Abstract
ObjectiveThe aim of this study was to clarify the clinical characteristics and long-term outcomes of ANCA-associated vasculitis (AAV) patients with pulmonary involvement from a single Chinese cohort.MethodsNewly diagnosed AAV patients with pulmonary involvement, as defined by CT, were recruited from January 2010 to June 2020. Clinical data and CT images were collected retrospectively. Baseline CTs were evaluated and re-classified into four categories: interstitial lung disease (ILD), airway involvement (AI), alveolar hemorrhage (AH), and pulmonary granuloma (PG).ResultsA total of 719 patients were newly diagnosed with AAV, 366 (50.9%) of whom combined with pulmonary involvement at baseline. Among the AAV cases with pulmonary involvement, 55.7% (204/366) had ILD, 16.7% (61/366) had AI alone, 14.8% (54/366) had PG, and 12.8% (47/366) had AH alone. During follow-up of a median duration of 42.0 months, 66/366 (18.0%) patients died, mainly died from infections. Survival, relapse, and infection were all significantly different based on the radiological features. Specifically, the ILD group tends to have a poor long-term prognosis, the PG group is prone to relapse, and the AI group is apt to infection. The AH group has a high risk of both early infection and relapse, thus a poor short-term prognosis.ConclusionAAV patients with diverse radiological features have different clinical characteristics and outcomes. Therefore, the intensity of immunosuppressive therapy must be carefully valued by considering the baseline CT findings among AAV patients with pulmonary involvement.
Highlights
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of disorders characterized by necrotizing inflammation of small- and medium-sized blood vessels and the presence of circulating ANCA
Clinical characteristics Three hundred and sixty-six patients newly diagnosed AAV with pulmonary involvement were eventually enrolled (Fig. 1), of which 249 patients were first admitted to the department of nephrology, 70 from the department of respiratory and critical care medicine, 43 from the department of rheumatology, 2 from the department of infectious disease, 1 from the department of neurology and 1 from the department of thoracic surgery
We found the independent predictors of all-cause mortality during long-term follow-up in AAV patients with pulmonary involvement were usual interstitial pneumonia (UIP) pattern, age ≥ 65 years at diagnosis, respiratory failure, infection needing hospitalization and alveolar hemorrhage (AH), but not including initial renal function, higher Birmingham Vasculitis Activity Score (BVAS), lower hemoglobin, and higher white cell count in other publications [9, 25,26,27, 30]
Summary
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of disorders characterized by necrotizing inflammation of small- and medium-sized blood vessels and the presence of circulating ANCA. Clinical disease phenotypes include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) [1]. Pulmonary involvement is frequent and prominent among various manifestations of AAV. Radiologic finding, mainly computed tomography (CT), is key for the identification of pulmonary involvement. Previous studies showed that 52–80% of patients with AAV had pulmonary abnormalities on chest CT [3,4,5], mainly including interstitial lung disease (ILD), granuloma (nodules, masses, with or without cavitation), alveolar hemorrhage (AH). MPA is the main AAV subtype associated with ILD [4, 8,9,10]. Granuloma is more prevalent in GPA [4, 11]
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