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

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.

Delta Neutrophil Index Is Associated with Vasculitis Activity and Risk of Relapse in ANCA-Associated Vasculitis

PURPOSE: Delta neutrophil index (DNI) represents the immature granulocytes count associated with neutrophil-consumption. We investigated whether DNI might be associated with Birmingham vasculitis activity score (BVAS) at diagnosis and could predict relapse during the follow-up in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). MATERIALS AND METHODS: We reviewed the medical records of 97 patients having DNI results. Twenty patients had granulomatosis with polyangiitis (GPA), 58 had microscopic polyangiitis (MPA), and 19 had eosinophilic GPA (EGPA). We collected clinical and laboratory data including BVAS, five factor score (FFS), and DNI. The correlation coefficient and cumulative relapse free survival rate were obtained. The optimal cut-off of DNI was extrapolated by calculating the area under the receiver operator characteristic curve. RESULTS: DNI was significantly related to cross-sectional BVAS. Furthermore, among continuous variables, only DNI could reflect BVAS of GPA and MPA, but not EGPA. Severe AAV was defined as BVAS ≥20 (the highest quartile). At diagnosis, patients having DNI ≥0.65% had a significantly higher risk of severe GPA and MPA than those having not (relative risk 4.255) at diagnosis. During the follow-up, DNI ≥0.65% could predict the higher relapse rate. CONCLUSION: DNI could reflect BVAS at diagnosis and furthermore, DNI ≥0.65% could not only identify severe AAV at diagnosis, but also predict relapse during the follow-up in patients with GPA and MPA.

Vasculites pulmonares para o pneumologista: uma "cartilha introdutória" / Pulmonary vasculitis for pulmonologists: a 'introductory primer

Vasculites pulmonares são doenças raras, mas com grande possibilidade de produzir morbimortalidade nos indivíduos acometidos, não somente pela sua potencial gravidade multisistêmica, mas também por seu diagnóstico geralmente tardio. A identificação de padrões de doença que remetem a estes diagnósticos e a utilização de novos meios de investigação - incluindo ANCA (anticorpo antineutrofílico citoplasmático), reconhecendo suas vantagens e limitações - são importantes, se quisermos utilizar o moderno arsenal terapêutico da forma adequada e com sucesso. Atenção às resultantes clínicas é fundamental no acompanhamento de longo curso destes pacientes

Pulmonary vasculitis are rare illnesses, although possessing substantial morbi-mortality in affected individuals, not only due to its multisystemic nature, but also because of commonly delayed diagnosis. Disease pattern recognition that remind us of that group of diseases, and the use of modern investigation methods - including ANCA (anti-neutrophil cytoplasmic antibodies), with the knowledge of its pros and cons, are key features if the state of the art therapeutic modalities are to be properly used with success. Attention to clinical outcomes is fundamental in the long range management of these patients