Identification of Key Predictors of Cryoglobulinemia Severity at Diagnosis: Threshold, Type, and Severity Score at Diagnosis

Résumé fourni par la source

Background: Cryoglobulinemia (CG) syndrome is a heterogeneous condition characterized by the presence of cryoglobulins in serum, often leading to vasculitis with protean clinical manifestations. Understanding the presentation of cryoglobulinemia-related symptoms based on cryoprecipitate levels, GC type, and severity at diagnosis is essential for effective management. Hence, this study aimed to provide a comprehensive analysis of patients with positive cryoglobulin detection to investigate these aspects. Methods: We conducted a retrospective review of clinical charts from patients with positive cryoglobulin detection at Colmar Hospital between May 2015 and April 2019. Results: Among 166 patients with positive cryoglobulins, the median cryoprecipitate value was 37 mg/L [IQR: 25–70], with 62% of patients below the 50 mg/L threshold. High cryoprecipitate levels were associated with C-virus hepatitis (p = 0.0007), increased fatigue (p = 0.001), fever (p = 0.0013), weight loss (p = 0.028), and musculoskeletal symptoms (p = 0.002). These patients also exhibited decreases in complement fractions (p-values 0.017 to 0.006). At the end of the one-year follow-up, they required frequent renal replacement therapy (p < 0.0001) and had a higher mortality rate (p = 0.02). Based on the CG type, patients with type I GC had splenomegaly (p = 0.039) and hemopathy (p = 0.001). According to severity at initial presentation, the severe patients had more purpura (p < 0.001), Raynaud’s phenomenon (p = 0.039), and leukocytoclastic vasculitis on skin biopsy (p < 0.001), along with higher cryoprecipitate levels (p = 0.011). Multivariate analysis identified purpura (OR: 10.25), hematological malignancy (OR: 7.06), Raynaud’s phenomenon (OR: 6.41), and cryoprecipitate levels (OR: 1.02) as significant markers of disease severity serving for the development of a severity score for clinical practice. Conclusions: This study identifies severity markers in patients with positive cryoprecipitate and proposes a score related to severity at diagnosis.

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