Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria
Yıl: 2011 Cilt: 9 Sayı: 1 Sayfa Aralığı: 56 - 62 Metin Dili: Türkçe İndeks Tarihi: 29-07-2022
Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria
Öz: Schnitzler sendromu, nadir bir ürtiker nedenidir. Monoklonal gammopati, kronik ürtiker veya ürtikeryal vaskülit birlikteliği ile ateş, artralji veya artrit, kemik ağrısı, hepato- ve/veya splenomegali, palpabl lenf nodları, artmış eritrosit sedimentasyon hızı ve lökositoz kriterlerinden en az ikisinin varlığı olarak tanımlanmaktadır. Sendromun patofizyolojisi tam olarak anlaşılamamıştır. Genellikle olgularda kortikosteroide direnç görülmekle beraber, bir IL-1 reseptör antagonisti olan anakinraya birçok olgu olumlu yanıt vermiştir. Sendromun ortalama başlangıç yaşı 51 olarak bildirilmektedir. Ancak nadiren yaşlı hastalar literatürde bulunmaktadır. Bu bildiride ateş, artralji, kemik ağrısı ve multipl lenfadenopatileri olan, artmış eritrosit sedimentasyon hızı, ürtikeryal vaskülit bulguları ve IgG kappa tipi önemi bilinmeyen monoklonal gammopati saptanması üzerine Schnitzler sendromu tanısı konulan 79 yaşında bir kadın hasta sunulmaktadır. Kapiller sistem ile bakılan serum protein elektroforezinde γ- bölgesi bozulmuş olarak saptanmıştır. Bu bulgu, aşikar bir monoklonal pik görülmemesine rağmen, IgG kappa tipi monoklonal immünglobulin olarak değerlendirilmiştir. Monoklonal proteinlerin belirlenmesinde altın standart metot kabul edilen immünfiksasyon yönteminde ise IgG kappa tipi bant saptanamamıştır. Bakılan kappa ve lambda serbest hafif zincir konsantrasyonları referans değerler içinde ölçülmüştür. Ancak kappa/lambda oranı artmış olarak bulunmuştur. Hastanın tedavisinde başlangıçta kortikosteroide olumlu yanıt alınmış, ancak steroid dozu azaltılırken alevlenme görülmesi üzerine tedaviye immünsüpresif ajan olan azatiopirin eklenmiştir. Klinik düzelme görüldükten sonra metilprednizolon azaltılarak kesilmiş, azatiopirin tedavisine devam edilmiştir. Üç aylık tedavi sonunda hasta klinik remisyonda kabul edilmiştir. Hastanın yaklaşık bir yıllık takibinde düşük doz azatiopirin ile iyilik halinin devam ettiği gözlenmiştir. (Asthma Allergy Immunol 2011;9:55-61)
Anahtar Kelime: Konular:
Schnitzler sendromu: Tedavisi güç nadir bir kronik ürtiker
Öz: Schnitzler syndrome is a rare cause of urticaria and is defined by monoclonal gammopathy and chronic urticaria or urticarial vasculitis combined by at least two of the following features: fever, arthralgia or arthritis, bone pain, hepato-and/or splenomegaly, palpable lymph nodes, elevated erythrocyte sedimentation rate, and leukocytosis. Although the pathophysiology is not completely evaluated, it usually presents with resistance to corticosteroids and many patients respond to anakinra, an IL-1 receptor antagonist. The mean age of onset is 51, but very few cases have been documented in elderly patients. Here we describe a 79-year-old woman diagnosed as Schnitzler syndrome who fulfilled the diagnostic criteria of the syndrome with multiple mediastinal lymph nodes, fever and arthralgia, bone pain, elevated erythrocyte sedimentation rate, urticarial vasculitis and a IgG kappa monoclonal gammopathy of undetermined significance. On capillary serum protein electrophoresis of the patient with the Capillarys System, the morphology of the γ-region was disturbed. Although there wasn’t any obvious monoclonal peak on Capillarys (immunotyping), the result was diagnosed as an IgG kappa type monoclonal immunoglobulin. For the confirmation of our