Pemfigus hastalarının retrospektif analizi: Tek merkezli deneyim
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Date
2021
Authors
Bozdağ, Ali
Journal Title
Journal ISSN
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Publisher
Bursa Uludağ Üniversitesi
Abstract
Pemfigus, mukokutanöz büller ve erozyonlar ile karakterize kronik seyirli otoimmün büllöz bir hastalıktır. Pemfigus hastalarında demografik özellikler, alt tipi, tutulum yerleri, laboratuvar özellikleri gibi tanımlayıcı özellikler ve tedavi parametreleri arasındaki ilişki retrospektif olarak değerlendirildi ve tedavi sonuçları, prognoz, sağkalım gibi özellikleri aydınlatılmaya çalışıldı. İkiyüzbir hasta kadın/erkek oranı 1.26:1, tanı yaşı ortalaması 49.10±14.35, tanıya kadar geçen süre ortalama 6.16±0.58 ay idi. En sık 178 olgu (%88.6) ile Pemfigus vulgaris saptandı. Pemfigus foliaceus 18 hasta (%9) ile ikinci sıradaydı. Tanı anı anti desmoglein 1 ve 3 (dsg1, dsg3) değerleri, tedavinin 6. ve 12. ayında belirgin azalma gösterdi (sırasıyla ve tümünde p=0.001). Tanı anında anti-dsg 3 değeriyle, ilk tedavi sonrası hastalık aktivite kontrol süresi ve yatış süresi arasında pozitif korelasyon saptandı (sırasıyla p=0.004 ve p=0.012). Takibin 12. ayındaki anti-dsg 3 değeriyle relaps sayısı arasında pozitif korelasyon saptandı (p=0.022, r=0.261). Başlangıç tedavisinde kortikosteroid (KS)+azatiopürin 148 hastada (%79.6), sadece KS tedavisi 39 hastada (%19.4) ve KS+mikofenolat tedavileri 14 hastada (%7) uygulandı. Hastaların 135’inde (%67.2) en az bir yan etki görüldü. En sık görülen yan etkiler kandidiyazis 77 hasta (38.3), Cushingoid görünüm 47 hasta (%23.4), Diyabetes Mellitus 28 hasta (13.9) ve hipertansiyon 15 hasta (7.5) idi. İmmunsupresif tedaviye bağlı mortalite gelişen beş hastanın (%2.5) tamamında neden pnömoniydi. Bir yıllık sağkalım %95.2, beş yıllık %94 ve on yıllık sağkalım %79.3 iken, intravenöz immunoglobülin G kullanımının sağkalımı uzattığı saptandı. Bu çalışma Güney Marmara bölgesindeki geniş bir seride pemfigus hastalığının yukarıda bahsedilen tanımlayıcı özelliklerin sunulduğu bir çalışma olması nedeniyle önemlidir. Tanı ve takip laboratuvar sonuçları literatür ile uyumlu iken; tedavi sonuçları incelendiğinde immünsüpresif ve iii immünmodülatör tedavi kullanımı daha fazla tercih edilmiş olduğu; bu nedenle mortalitenin literatüre göre daha düşük saptandığı söylenebilir.
