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loprost ve Bosentan Kombinasyon Tedavisi Uygulanan Bir Sjgren Sendromu likili Pulmoner Hipertansiyon Olgusu [Respir Case Rep]
Respir Case Rep. 2018; 7(2): 59-62 | DOI: 10.5505/respircase.2018.78557

loprost ve Bosentan Kombinasyon Tedavisi Uygulanan Bir Sjgren Sendromu likili Pulmoner Hipertansiyon Olgusu

Aye Baha1, Berkay Ekici2, Nalan Ogan1, Evrim Eylem Akpnar1
1Ufuk niversitesi Tp Fakltesi Gs Hastalklar Anabilim Dal, Ankara
2Ufuk niversitesi Tp Fakltesi Kardiyoloji Anabilim Dal, Ankara

Sjgren Sendromu (SS) ekzokrin glandlarn lenfositik infiltrasyonu ile karakterize, romatoid artritten sonra ikinci en sk grlen multisitem otoimmn hastalktr. Pulmoner tutulum %9-20 sklkta bildirilmitir ve genellikle hava yolu hastal ya da interstisyel akcier hastal eklinde grlr. Pulmoner Arteriyel Hipertansiyon (PAH) geliimi SSda ok nadir bir durumdur. SS ilikili PAHda hastalar genellikle fonksiyonel snf WHO-III/IVdr ve spesifik bir tedavi rejimi yoktur. PAHn nonspesifik tedavisine ek olarak altta yatan hastaln tedavisi nemlidir. Bu yazdaki amacmz iloprost ve bosentan kombinasyon tedavisi altnda izlediimiz bir SS ilikili PAH (SP) olgusunu takdim etmektir.

Anahtar Kelimeler: Sjgren Sendromu, Pulmoner Hipertansiyon, Kombinasyon Tedavisi, loprost, Bosentan

A Case of Sjgrens Syndrome-Related Pulmonary Arterial Hypertension Treated with Iloprost and Bosentan Combination Therapy

Aye Baha1, Berkay Ekici2, Nalan Ogan1, Evrim Eylem Akpnar1
1Ufuk University Faculty of Medicine, Department of Pulmonary Medicine, Ankara, Turkey
2Ufuk University Faculty of Medicine, Department of Cardiology, Ankara, Turkey

Sjgrens syndrome (SS), which is characterized by lymphocytic infiltration of the exocrine glands, is the second most common multisystem autoimmune disease after rheumatoid arthritis. Pulmonary involvement ranges from 9% to 20% and it usually occurs as an airway disease or interstitial lung disease. Pulmonary arterial hypertension (PAH) is a very rare condition in SS. In SS-associated PAH, the functional class of patients is usually World Health Organization (WHO) Class III/IV and there is no specific treatment regimen. In addition to nonspecific medical treatment for PAH, treatment of the underlying disease and supportive therapy are important. The aim of this manuscript was to present a case of SS-related PAH (SRP) followed under bosentan and iloprost combination therapy.

Keywords: Sjgren Syndrome, Pulmonary Hypertension, Combination Therapy, Iloprost, Bosentan

Aye Baha, Berkay Ekici, Nalan Ogan, Evrim Eylem Akpnar. A Case of Sjgrens Syndrome-Related Pulmonary Arterial Hypertension Treated with Iloprost and Bosentan Combination Therapy. Respir Case Rep. 2018; 7(2): 59-62

Sorumlu Yazar: Aye Baha, Trkiye
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