Relapsing Polychondritis
Özet
Relapsing polychondritis (RP) is a rare autoimmune disease characterized by recurrent inflammation of cartilage and proteoglycan-rich tissues, affecting the auricular, nasal, respiratory, and articular structures, as well as non-cartilaginous organs like the eyes, skin, heart, and central nervous system. Cardiovascular involvement, though less frequent, is the second leading cause of mortality after respiratory complications. Cardiovascular manifestations in RP range from valvular and vascular complications to arrhythmias, conduction defects, and pericardial or myocardial involvement, often with insidious onset. Early screening using echocardiography, CT/MR angiography, and PET/CT is crucial for timely diagnosis. Despite treatment challenges, corticosteroids remain the primary therapy, while immunosuppressive agents such as methotrexate, azathioprine, infliximab, cyclophosphamide, and tocilizumab serve as second-line options. This chapter highlights the epidemiology, pathogenesis, clinical presentation of RP, with a focus on its cardiovascular complications, and summarizing the treatment approaches.
Tekrarlayan polikondrit (RP), kıkırdak ve proteoglikan açısından zengin dokuların tekrarlayan iltihabı ile karakterize nadir bir otoimmün hastalıktır ve kulak, burun, solunum ve eklem yapılarının yanı sıra gözler, cilt, kalp ve merkezi sinir sistemi gibi kıkırdak dışı organları da etkiler. Kardiyovasküler tutulum, daha az sıklıkta görülse de, solunum komplikasyonlarından sonra ikinci sırada yer alan ölüm nedenidir. RP'deki kardiyovasküler belirtiler kapak ve damar komplikasyonlarından aritmilere, iletim kusurlarına ve perikardiyal veya miyokardiyal tutuluma kadar uzanır ve genellikle sinsi başlangıçlıdır. Ekokardiyografi, BT/MR anjiyografi ve PET/BT kullanılarak erken tarama, zamanında tanı için çok önemlidir. Tedavi zorluklarına rağmen, kortikosteroidler birincil tedavi olmaya devam eder. Metotreksat, azatioprin, infliksimab, siklofosfamid ve tocilizumab gibi immünosüpresif ajanlar ikinci basamakta yer alan tedavi seçenekleridir. Bu bölümde RP'nin epidemiyolojisi, patogenezi, klinik görünümü vurgulanmakta, özellikle kardiyovasküler komplikasyonlara odaklanılmakta ve tedavi yaklaşımları özetlenmektedir.
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