Objectives. This study investigated the frequency and patterns of interstitial lung disease (ILD) and their clinical effect on all-cause mortality during the follow-up period in patients with antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) in Korea. Methods. The medical records of 255 AAV patients with ILD were retrospectively reviewed. ILD and its patterns, the usual interstitial pneumonia (UIP) and non-UIP patterns, were confirmed using high-resolution computed tomography both at AAV diagnosis and during follow-up. Forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DLCO) were also obtained. Results. The median age was 65.0 years, and 34.9% were male. ILD occurred in 53 patients, among whom 49.1% developed ILD after AAV diagnosis. Among AAV subtypes, the frequencies of ILD were significantly higher in both patients with microscopic polyangiitis (MPA) and those with AAV having myeloperoxidase (MPO)-ANCA (or P-ANCA) compared to other subtypes. However, there was no statistical significance in AAV subtypes or FVC/DLCO ratio between patients with the UIP and non-UIP patterns. In particular, the cumulative patients’ survival rate was lower in patients with AAV and ILD than in those without ILD. Conclusions. ILD occurred in one-fifth of Korean patients with AAV in this study and was associated with MPA and MPO-ANCA (or P-ANCA). In addition, ILD significantly increased the rate of all-cause mortality in these patients with AAV. Therefore, we suggest the need for more attention and more frequent regular visit for patients with AAV and ILD regardless of the time of ILD occurrence.
Objective
To investigate the significance of rheumatoid factor (RF) positivity in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients.
Methods
AAV patients were divided into groups as follows: RF (+)/ANCA (+) (n = 94), RF (-)/ANCA (+) (n = 80), RF (+)/ANCA (-) (n = 15), and RF (-)/ANCA (-) (n = 25). Their clinical data, organ involvement patterns, laboratory data, and patient outcomes were assessed. Kaplan–Meier analysis and propensity score matching (PSM) were performed to compare outcomes and analyse differences between the groups.
Results
Of the 214 patients, RF and ANCA positivity was found in 109 (50.9%) and 174 (81.3%) patients, respectively. RF (+)/ANCA (+) patients more frequently presented with general manifestations (58.5%) than the other groups. Additionally, compared with those of RF (-)/ANCA (+) group, RF (+)/ANCA (+) patients were older, had higher white blood cell, neutrophil, platelet counts, and acute phase reactants; however, creatinine and albumin levels were lower. The end-stage kidney disease-free survival rate was significantly higher in the RF (+)/ANCA (+) group (p= 0.013), while the proportion of renal involvement was comparable to the RF (-)/ANCA (+) group. PSM showed no difference in patient outcomes between the two groups after adjustment.
Conclusion
RF positivity was associated with a distinct phenotype in AAV patients. In particular, difference was observed in clinical features and outcomes between RF (+)/ANCA (+) and RF (-)/ANCA (+) groups, although the direct prognostic implication of RF was not evident.
Objective. We investigated whether antineutrophil cytoplasmic antibody (ANCA) positivity at diagnosis may be associated with the cross-sectional clinical features at diagnosis and predicting all-cause mortality during follow-up in Korean patients with systemic sclerosis (SSc). In addition, we assessed the incidence of SSc and ANCA-associated vasculitis (AAV) overlap syndrome in patients with ANCA positivity. Methods. We retrospectively reviewed the clinical and laboratory features through the medical records of 177 SSc patients who fulfilled the inclusion and exclusion criteria. SSc was classified by the 2013 American College of Rheumatology/European League Against Rheumatism classification criteria. AAV was classified by the 2007 European Medicine Agency algorithms and the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Results. The median age was 52 years, and 23 patients were males. The detection rate of ANCA in Korean patients with SSc was 20.3%. Unlike a previous study, ANCA positivity at diagnosis was significantly associated with neither the cross-sectional clinical and laboratory variables at diagnosis nor the rate of all-cause mortality during followup in Korean patients with SSc. However, three female patients (8.3%) with ANCA could be classified as having microscopic polyangiitis (MPA) during follow-up. Conclusion. No significant associations of ANCA positivity with the cross-section clinical features or allcause mortality during follow-up were observed in this study. But, given that 3 of 36 SSc patients with ANCA were classified as having AAV based on the histological confirmation, we suggest that physicians should consider recommending a biopsy when AAV is strongly suspected in SSc patients with ANCA.
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