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Target Agent(s)Blue Cross Blue Shield of Kansas

systemic sclerosis-associated interstitial lung disease (SSc-ILD)

Initial criteria

  • ONE of the following must be met:
  • A. Diagnosis of idiopathic pulmonary fibrosis (IPF) AND ALL of the following:
  • 1. Other known causes of interstitial lung disease have been excluded (e.g., environmental exposures, connective tissue diseases, drug toxicities, alternative diagnoses) AND
  • 2. ONE of the following:
  • A. High-resolution computed tomography (HRCT) showing usual interstitial pneumonia (UIP) pattern OR
  • B. Surgical lung biopsy confirming UIP OR
  • C. HRCT showing probable UIP pattern AND surgical lung biopsy indicating probable UIP;
  • B. Diagnosis of systemic sclerosis–associated interstitial lung disease (SSc‑ILD) AND ALL of the following:
  • 1. Diagnosis confirmed by HRCT or chest radiography, AND
  • 2. ONE of the following:
  • A. Tried and had inadequate response to ONE conventional agent (e.g., mycophenolate mofetil, cyclophosphamide, azathioprine) OR
  • B. Intolerance or hypersensitivity to ONE conventional agent OR
  • C. FDA labeled contraindication to ALL conventional agents;
  • C. Diagnosis of chronic fibrosing interstitial lung disease (ILD) with a progressive phenotype AND BOTH of the following:
  • 1. Meets TWO of these criteria within the past year: worsening respiratory symptoms OR physiologic evidence of disease progression OR radiologic evidence of disease progression AND
  • 2. Alternative explanations of worsening features have been excluded;
  • D. Has another FDA‑labeled indication for the requested agent and route of administration;
  • AND if patient has an FDA‑labeled indication, ONE of the following must also apply:
  • A. Patient’s age is within FDA labeling for the requested indication, OR
  • B. There is support for using the agent for patient’s age for the requested indication;
  • AND
  • The prescriber is a specialist (pathologist, pulmonologist, radiologist, or rheumatologist) or has consulted with one;
  • AND
  • The requested agent will NOT be used in combination with another agent included in this prior authorization program;
  • AND
  • The patient does NOT have any FDA‑labeled contraindications to the requested agent.

Reauthorization criteria

  • Patient has been previously approved for the requested agent through the plan’s Prior Authorization process;
  • AND patient has had clinical benefit with the requested agent;
  • AND prescriber is a specialist in area of diagnosis or has consulted one;
  • AND agent will not be used in combination with another agent included in this prior authorization program;
  • AND patient does not have any FDA labeled contraindications to the requested agent.

Approval duration

12 months