipertansiyonu (SİPH) tahmin etmede yol gösterici olabileceğini göstermektedir.

Keywords

Pulmoner hipertansiyon, pulmoner arter çapı, sarkoidoz, sistolik pulmoner arter basıncı

References

• Grunewald J, Grutters JC, Arkema EV, Saketkoo LA, Moller DR, Müller-Quernheim J. Sarcoidosis. Nat Rev Dis Primers. 2019;5(1):45.
• Galie N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) (vol 46, pg 903, 2015. Eur Respır J. 2015;46(6):1855-1856. doi: 10.1183/13993003.51032-2015
• Boucly A, Cottin V, Nunes H, et al. Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension. Eur Respir J. 2017;50(4):1-12.
• Shlobin OA, Baughman RP. Sarcoidosis-Associated Pulmonary Hypertension. Semin Respir Crit Care Med. 2017;38(4):450-462.
• Baughman RP, Engel PJ, Meyer CA, Barrett AB, Lower EE. Pulmonary hypertension in sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2006;23(2):108-116.
• Baughman RP. Pulmonary hypertension associated with sarcoidosis. Arthritis Res Ther. 2007;9 Suppl 2(Suppl 2):S8.
• Shlobin OA, Kouranos V, Barnett SD, et al. Physiological predictors of survival in patients with sarcoidosis-associated pulmonary hypertension: results from an international registry. Eur Respir J. 2020;55(5):1-28.
• Walsh SL, Wells AU, Sverzellati N, et al. An integrated clinicoradiological staging system for pulmonary sarcoidosis: a case-cohort study. Lancet Respir Med. 2014;2(2):123-130.
• Kirkil G, Lower EE, Baughman RP. Predictors of Mortality in Pulmonary Sarcoidosis. Chest. 2018;153(1):105-113.
• Judson MA, Costabel U, Drent M, et al. The WASOG Sarcoidosis Organ Assessment Instrument: An update of a previous clinical tool. Sarcoidosis Vasc Diffuse Lung Dis. 2014;31(1):19-27.
• Greco FG, Spagnolo P, Muri M, et al. The value of chest radiograph and computed tomography in pulmonary sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2014;31(2):108-116.
• Runo JR, Vnencak-Jones CL, Prince M, et al. Pulmonary veno-occlusive disease caused by an inherited mutation in bone morphogenetic protein receptor II. Am J Respir Crit Care Med. 2003;167(6):889-894.
• Mahammedi A, Oshmyansky A, Hassoun PM, Thiemann DR, Siegelman SS. Pulmonary artery measurements in pulmonary hypertension: the role of computed tomography. J Thorac Imaging. 2013;28(2):96-103.
• Huitema MP, Spee M, Vorselaars VM, et al. Pulmonary artery diameter to predict pulmonary hypertension in pulmonary sarcoidosis. European Respiratory Journal. 2016;47(2):673-676.
• Zimmerman I, Mann N. Boeck's sarcoid: a case of sarcoidosis complicated by pulmonary emphysema and cor pulmonale. Annals of internal medicine. 1949;31(1):153-162.
• Handa T, Nagai S, Miki S, et al. Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest. 2006;129(5):1246-1252.
• Bourbonnais JM, Samavati L. Clinical predictors of pulmonary hypertension in sarcoidosis. Eur Respir J. 2008;32(2):296-302.
• Alhamad EH, Idrees MM, Alanezi MO, Alboukai AA, Shaik SA. Sarcoidosis-associated pulmonary hypertension: Clinical features and outcomes in Arab patients. Ann Thorac Med. 2010;5(2):86-91.
• Huitema MP, Bakker ALM, Mager JJ, et al. Prevalence of pulmonary hypertension in pulmonary sarcoidosis: the first large European prospective study. Eur Respir J. 2019;54(4):1-4.
• Sulica R, Teirstein AS, Kakarla S, Nemani N, Behnegar A, Padilla ML. Distinctive clinical, radiographic, and functional characteristics of patients with sarcoidosis-related pulmonary hypertension. Chest. 2005;128(3):1483-1489.
• Rodman DM, Lindenfeld J. Successful treatment of sarcoidosis-associated pulmonary hypertension with corticosteroids. Chest. 1990;97(2):500-502.