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

Sjögren’s 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.

Ýloprost ve Bosentan Kombinasyon Tedavisi Uygulanan Bir Sjögren Sendromu Ýliþkili Pulmoner Hipertansiyon Olgusu

Sjögren Sendromu (SS) ekzokrin glandlarýn lenfositik infiltrasyonu ile karakterize, romatoid artritten sonra ikinci en sýk görülen multisitem otoimmün hastalýktýr. Pulmoner tutulum %9-20 sýklýkta bildirilmiþtir ve genellikle hava yolu hastalýðý ya da interstisyel akciðer hastalýðý þeklinde görülür. Pulmoner Arteriyel Hipertansiyon (PAH) geliþimi SS’da çok nadir bir durumdur. SS iliþkili PAH’da hastalar genellikle fonksiyonel sýnýf WHO-III/IV’dür ve spesifik bir tedavi rejimi yoktur. PAH’ýn nonspesifik tedavisine ek olarak altta yatan hastalýðýn tedavisi önemlidir. Bu yazýdaki amacýmýz iloprost ve bosentan kombinasyon tedavisi altýnda izlediðimiz bir SS iliþkili PAH (SÝP) olgusunu takdim etmektir.