Anca İlişkili Vaskülitlerde Tek Merkez Deneyimi

Year 2021, Volume: 31 Issue: 2, 159 - 162, 30.06.2021

Muhammet Limon Dilek Tezcan , Semral Gülcemal , Sema Yilmaz

https://doi.org/10.15321/GenelTipDer.2021.305

Abstract

Giriş-Amaç: Anti-nötrofil sitoplazmik antikor (ANCA) ilişkili küçük damar vaskülitleri granülomatöz polianjjiitis(GPA), eozinofilik granülomatöz polianjiiitis(EGPA) ve mikroskopik polianjiitistir(MPA). Bu çalışma ile kliniğimizde takip edilen ANCA ilişkili vaskülit sıklığı ve tedavi cevapları değerlendirilmiştir.

Gereç-Yöntem: Bu çalışma retrospektif olarak Ocak 2015-Aralık 2018 arasında yapıldı. Çalışmaya 18 yaş üstü 2012 Chapel Hill sınıflamasına göre ANCA ilişkili vaskülit tanısı alan hastalar alındı.

Bulgular: Kliniğimizde 18 tane ANCA ilişkili vaskülit hastası retrospektif olarak değerlendirildi. Hastaların 12’si kadın, 6’sı erkekti. Hastaların yaş ortalaması 57.8 olarak bulundu. Hastaların 2 tanesi EGPA, 3 tanesi MPA, 13 tanesi GPA tanısı ile izlendiği görüldü. GPA tanısı ile izlenen hastaların 11 tanesi sistemik tutulumla, 2 tanesinin sınırlı tutulumla seyrettiği gözlendi. GPA tanısı ile izlenen hastalarda en sık tutulumun %76 ile akciğer tutulumu, 2. sıklıkta %53 oranında böbrek tutulumu olduğu tespit edildi. MPA ile izlenen hastaların 2 hastada böbrek tutulumu, 2 hastada akciğer tutulumu olduğu saptandı. EGPA tanısı ile izlenen hastaların 2 tanesinde akciğer tutulumu olduğu gözlendi. RF ve ANA pozitifliği GPA’da en yüksek tespit edildi. ANCA ilişkili vaskülitlerde indüksiyon tedavisinde pulse steroid ve siklofosfamid ile kombine olarak verildiği gözlendi. 3 GPA hastasının nüks olduğu ve nüks olan hastaların rituksimab ile izlendiği görüldü.

Tartışma-Sonuç: ANCA ilişkili vaskülitlerin klinik ve tedavisi benzerdir. ANCA ilişkili vaskülitlerin tedavisinde kısa sürede remisyon sağlanmalıdır. ANCA ilişkili vaskülitlerin takibinde nüks önemli bir problemdir. Nüks eden olgularda rituksimab tercih edilebilir.

Keywords

Vaskülit, sıklık, tedavi

Single Center Experience in Anca-Related Vasculitides

Abstract

İntroduction: Granulomatosis polyangitiis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA and microscopic polyangiitis(MPA) are small vessel vasculitides associated with anti-neutrophil cytoplasmic antibody (ANCA). İn this study, the frequency of ANCA associated vasculitis and treatment responses in our clinic are evaluated.

Methods: This retrospective study was performed between January 2015- December 2018. 18 patients who older than 18 and diagnosed ANCA associated vasculitis according to the 2012 Chapel Hill Classification were included.

Results: İn totals, 18 patients who diagnosed ANCA associated vasculitis were included, 12 of whom females, 6 of whom males. The mean age of the patients was 57.8. Two of the patients were diagnosed with EGPA, 3 with MPA and 13 with GPA. 11 of the patients followed up with GPA with systemic involvement and 2 with limited involvement. The most common involvement in patients with GPA was pulmonary involvement with 76% and renal involvement in 53% of the patients. Pulmonary involvement was detected in 2 patients and lung involvement in 2 patients in MPA. Pulmonary involvement was observed in 2 patients with EGPA. RF and ANA positivity was highest in GPA. İt was observed in combination with pulse steroid and cyclophosphamide in the induction treatment of ANCA-associated vasculitis. It was seen that 3 GPA patients had recurrence and followed up with rituximab.

Discussion-conclusion: ANCA related vasculitides treatment and clinic are similar. İn the treatment of ANCA-associated vasculitis, remission should be achieved in a short time. In recurrent cases, rituximab may be preferred.

Keywords

Vasculitis, frequency, treatment

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