Yonsei Med J.  2018 Mar;59(2):294-302. 10.3349/ymj.2018.59.2.294.

Red Blood Cell Distribution Width Can Predict Vasculitis Activity and Poor Prognosis in Granulomatosis with Polyangiitis

• Kim HJ
• Yoo J
• Jung SM
• Song JJ
• Park YB
• Lee SW

• KMID: 2418794
• DOI: http://doi.org/10.3349/ymj.2018.59.2.294

Abstract

PURPOSE
We investigated whether red blood cell distribution width (RDW) predicts vasculitis activity based on Birmingham vasculitis activity score (BVAS) or BVAS for granulomatosis with polyangiitis (GPA) at diagnosis and poor prognosis during follow-up in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
MATERIALS AND METHODS
We reviewed the medical records of 150 patients with AAV. We defined severe GPA as BVAS for GPA ≥7 (the highest quartile). Correlation and standardised correlation coefficients were analysed by linear regression tests. The differences between groups were evaluated by Mann-Whitney test. Relative risk (RR) was assessed by chi square test and Cox hazards model.
RESULTS
RDW was correlated only with the vasculitis activity of GPA among patients with AAV. An increase in RDW was associated with the absence of ear nose throat (ENT) manifestation, but not proteinase 3-ANCA. Significant differences were noted in cumulative refractory free survival according to RDW ≥15.4% (p=0.007) and the absence of ENT manifestation (p=0.036). Multivariate Cox hazards analysis identified RDW ≥15.4% as the only significant predictor of refractory disease in GPA (RR 17.573).
CONCLUSION
RDW predicts vasculitis activity in GPA, and RDW ≥15.4% at diagnosis may increase the risk of severe GPA at diagnosis and predict refractory diseases during follow-up.

Keyword

Red blood cell distribution width; ANCA-associated vasculitis; granulomatosis with polyangiitis; vasculitis activity score; prognosis

MeSH Terms

Aged
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/*blood/diagnosis
*Erythrocyte Indices
Female
Follow-Up Studies
Granulomatosis with Polyangiitis/*blood/diagnosis
Humans
Linear Models
Middle Aged
Multivariate Analysis
Prognosis

Figure

• 

  Fig. 1 Comparison of RDW between the presence and absence of clinical aspects and ANCA types in patients with GPA at diagnosis. In GPA patients, among organ-based clinical manifestations, only patients with ENT symptoms had the lower mean RDW than those without (13.9% vs. 19.0%, p=0.001). However, there was no significant difference in RDW between the presence and absence of PR3-ANCA. RDW, red blood cell distribution width; ANCA, antineutrophil cytoplasmic antibody; GPA, granulomatosis with polyangiitis; ENT, ear nose throat; MPO, myeloperoxidase; PR3, protainase 3.

• 

  Fig. 2 Cumulative refractory disease free survival rates during the follow-up of GPA. (A, B) There were significant differences in cumulative refractory disease free survival rates according to RDW ≥15.4% (p=0.007) and the absence of ENT manifestation (p=0.036). Patients with RDW ≥15.4% and no ENT symptoms at diagnosis exhibited a significantly increased risk of refractory disease of GPA than those with RDW <15.4% and ENT manifestation (RR 19.527, 95% CI 2.271–167.905, and RR 0.177, 95% CI 0.035–0.890, respectively). GPA, granulomatosis with polyangiitis; RDW, red blood cell distribution width; ENT, ear nose throat; RR, relative risk; CI, confidence interval.

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