The incidence of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis in patients with idiopathic pulmonary fibrosis (IPF) is higher than that of the general population, according to a new case report and literature review. The study, published in Medicine, described a successful outcome with cyclophosphamide in combination with methylprednisolone as remission induction therapy.

The case involved a 64-year-old woman who presented with ground-glass opacities and usual interstitial pneumonia (UIP) alterations on chest high-resolution computed tomography (HRCT), resulting in a diagnosis of IPF. One year later, she was hospitalized with diffuse alveolar hemorrhage and progressive glomerulonephritis, with ANCA-associated vasculitis as a differential diagnosis, and she was administered cyclophosphamide and high-dose glucocorticoids to induce remission.

“The patient achieved clinical and serological remission within 6 weeks of diagnosis with renal function recovery, resolution of ground-glass opacities on chest HRCT, and normalization of [antimyeloperoxidase] antibody levels,” the authors wrote. “Maintenance therapy consisted of mycophenolate mofetil (2 g/d) and prednisone (0.25 mg/kg/d).” Six months after the diagnosis of vasculitis, the patient was still in remission.

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The report notes that up to 20% of patients with IPF present with ANCA positivity at the time of diagnosis or during follow-up. ANCA-positive patients have a higher prevalence of ground-glass opacities superimposed on the UIP alterations as well as a greater number of neutrophils in bronchoalveolar lavage fluid than those without ANCAs.

Typically, there are no clinical symptoms of systemic vasculitis on diagnosis, but a significant proportion of these patients will go on to develop vasculitis. The mainstay of treatment of ANCA-associated vasculitis is based on international guidelines.

The authors concluded that IPF with ANCA positivity could represent a distinct IPF phenotype. Due to the clinical and therapeutic implications of the development of vasculitis, they recommend autoimmune testing for ANCAs as part of the routine diagnostic workup for patients with IPF.


Traila D, Marc MS, Pescaru C, Manolescu D, Fira-Mladinescu O. ANCA-associated vasculitis in idiopathic pulmonary fibrosis: a case report and brief review of the literature. Medicine (Baltimore). 2022;101(9):e29008. doi:10.1097/MD.0000000000029008