Sjogren’s Syndrome
Özet
Sjogren’s syndrome (SS) is an autoimmune connective tissue disease mainly affects exocrine glands and characterized by dry mouth and dry eyes. Apart from exocrine glands; musculoskeletal, skin, pulmonary, cardiovascular, hepatobiliary, renal, hematological and neurological system involvement may be seen. Cardiovascular system involvement is uncommon, mild asymtomatic pericardial effusion being the most common form of the cardiac involement. Pericarditis and myocarditis has been reported rarely. Pulmonary hypertension should be kept in mind in case of other accompanying connective tissue disease espacially systemic sclerosis. As the other rheumatic diseases, an increased risk of inflammation related atherosclerosis is reported in these patients, and monitoring and management of cardiovascular risk factors is reccommended.
The main antibodies detected in SS are anti-Ro/SSA and anti-La/SSB. Antibody mediated leymphocytic infiltration constitues the basic pathogenetic mechanism of the disease. Congenital heart block is the most serious form of cardiovascular involvement. Transplacentally transmitted antibodies target fetal cardiomyocites and cause abnormalities in the cardiac conduction system. Third- degree heart block is frequently detected, and heart failure is observed in 5-10% of these babies. Hydroxychloroquine use and weekly fetal echocardiography monitoring between 16 and 28 weeks are recommended for the prevention of fetal heart block in pregnancies of mothers with SS associated antibodies. Depending on the degree of the block and signs of heart failure, corticosteroids, intravenous immunglobulins, and plasma exchange may be considered.
Sjögren sendromu (SS) sıklıkla ekzokrin bezleri etkileyen, ağız ve göz kuruluğu ile karakterize otoimmun bir bağ doku hastalığıdır. Ekzokrin bezler dışında; kas-iskelet, cilt, pulmoner, kardiyovasküler, hepatobiliyer, renal, hematolojik ve nörolojik sistem tutulumları görülebilir. Kardiyovasküler sistem tutulumu sık değildir, hafif asemptomatik perikardiyal effüzyon en sık saptanan kardiak tutulum şeklidir. Perikardit ve myokardit nadir olarak bildirilmiştir. Eşlik eden diğer bağ doku hastalıkları, özellikle sistemik skleroz, olması durumunda pulmoner hipertansiyon akılda tutulmalıdır. Diğer romatizmal hastalıklarda olduğu gibi, bu hastalarda inflamasyon ilişkili artmış ateroskleroz riski bildirilmektedir ve kardiyovasküler risk faktörleri açısından takip ve tedavi önerilir.
SS’da saptanan ana antikorlar Anti-Ro/SSA ve anti-La/SSB’dir. Antikor aracılı lenfositik infiltrasyon hastalığın temel patogenetik mekanizmasını oluşturmaktadır. Konjenital kalp bloğu en ciddi kardivasküler tutulum şeklidir. Transplasental olarak geçen antikorlar fetal kardiyomiyositleri hedef alarak kardiyak iletim sisteminde anormalliklere neden olmaktadır. Sıklıkla üçüncü derece kalp bloğu saptanmakta, bu bebeklerin %5-10’unda kalp yetmezliği görülmektedir. SS ilişkili antikorlara sahip annelerin gebeliklerinde fetal kalp bloğunun önlenmesi için hidroksiklorokin kullanımı ve 16-28. haftalar arasında haftalık fetal ekokardiyografi takibi önerilir. Bloğun şiddetine ve kalp yetmezliği bulgularına göre kortikosteroidler, intravenöz immunglobulinler, plazma değişimi düşünülebilir.
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