Pulmonary Fibrosis in Antineutrophil Cytoplasmic Antibodies (ANCA)-Associated Vasculitis

Abstract

Pulmonary fibrosis (PF) is an uncommon manifestation observed in patients with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), particularly microscopic polyangiitis (MPA). While patients with PF associated with AAV seem to have a worse prognosis, these patients have been described only in case reports or small retrospective case series. In this retrospective multicenter study, we report the main features and long-term outcomes of patients with PF associated with AAV, fulfilling the American College of Rheumatology criteria and/or Chapel Hill definitions. Forty-nine patients (30 men [61%]; median age at diagnosis of AAV, 68 [interquartile range, 58–73] years) with PF associated with AAV were identified. Forty (81.6%) patients had MPA and 9 (18.4%) had granulomatosis with polyangiitis. The diagnosis of PF preceded the onset of vasculitis in 22 (45%) patients. Usual interstitial pneumonia was the main radiologic pattern (n = 18, 43%). ANCA were mostly of antimyeloperoxidase specificity (88%). All patients were treated with glucocorticoids as induction therapy, combined with cyclophosphamide (CYC) (n = 36, 73.5%) or rituximab (RTX) (n = 1, 2%). Factors associated with mortality included occurrence of chronic respiratory insufficiency (hazard ratio [HR], 7.44; 95% confidence interval [CI], 1.6–34.5; p = 0.003), induction therapy with glucocorticoids alone (HR, 2.94; CI, 1.05–8.33; p = 0.04), and initial weigh loss (HR, 2.83; CI, 1.05–7.65; p = 0.041). The 3-year survival rate in patients treated with glucocorticoids alone or combined with an immunosuppressant (CYC or RTX) as induction therapy was 64% (95% CI, 41–99) and 94% (95% CI, 86–100), respectively (p = 0.03). After a median follow-up of 48 months [interquartile range, 14–88 mo], 18 (37%) patients died, including 11 related to respiratory insufficiency. PF is a rare manifestation of AAV with a very poor prognosis. Induction therapy with CYC might improve the outcome.

Interstital lung disease in ANCA vasculitis

“…Prevalence of ILD is higher in MPA than in GPA [15,18,51]. PF has been reported in 23% of GPA cases [52] and in 2.7–45% of MPA patients.…”

“…In these cases, interstitial disease was usually identified at disease onset [7,11,17–20,48,53,54]. Importantly, there is a significant predominance of MPO-ANCA in published series, i.e., 46–71% of all cases compared to 0–29% for PR3-ANCA [13,27,51,52,54–56]. …”

Interstitial Pneumonia in a 94-Year-Old Woman with MPO-ANCA Positive Vasculitis

“…Mortality was relatively high and life threatening acute exacerbation may occur. The previous reports show that median age at diagnosis of IP by MPO-ANCA associated vasculitis was over 60 (range; 4 -85) years, with a male predominance [1]- [3]. Most patients died related to respiratory insufficiency.…”

“…Mortality was relatively high and life threatening acute exacerbation may occur. The age of patients in IP associated with MPO-ANCA may be related poor prognostic course, since they suffer the other significant sickness and their symptoms may be masked by their holding diseases [1]- [3].…”

Pulmonary Fibrosis in Antineutrophil Cytoplasmic Antibodies (ANCA)-Associated Vasculitis

“…In Figure 1 of the article “Pulmonary Fibrosis in Antineutrophil Cytoplasmic Antibodies (ANCA)-Associated Vasculitis: A Series of 49 Patients and Review of the Literature”, 1 which appeared in Volume 93, Issue 24 of Medicine , the descriptions for panels B and C were originally reversed. The correct legend is given below.…”