Burcu YAĞIZ, Belkıs Nihan COŞKUN, Seda ÇELİK, Yavuz PEHLİVAN, Hüseyin Ediz DALKILIÇ

Nadir Bir Hastalık Olan Tekrarlayan Polikondrit İle Takip Ettiğimiz Hastalarımızın Klinik Özellikleri: Tek Merkez Deneyimi

Tekrarlayan polikondrit (TP), özellikle kulaklar, burun, gözler, eklemler ve solunum yolu olmak üzere vücuttaki kıkırdaklı yapıları ve diğer dokuları etkileyen, immun aracılı, sistemik inflamatuar, dejeneratif bir hastalıktır. TP'nin hedef dokuları sadece kıkırdak içeren yapılar olmayıp, deri, böbrek, kalp ve merkezi sinir sistemi gibi kıkırdak içermeyen yapılar da etkilenir. Nadir bir hastalık olması nedenli epidemiyolojisi konusunda veriler yetersizdir. Sıklıkla 40-60 yaşları arasında görülür. Erkekler ve kadınlar eşit olarak etkilenir. Klinik özellikleri hastalar arasında değişkenlik göstermektedir. Hastalığın nadir görülmesi ve geniş klinik yelpazesi sıklıkla yanlış tanıya veya tanıda gecikmeye yol açar. TP'nin erken teşhisi ve hızlı tedavisi, ilişkili komplikasyonları ve ölümü önlemek, prognozu iyileştirmek için kritik öneme sahiptir. Tedavi seçenekleri arasında glukokortikoidler, dapson, hastalık modifiye edici antiromatizmal ilaçlar (DMARDs) ve biyolojik ajanlar yer alır. Prognoz, organ hasarının ciddiyetine bağlı olarak klinik tablo kadar heterojendir. TP tanısı ile izlediğimiz 10 hastanın ortalama hastalık başlama yaşı 49,5±4,1 idi. Semptom başlangıcı ile tanı arası süre ortanca 3 aydı (2-60). Hastaların %80’i erkekti. En sık rastlanan klinik bulgu aurikuler kondritti (%100). Hastaların tümü tedavileri sırasında en az bir kez oral prednizolon aldı. İki hastaya intravenöz metilprednizolon uygulandı. Bir hastada DMARDs yanıtsız olması nedenli infliksimaba geçildi. Bir hasta pnömosepsis nedenli kaybedildi. Bu yazıda, TP'nin patogenezi, klinik seyri, teşhisi ve tedavisi ile ilgili mevcut bilgilere genel bir bakış sunarak nadir görülen ancak pek çok sistemi etkileyebilen bu hastalıkla ilgili hekimler arasındaki farkındalığı artırmayı amaçladık.

Anahtar Kelimeler:

İnflamasyon, Kıkırdak, Nadir, Relapsing polikondrit, Sistemik

Clinical Characteristics of Our Follow-Up Patients with the Rare Disease Relapsing Polychondritis: A Single-Center Study

Relapsing polychondritis (RP) is an immune-mediated, systemic inflammatory and degenerative disease that affects cartilaginous structures particularly the ears, nose, eyes, joints, and respiratory tract and other tissues in the body. RP targets non-cartilaginous structures such as skin, kidney, heart, and the central nervous system in addition to cartilage-containing structures. Since it is a rare disease, data on its epidemiology are insufficient. It is prevalent between the ages of 40 and 60. Men and women are affected equally. Clinical characteristics vary among patients. The disease's rarity and broad clinical spectrum frequently result in misdiagnosis or delayed diagnosis. To prevent related complications and death, and to improve prognosis, early diagnosis and timely treatment of RP are crucial. Glucocorticoids, dapsone, disease-modifying antirheumatic drugs (DMARDs), and biologics are available as treatment options. The prognosis, such as the clinical presentation, varies depending on the extent of organ damage. 10 patients with RP had a mean onset age of 49.5±4.1. The median time between symptom onset and diagnosis was 3 months (2-60). 80% of the patients were male. The most frequent clinical manifestation is auricular chondritis (100%). During treatment, oral prednisolone was administered to all patients at least once. Intravenous methylprednisolone was administered to two patients. Due to ineffectiveness of DMARDs, one patient was switched to infliximab. A patient died due to pneumosepsis. This article aims to increase clinicians' awareness of this rare disease that can affect multiple systems by providing an overview of its pathogenesis, clinical course, diagnosis, and treatment.

Keywords:

Inflammation, cartilage, rare, relapsing polychondritis, systemic,

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