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Interstitial Lung Disease (ILD) — Management

Overview

ILD is a heterogeneous group of lower respiratory tract disorders characterized by cough, progressive dyspnea, and abnormal pulmonary physiology without infection or neoplasm. The key to management is accurate diagnosis — treatment strategy varies substantially by subtype. A multidisciplinary team (pulmonology, radiology, pathology with ILD expertise) is standard.

General Principles

  • HRCT of the chest is mandatory for all ILDs — it is more sensitive than plain radiography and guides both diagnosis and treatment decisions.
  • Lung biopsy (VATS preferred over transbronchial) when HRCT is non-diagnostic.
  • Identify and address reversible causes: drug toxicity, occupational/environmental exposures, connective tissue disease (CTD).
  • Supportive care (pulmonary rehab, supplemental O₂, vaccinations) applies to all patients.
  • Lung transplant is a consideration for end-stage ILD of any subtype — refer early.

Management by ILD Subtype

1. Idiopathic Pulmonary Fibrosis (IPF)

IPF is the most common idiopathic interstitial pneumonia. Risk factors: age >60, male sex, smoking, GERD, familial/genetic variants (MUC5B, TERT, TERC, SFTPC).
Antifibrotic therapy (first-line, disease-modifying):
DrugMechanismKey Side Effects
Pirfenidone (oral)Antifibrotic, anti-inflammatoryPhotosensitivity, rash (30%), nausea, diarrhea; monitor LFTs
Nintedanib (oral, 150 mg BID)Tyrosine kinase inhibitorDiarrhea (62%), nausea, vomiting; monitor LFTs
Both slow the rate of FVC decline and may confer a mortality benefit. LFT monitoring is mandatory for both agents.
⚠️ Avoid: Combined NAC + azathioprine + prednisone — associated with increased mortality and hospitalization.
Acute exacerbations of IPF: Treat with high-dose corticosteroids (benefit unproven); mortality is high and patients rarely return to pre-exacerbation baseline.
Pulmonary rehabilitation: Improves 6-minute walk distance and quality of life.
Prognosis (median survival by spirometric severity):
  • Mild: 55.6 months | Moderate: 38.7 months | Severe: 27.4 months
Poor prognostic markers:
  • FVC decline >10% over 6 months
  • DLCO decrease >15% over 6 months
  • 6-minute walk distance decrease >150 m over 12 months
Lung transplantation: Refer at time of IPF diagnosis — the ultimate therapy for advanced disease.

2. Nonspecific Interstitial Pneumonia (NSIP)

NSIP is most commonly secondary (CTD, drugs, HIV) rather than idiopathic. Patients are typically younger and more often female than IPF patients.
CTD-associated NSIP (most common):
  • Immunosuppression with corticosteroids + steroid-sparing agents:
    • Mycophenolate, azathioprine, rituximab, cyclophosphamide
  • For SSc-ILD: Nintedanib (150 mg BID) slows FVC decline; tocilizumab (162 mg SC weekly) may also be added
  • Multidisciplinary management is essential
Drug-induced NSIP: Discontinue the culprit agent (common offenders: amiodarone, nitrofurantoin, methotrexate, statins, chemotherapy)
Secondary causes (HIV, IgG4): Treat underlying disease (HAART; steroids for IgG4)
Prognosis is significantly better than IPF.

3. Hypersensitivity Pneumonitis (HP)

HP spans a spectrum from non-fibrotic (reversible) to fibrotic/honeycombing (poor prognosis).
Prognosis by HRCT phenotype:
  • No fibrosis: median survival >14 years
  • Fibrosis: median survival ~8 years
  • Fibrosis + honeycombing: median survival ~2.8 years
Management:
  1. Antigen avoidance — the single most important intervention; significantly improves survival when the antigen is identified (identified in <50% of cases)
  2. Corticosteroids — classic mainstay for fibrotic disease; RCT data lacking
  3. Antifibrotic therapy (nintedanib) — for fibrotic/progressive HP
  4. Immunosuppressive therapy for refractory cases

4. Connective Tissue Disease–Associated ILD (CTD-ILD)

Includes RA-ILD, SSc-ILD, myositis-ILD, Sjögren-ILD, antisynthetase syndrome-ILD.
  • Pattern may be UIP, NSIP, or COP
  • Corticosteroids ± immunosuppressive agents (cyclophosphamide, azathioprine, mycophenolate, rituximab)
  • For SSc-ILD: nintedanib + tocilizumab evidence-based
  • UIP in RA carries worse prognosis than NSIP in RA; UIP in RA has worse prognosis than UIP in other CTDs

5. Sarcoidosis

  • Corticosteroids — first-line
  • Immunosuppressive therapy for refractory/steroid-dependent disease: methotrexate, azathioprine, infliximab

6. Smoking-Related ILD (DIP, RB-ILD)

  • Smoking cessation — the cornerstone of treatment
  • Corticosteroids (likely of limited benefit)

7. Cryptogenic Organizing Pneumonia (COP)

  • Corticosteroids — highly responsive; most patients improve significantly
  • Relapses are common on steroid taper; prolonged course may be needed

8. Progressive Pulmonary Fibrosis (PPF)

A phenotype (not a diagnosis) — applies when non-IPF ILDs behave progressively (SSc-ILD, HP, others). Nintedanib has been shown to slow progression in PPF regardless of underlying cause (supported by meta-analyses).

Supportive & Adjunctive Measures (All ILDs)

InterventionIndication
Supplemental O₂Resting or exertional hypoxemia
Pulmonary rehabilitationAll ILDs, especially IPF
Influenza/pneumococcal vaccinesAll patients
GERD treatmentIPF (may be contributory)
Pulmonary hypertension screeningHRCT + Doppler echo if suspected
Lung transplant referralAt diagnosis for IPF; advanced disease in others

Recent Evidence (2024–2025)

  • Corticosteroids in acute ILD exacerbations [PMID 39721758] — a 2025 systematic review confirms their use but highlights unproven benefit and high mortality.
  • Antisynthetase syndrome-ILD [PMID 39083028] — 2025 SR/MA supports immunosuppression; rituximab emerging as effective option.
  • ERS guidelines on pulmonary alveolar proteinosis [PMID 39147411] — whole-lung lavage remains standard.
— Washington Manual of Medical Therapeutics, pp. 365–370; Goldman-Cecil Medicine, pp. 934, 2830; Murray & Nadel's Textbook of Respiratory Medicine
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