Pemphigus is a chronic autoimmune bullous disease characterized by mucocutaneous bullae and erosions. The relationship between the descriptive characteristics such as demographic characteristics, subtype, involvement sites, laboratory features and treatment parameters in pemphigus patients were evaluated retrospectively, and features such as treatment results, prognosis, and survival were tried to be elucidated. Two hundred and one patient female / male ratio was 1.26: 1, the mean age at diagnosis was 49.10 ± 14.35, and the mean time to diagnosis was 6.16 ± 0.58 months. Pemphigus vulgaris was detected most frequently with 178 cases (88.6%). Pemphigus foliaceus ranked second with 18 patients (9%). Anti-desmoglein 1 and 3 (dsg1, dsg3) values at the time of diagnosis decreased significantly at 6 and 12 months of treatment (p = 0.001, respectively, and all). A positive correlation was found between the anti-dsg 3 value at the time of diagnosis and the disease activity control period after the first treatment and the hospitalization period (p = 0.004 and p = 0.012, respectively). A positive correlation was found between the anti-dsg 3 value at the 12th month of follow-up and the number of relapses (p = 0.022, r = 0.261). In the initial treatment, corticosteroid (CS) + azathioprine was administered in 148 patients (79.6%), only CS treatment in 39 patients (19.4%), and CS + mycophenolate treatments in 14 patients (7%). At least one side effect was observed in 135 (67.2%) of the patients. The most common side effects were candidiasis 77 patients (38.3), Cushingoid appearance 47 patients (23.4%), Diabetes Mellitus 28 patients (13.9) and hypertension 15 patients (7.5). Pneumonia was the cause in all five patients (2.5%) who developed mortality due to immunosuppressive therapy. One-year survival was 95.2%, five-year 94% and ten-year survival 79.3%, while intravenous immunoglobulin G use was found to prolong survival. v This study is important because it is a study in which the above-mentioned descriptive features of pemphigus disease were presented in a large series in the South Marmara region. While the diagnosis and follow-up laboratory results are compatible with the literature; When the treatment results were examined, the use of immunosuppressive and immunomodulatory treatment was more preferred; Therefore, it can be said that mortality is lower than the literature.
Pemphigus is a chronic autoimmune bullous disease characterized by mucocutaneous bullae and erosions. The relationship between the descriptive characteristics such as demographic characteristics, subtype, involvement sites, laboratory features and treatment parameters in pemphigus patients were evaluated retrospectively, and features such as treatment results, prognosis, and survival were tried to be elucidated. Two hundred and one patient female / male ratio was 1.26: 1, the mean age at diagnosis was 49.10 ± 14.35, and the mean time to diagnosis was 6.16 ± 0.58 months. Pemphigus vulgaris was detected most frequently with 178 cases (88.6%). Pemphigus foliaceus ranked second with 18 patients (9%). Anti-desmoglein 1 and 3 (dsg1, dsg3) values at the time of diagnosis decreased significantly at 6 and 12 months of treatment (p = 0.001, respectively, and all). A positive correlation was found between the anti-dsg 3 value at the time of diagnosis and the disease activity control period after the first treatment and the hospitalization period (p = 0.004 and p = 0.012, respectively). A positive correlation was found between the anti-dsg 3 value at the 12th month of follow-up and the number of relapses (p = 0.022, r = 0.261). In the initial treatment, corticosteroid (CS) + azathioprine was administered in 148 patients (79.6%), only CS treatment in 39 patients (19.4%), and CS + mycophenolate treatments in 14 patients (7%). At least one side effect was observed in 135 (67.2%) of the patients. The most common side effects were candidiasis 77 patients (38.3), Cushingoid appearance 47 patients (23.4%), Diabetes Mellitus 28 patients (13.9) and hypertension 15 patients (7.5). Pneumonia was the cause in all five patients (2.5%) who developed mortality due to immunosuppressive therapy. One-year survival was 95.2%, five-year 94% and ten-year survival 79.3%, while intravenous immunoglobulin G use was found to prolong survival. v This study is important because it is a study in which the above-mentioned descriptive features of pemphigus disease were presented in a large series in the South Marmara region. While the diagnosis and follow-up laboratory results are compatible with the literature; When the treatment results were examined, the use of immunosuppressive and immunomodulatory treatment was more preferred; Therefore, it can be said that mortality is lower than the literature.
Description
Keywords
Pemfigus, Epidemiyoloji, Tedavi, Tedavi, Mortalite, Sağkalım, Pemphigus, Epidemiology, Treatment, Mortality, Survival
Citation
Bozdağ, A. (2021). Pemfigus hastalarının retrospektif analizi: Tek merkezli deneyim. Yayınlanmamış tıpta uzmanlık tezi. Bursa Uludağ Üniversitesi Tıp Fakültesi.