What is the recommended treatment for a 69-year-old patient with Rheumatoid Arthritis (RA) and Connective Tissue Disease-Interstitial Lung Disease (CTD-ILD) with a Usual Interstitial Pneumonia (UIP) pattern, currently on Tocilizumab (tocilizumab) monthly?

Treatment of RA-Associated CTD-ILD with UIP Pattern

For a 69-year-old patient with rheumatoid arthritis and progressive UIP pattern CTD-ILD currently on tocilizumab, I recommend adding nintedanib as the most appropriate treatment option based on the latest guidelines.

Assessment of Current Situation

The patient presents with:

• 69-year-old with rheumatoid arthritis
• Positive RF and high CCP antibodies
• Elevated inflammatory markers
• Active joint symptoms
• UIP pattern interstitial lung disease with progression of fibrosis
• Currently on tocilizumab monthly

First-Line Treatment Options for RA-ILD with UIP Pattern

Current Treatment Analysis

• The patient is already on tocilizumab, which is conditionally recommended for RA-ILD by the 2023 ACR/CHEST guidelines 1
• However, the patient shows progression of fibrosis despite tocilizumab therapy, indicating need for additional treatment

Recommended Treatment Approach

Add Nintedanib

• For patients with RA-ILD progression despite first ILD treatment, nintedanib is conditionally recommended as a treatment option 1
• Nintedanib is particularly appropriate for patients with UIP pattern on HRCT and progressive fibrosing disease 1
• The 2023 ACR/CHEST guidelines specifically note that some panelists consider nintedanib as a first-line ILD therapy option in patients with RA-ILD with a fibrotic/UIP pattern 1

Alternative Options to Consider

1. Mycophenolate mofetil (MMF):

   • Conditionally recommended for SARD-ILD progression despite first ILD treatment 1
   • Has shown efficacy in stabilizing lung function in CTD-ILD, though less impressive results in UIP pattern specifically 2

2. Pirfenidone:

   • Conditionally recommended specifically for RA-ILD progression despite first ILD treatment 1
   • May attenuate FVC progression in RA-ILD, particularly with UIP pattern 1

3. Rituximab:

   • Conditionally recommended for SARD-ILD progression despite first ILD treatment 1
   • May be more effective in NSIP pattern than UIP pattern 3
   • Recent evidence shows promising results when combined with MMF in NSIP pattern 4

Treatment Algorithm

1. First step: Add nintedanib to current tocilizumab therapy

   • Recommended dose: 150 mg twice daily 5
   • Monitor for gastrointestinal side effects (particularly diarrhea)
   • Regular liver function tests

2. If nintedanib is not tolerated or ineffective:

   • Consider adding pirfenidone as an alternative antifibrotic specifically recommended for RA-ILD 1
   • OR
   • Consider switching to or adding mycophenolate mofetil (2g daily) 2

3. For refractory disease:

   • Consider rituximab, particularly if there are features suggesting antibody-mediated disease 3
   • Consider referral for lung transplantation evaluation if rapid progression despite therapy 1

Monitoring Recommendations

• Pulmonary function tests every 3-6 months (FVC and DLCO)
• HRCT annually or if unexplained clinical changes occur
• Regular assessment of oxygen saturation
• Monitor for medication side effects
• Implement supportive care measures:
  • Pulmonary rehabilitation
  • Oxygen therapy if indicated
  • Vaccination (influenza, pneumococcal)
  • Gastroesophageal reflux management

Important Caveats

• Avoid long-term glucocorticoids in RA-ILD with UIP pattern, as they have not been shown to improve outcomes and carry significant risks 1
• UIP pattern generally has less favorable response to immunosuppressive therapy compared to NSIP pattern 2, 3
• Combination therapy with antifibrotics and immunosuppressants requires careful monitoring for adverse effects
• The presence of progressive fibrosis despite tocilizumab indicates a more aggressive phenotype that warrants targeted antifibrotic therapy