OCCURRENCE OF THROMBOTIC MICROANGIOPATHY IN A PATIENT WITH GRANULOMATOSIS WITH POLYANGIITIS AFTER REMISSION INDUCTION THERAPY: A RARE PRESENTATION

Öz

In the literature thrombotic microangiopathy (TMA) associated with ANCA-associated vasculitis (AAV) has only been reported in isolated case reports. Here, we report a patient with granulomatosis with polyangiitis (GPA), who presented with TMA after initiation of remission induction therapy. A 36-yearold male patient presented with dyspnea and decreased urine output. Laboratory results demonstrated elevated creatinine, low albumin, low hemoglobin, normal leukocyte and platelet count, normal LDH, and elevated acute phase reactants. Urinalysis revealed proteinuria (1275 mg/day) and an active urine sediment. Serum complement levels were normal and proteinase 3 ANCA titer was> 200 IU/ml. Urinary ultrasound revealed normal kidney sizes and normal parenchymal thicknesses with increased renal parenchymal echogenicity. A kidney biopsy revealed pauciimmune crescentic glomerulonephritis. The diagnosis was GPA and an induction treatment of pulse steroid, intravenous cyclophosphamide, and plasma exchange was initiated. After two doses of cyclophosphamide, rituximab treatment was initiated. Fifteen days after the second dose of rituximab, thrombotic microangiopathy (TMA) was considered in the patient who had no increase in hemoglobin value (despite initiation of erythropoetin) and decreased platelet count. Peripheral blood smear revealed 5-9 schistocytes in each area. A corrected reticulocyte count was elevated, and haptoglobin was low. ADAMTS13 activity was normal. Plasma exchange was not reinitiated. The kidney biopsy was re-evaluated, but no histopathological changes consistent with TMA were found. The patient was under follow-up for TMA by checking his hematological parameters once a week. Two months later, at the third month of rituximab treatment, an increase in hemoglobin and platelet values was observed. Reticulocyte percent and haptoglobin were within normal limits. His follow-up as an outpatient is continuing. In most of the reported cases of TMA associated with ANCA-associated vasculitis, TMA appeared in the course of active vasculitis. Our case is noteworthy due to the fact that TMA developed after the active phase of GPA, even after the initiation of potent remission induction therapy.

Anahtar Kelimeler

• Granulomatosis with polyangiitis
• remission induction
• thrombotic microangiopathy

GRANÜLOMATÖZ POLİANJİT TANILI BİR HASTADA REMİSYON İNDÜKSİYON TEDAVİSİ SONRASI TROMBOTİK MİKROANJİYOPATİ GELİŞMESİ: NADİR BİR PREZENTASYON

Öz

Literatürde ANCA ilişkili vaskülit (AİV) ile ilişkili trombotik mikroanjiyopati (TMA) sadece olgu sunumlarında bildirilmiştir. Burada remisyon indüksiyonu tedavisi sonrasında TMA tablosuyla başvuran granülomatöz polianjit (GPA) tanılı bir hastayı sunmayı amaçladık. Otuz altı yaşında erkek hasta nefes darlığı ve idrar çıkışında azalma şikayetleriyle başvurdu. Hastanın laboratuvar tetkiklerinde kreatinin ve akut faz reaktanı yüksekliği, albümin ve hemoglobin düşüklüğü, normal lökosit ve trombosit sayısı ve normal LDH düzeyi saptandı. İdrar tahlilinde 1275 mg/gün proteinüri ve aktif idrar sedimenti mevcuttu. Serum kompleman seviyeleri normaldi ancak proteinaz 3 ANCA titresi > 200 IU/ml olarak saptandı. Üriner ultrasonografide normal böbrek boyutları ve normal renal parankim kalınlığı ile artmış renal parankimal ekojenitesi tespit edildi. Böbrek biyopsisinde immün birikimden fakir kresentik glomerülonefrit saptandı. Hastaya GPA tanısı kondu ve yüksek doz steroid, intravenöz siklofosfamid ve plazma değişiminden oluşan güçlü bir indüksiyon tedavisi başlandı. İki doz siklofosfamid sonrası rituksimab tedavisi verildi Eritropoetin başlanmasına rağmen hemoglobin değerinde artış olmayan ve trombosit sayısında azalma olan hastada ikinci rituksimab dozundan 15 gün sonra trombotik mikroanjiyopati (TMA) düşünüldü. Periferik yaymada her alanda 5-9 şistosit görüldü. Düzeltilmiş retikülosit sayısı yüksek, haptoglobin düzeyi düşük, ADAMTS13 aktivitesi normaldi. TMA tablosu için plazmafereze tekrar başlanmadı. Böbrek biyopsisi tekrar değerlendirildi ancak TMA ile uyumlu histopatolojik değişiklik bulunamadı. Hasta TMA açısından haftalık olarak hematolojik parametrelerle takip edildi. İki ay sonra rituksimab tedavisinin üçüncü ayında hemoglobin ve trombosit değerleri arttı, retikülosit yüzdesi ile haptoglobin normal olarak saptandı. Hastanın poliklinik takibi devam etmektedir. Olgumuz, GPA'nın aktif fazından sonra, hatta güçlü remisyon indüksiyon tedavisi başlandıktan sonra TMA tablosunun gelişmesi nedeniyle dikkat çekicidir.

Anahtar Kelimeler

• Granülomatöz polianjit
• remisyon indüksiyonu
• trombotik mikroanjiyopati

Kaynakça

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