finding we performed immunofixation electrophoresis which is accepted as the gold standard method for the characterization of monoclonal proteins, although it failed to detect an IgG kappa type band. Both kappa and lambda free light chain concentrations were within the reference range, however the kappa/lambda ratio was abnormal. The patient initially responded well to corticosteroids, but later needed to be treated with azathioprine as a corticosteroid sparing drug. After total clinical improvement methylprednisolone was discontinued with tapering and azathioprine was kept on. At the end of 3 months of therapy, she was accepted to be in clinical remission. During her follow up she has remained in remission approximately for a year with the low dose of azothiopurine. (Asthma Allergy Immunol 2011;9:55-61)
Anahtar Kelime: Konular:
Belge Türü: Makale Makale Türü: Olgu Sunumu Erişim Türü: Erişime Açık
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| APA | GELİNCİK A, DEMİRTÜRK M, Buyukozturk S, TELCİ A, BÜYÜKBABANI N, ÇOLA KOĞLU B, DAL M (2011). Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria. , 56 - 62. |
| Chicago | GELİNCİK Aslı,DEMİRTÜRK Mustafa,Buyukozturk Suna,TELCİ Ayşegül,BÜYÜKBABANI NESIMI,ÇOLA KOĞLU Bahauddin,DAL Murat Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria. (2011): 56 - 62. |
| MLA | GELİNCİK Aslı,DEMİRTÜRK Mustafa,Buyukozturk Suna,TELCİ Ayşegül,BÜYÜKBABANI NESIMI,ÇOLA KOĞLU Bahauddin,DAL Murat Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria. , 2011, ss.56 - 62. |
| AMA | GELİNCİK A,DEMİRTÜRK M,Buyukozturk S,TELCİ A,BÜYÜKBABANI N,ÇOLA KOĞLU B,DAL M Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria. . 2011; 56 - 62. |
| Vancouver | GELİNCİK A,DEMİRTÜRK M,Buyukozturk S,TELCİ A,BÜYÜKBABANI N,ÇOLA KOĞLU B,DAL M Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria. . 2011; 56 - 62. |
| IEEE | GELİNCİK A,DEMİRTÜRK M,Buyukozturk S,TELCİ A,BÜYÜKBABANI N,ÇOLA KOĞLU B,DAL M "Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria." , ss.56 - 62, 2011. |
| ISNAD | GELİNCİK, Aslı vd. "Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria". (2011), 56-62. |
| APA | GELİNCİK A, DEMİRTÜRK M, Buyukozturk S, TELCİ A, BÜYÜKBABANI N, ÇOLA KOĞLU B, DAL M (2011). Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria. Astım Allerji İmmünoloji, 9(1), 56 - 62. |
| Chicago | GELİNCİK Aslı,DEMİRTÜRK Mustafa,Buyukozturk Suna,TELCİ Ayşegül,BÜYÜKBABANI NESIMI,ÇOLA KOĞLU Bahauddin,DAL Murat Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria. Astım Allerji İmmünoloji 9, no.1 (2011): 56 - 62. |
| MLA | GELİNCİK Aslı,DEMİRTÜRK Mustafa,Buyukozturk Suna,TELCİ Ayşegül,BÜYÜKBABANI NESIMI,ÇOLA KOĞLU Bahauddin,DAL Murat Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria. Astım Allerji İmmünoloji, vol.9, no.1, 2011, ss.56 - 62. |
| AMA | GELİNCİK A,DEMİRTÜRK M,Buyukozturk S,TELCİ A,BÜYÜKBABANI N,ÇOLA KOĞLU B,DAL M Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria. Astım Allerji İmmünoloji. 2011; 9(1): 56 - 62. |
| Vancouver | GELİNCİK A,DEMİRTÜRK M,Buyukozturk S,TELCİ A,BÜYÜKBABANI N,ÇOLA KOĞLU B,DAL M Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria. Astım Allerji İmmünoloji. 2011; 9(1): 56 - 62. |
| IEEE | GELİNCİK A,DEMİRTÜRK M,Buyukozturk S,TELCİ A,BÜYÜKBABANI N,ÇOLA KOĞLU B,DAL M "Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria." Astım Allerji İmmünoloji, 9, ss.56 - 62, 2011. |
| ISNAD | GELİNCİK, Aslı vd. "Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria". Astım Allerji İmmünoloji 9/1 (2011), 56-62. |
