Evaluation of Causes of Hypofibrinogenemia in Rheumatology Practice: Retrospective, Single Center Experience

Öz

Hypofibrinogenemia that occurs during the follow-up of rheumatological diseases has not been investigated so far. Our aim was to reveal the causes of hypofibrinogenemia in rheumatology practice. Patients who were followed up in the rheumatology department and were detected to have low fibrinogen levels (<200 mg/dL) were retrospectively screened for etiology. Clinical findings, laboratory data, treatments given for low fibrinogen levels, and patient outcomes of patients with hypofibrinogenemia were evaluated in detail. We found the frequency of hypofibrinogenemia to be 1,78% (28/1573) in patients followed up in the rheumatology department over a 10-year period. 19 (67,9%) of the 28 patients with hypofibrinogenemia were female and the mean age was 51,25±11,88 (28-75) years. During hypofibrinogenemia, 8 patients (28,6%) had fever, 7 patients (28,6%) had hepatomegaly, and 4 patients (14,3%) had bleeding findings. The mean fibrinogen values when hypofibrinogenemia was detected were 134,25±44,80 mg/dL (46,50-194,80). Hypofibrinogenemia was due to tocilizumab side effect in 8 (28,6%) patients, disseminated intravascular coagulation (DIC) in 7 (25%) patients, chronic liver disease in 5 (17,9%) patients, plasmapheresis in 3 (10,7%) patients, macrophage activation syndrome (MAS) in 2 (7,1%) patients, and hemophagocytic lymphohistiocytosis (HLH) in 1 (3,6%) patient. The etiology could not be elucidated in 2 patients. Four (14,3%) patients died after 5,25±4,64 (1-11) days. The mean fibrinogen value of the patients who died was 84,59±52,42 mg/dL and all patients had DIC (p=0,029). In conclusion, it is possible to encounter hypofibrinogenemia during follow-up of rheumatologic diseases. The most common cause is drugs that are interleukin (IL)-6 receptor antagonists. Multicenter, prospective studies with larger patient numbers are needed to determine the true frequency, causes, risk factors, and independent risk factors for death of hypofibrinogenemia in rheumatic diseases.

Anahtar Kelimeler

• Rheumatology
• Tocilizumab
• Hypofibrinogenemia

Romatoloji Pratiğinde Hipofibrinojenemi Nedenlerinin Değerlendirilmesi: Retrospektif, Tek Merkez Deneyimi

Öz

Romatolojik hastalıkların takipleri sırasında ortaya çıkan hipofibrinojenemi şimdiye kadar araştırılmamıştır. Amacımız, romatoloji pratiğinde hipofibrinojenemi nedenlerini ortaya koymaktı. Romatoloji bölümünde takip edilen ve fibrinojen düzeyi düşük (<200 mg/dL) saptanan hastalar etyolojiye yönelik olarak retrospektif olarak tarandı. Hipofibrinojenemisi olan hastaların klinik bulguları, laboratuvar verileri, fibrinojen düşüklüğü için verilen tedaviler ve hasta sonlanımları ayrıntılı olarak değerlendirildi. Romatoloji bölümünde izlenen hastalarda 10 yıllık süre içinde hipofibrinojenemi sıklığını %1,78 (28/1573) olarak saptadık. Hipofibrinojenemi saptanan 28 hastanın 19’u (%67,9) kadın ve ortalama yaş ise 51,25±11,88 (28-75) yıl idi. Hipofibrinojenemi sırasında 8 hastada (%28,6) ateş, 7 hastada (%28,6) hepatomegali ve 4 hastada ise (%14,3) kanama bulguları vardı. Hipofibrinojenemi tespit edildiği sırada fibrinojen değerleri ortalaması 134,25±44,80 mg/dL (46,50-194,80) idi. Hipofibrinojenemi 8 (%28,6) hastada tosilizumab yan etkisi, 7 (%25) hastada dissemine intravasküler koagülasyon (DİK), 5 (%17,9) hastada kronik karaciğer hastalığı, 3 (%10,7) hastada plazmaferez, 2 (%7,1) hastada makrofaj aktivasyon sendromu (MAS) ve 1 (%3,6) hastada ise hemofagositik lenfohistiyositoza (HLH) bağlıydı. İki hastada etyoloji aydınlatılamadı. Dört (%14,3) hasta 5,25±4,64 (1-11) gün sonra ölmüştü. Ölen hastaların fibrinojen değerleri ortalaması 84,59±52,42 mg/dL idi ve tüm hastalarda DİK mevcuttu (p=0,029). Sonuç olarak, romatolojik hastalıkların takibinde hipofibrinojenemi ile karşılaşmak mümkündür. En sık neden interlökin (IL)-6 reseptör antagonisti olan ilaçlardır. Romatolojik hastalıklarda hipofibrinojeneminin gerçek sıklığını, nedenlerini, risk faktörlerini, ölüm için bağımsız risk faktörlerini belirlemek için çok merkezli, daha fazla hasta sayısını içeren prospektif çalışmalara gereksinim vardır.

Anahtar Kelimeler

• Hipofibrinojenemi
• Romatoloji
• Tosilizumab

Kaynakça

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