FIGURE 2.

Suggested approach to diagnosis and treatment of antineutrophil cytoplasmic antibody (ANCA)-associated interstitial lung disease (ILD). HRCT: high-resolution computed tomography; AAV: ANCA-associated vasculitis; MDD: multidisciplinary discussion; UIP: usual interstitial pneumonia (the most common pattern of ANCA-ILD); NSIP: nonspecific interstitial pneumonia; OP: organising pneumonia, GCS: glucocorticoids; MMF: mycophenolate mofetil; ^#: Transbronchial biopsy (TBBx), cryobiopsy, or surgical lung biopsy (SLB), as per the treating clinician's discretion, local expertise and/or MDD; elective SLB in patients may be considered if TBBx and/or cryobiopsy is non-diagnostic in patients who are stable and are not at high risk for surgical complications. ^¶: Pursue alternative diagnoses guided by appropriate clinical setting and guidelines (e.g., idiopathic pulmonary fibrosis, hypersensitivity pneumonitis, sarcoidosis, etc.).