Background: The association between interstitial lung disease (ILD) and antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) has been increasingly recognized in recent years. The clinical features and prognostic differences between AAV-associated ILD and isolated ANCA-positive idiopathic interstitial pneumonias (IIPs) remain unclear. The purpose of this study was to determine the clinical significance and prognosis of ANCA-positive ILD to further guide clinical management. Methods: This study retrospectively reviewed the data of 379 ILD patients with available ANCA results and ultimately analysed 49 ANCA-positive patients. AAV diagnosis was based on the 2012 revised Chapel Hill Consensus Conference (CHCC) criteria, and 33 of 49 patients were diagnosed with microscopic polyangiitis (MPA). The baseline clinical information and laboratory parameters were collected and analysed at each patient’s initial diagnosis. Results: Among 49 ANCA-positive ILD patients, the high-resolution computed tomography (HRCT) pattern was mainly usual interstitial pneumonia (UIP) (59.18%), followed by nonspecific interstitial pneumonia (NSIP) (26.53%). The C-reactive protein (CRP) level (43.89± 40.61 versus 18.74± 20.05, p = 0.028) and erythrocyte sedimentation rate (ESR) (71.97± 42.73 versus 40.69± 28.46, p = 0.011) were significantly higher in the MPA-ILD group than in the ANCA-IIP group. Haemoglobin (113.09 ± 24.47 versus 132.19± 13.34, p = 0.006) and albumin (32.95± 5.84 versus 36.52± 3.94, p = 0.032) levels were significantly lower. Survival was shorter among MPA-ILD patients than among ANCA-IIP patients [hazard ratio (HR) 3.38, 95% confidence interval (CI) 1.32–8.67, p = 0.040]. In the multivariable Cox analysis, a diagnosis of MPA (HR 3.91, 95% CI 1.07–14.08, p = 0.038) and acute exacerbation (AE) of ILD (HR 9.43, 95% CI 2.89–30.30, p < 0.001) were significantly independently associated with shorter survival in ANCA-positive ILD patients, and the NSIP pattern (HR 0.07, 95% CI 0.01–0.41, p = 0.003) was independently associated with prolonged survival. Conclusion: ANCA-ILD patients mostly have myeloperoxidase (MPO)-ANCA positivity and an MPA diagnosis. Survival was shorter among MPA-ILD patients than among ANCA-IIP patients. Respiratory failure and AE were associated with poorer prognosis. Early antifibrotic treatment may be a reasonable treatment option in fibrotic ILD patients with ANCA positivity.

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Background Lactate dehydrogenase (LDH) is an easily obtained biological marker and a promising index for determining the severity of amyopathic dermatomyositis with interstitial lung disease (ADM-ILD). In this study, we retrospectively analysed the clinical signs and laboratory data from ADM-ILD patients displaying nonspecific interstitial pneumonia (NSIP) or organizing pneumonia (OP) ILD radiological patterns, and we identified the serum LDH level as a useful biomarker for early ADM-ILD detection. Methods In our cohort study, 158 Chinese patients were divided into four groups according to whether NSIP or OP patterns were present on high-resolution computed tomography (HRCT). The 4 groups included 25 ADM-NSIP and 92 control-NSIP patients, 15 ADM-OP patients, and 29 control-OP patients. Patient’s demographic features, clinical presentation, laboratory parameters, duration of ILD, and follow-up data were recorded and analysed. Results ADM-ILD patients generally had higher AST (45.96 ± 42.45 in NSIP, 64.53 ± 75.75 in OP, U/L) and LDH (317.20 ± 104.29 in NSIP, 356.60 ± 214.84 in OP, U/L) levels than the corresponding control groups. A total of 40.53% (15/37) and 86.49% (32/37) of ADM-ILD patients had high serum levels of AST and LDH above the normal range, respectively. At a cut-off level of 235.5 U/L, identified by the ROC curve, serum LDH yielded a sensitivity of 78.38% and specificity of 82.64% for ADM with NSIP or OP radiological patterns. The 5-year overall survival rate of ADM-OP patients (73.33%) was significantly worse than that of control-OP patients (94.12%) (p=0.007). No difference was evident in the survival rate between the NSIP groups. The presence of skin ulcers (p=0.028) and OI<300 mmHg (p=0.029) were significant risk factors for poor outcomes in ADM-ILD patients. A lower serum level of ferritin (< 500 ng/ml) indicated a better patient prognosis (p=0.023). The presence of coexistent myositis-associated autoantibodies with anti-melanoma differentiation-associated gene 5 (anti-MDA5) seems to protect ADM-ILD patients. Conclusions The LDH level can be used as a screening biomarker for patients with NSIP or OP ILD radiological patterns. It is important to recognize ADM-ILD early, especially in patients with the OP radiological pattern, which appears to impact patient treatment and outcomes.

Background The association between pulmonary involvement and microscopic polyangiitis (MPA) has been increasingly recognized in recent years. Whether interstitial lung disease (ILD) and bronchiectasis (BE) are disease manifestations of MPA, preexisting comorbidities or important complications remains unclear. The purpose of this study was to determine the clinical characteristics and prognosis of MPA with pulmonary involvement to further guide clinical management. Methods The data for 97 patients with a definitive diagnosis of MPA were retrospectively reviewed. The MPA diagnosis was based on the 2012 revised Chapel Hill Consensus Conference (CHCC) criteria. The baseline clinical information and laboratory parameters were collected and analysed at each patient’s initial diagnosis. Results Forty-seven out of the 97 (48.5%) patients who were diagnosed with MPA presented with pulmonary involvement, including 37 patients with ILD, 12 patients with BE and two patients with diffuse alveolar haemorrhage (DAH). ILD and BE antedated MPA in 56.76% and 75.00% of the patients, respectively. Compared with that in the MPA-BE group, the serum LDH level (222.86 ± 68.19 vs. 171.58 ± 31.43, p = .016) in the MPA-ILD group was significantly higher. In the multivariate Cox analysis, elevated serum creatinine (HR 4.08, confidence interval (CI) 1.38–12.05, p = .011) was an independent risk factor for shorter survival in MPA patients with pulmonary involvement, and treatment with glucocorticoid pulse cyclophosphamide therapy (HR 0.095, 95% CI 0.019–0.47, p = .004) was independently associated with prolonged survival. Among the patients in the MPA-ILD group, acute exacerbations of ILD (HR 4.55 CI 1.16–17.86, p = .029) and elevated serum creatinine (HR 4.95, CI 1.39–17.54, p = .014) were independently associated with a poor prognosis, and treatment with glucocorticoids (HR 0.057, 95% CI 0.012–0.28, p < .001) was independently associated with significant prolongation of survival. Conclusions Patients with MPA have a high prevalence of pulmonary involvement, and ILD is the most common subtype of MPA. ILD and BE can be considered preexisting comorbidities of MPA. Elevated serum creatinine was associated with shorter survival. However, remission induction regimens with glucocorticoids and/or immunosuppressants may improve this outcome.

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