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Table 7 Therapies for select types of ILD

From: Diagnosis and management of interstitial lung disease

ILD type

Key features of immunopathogenesis

Current therapy*

Additional and/or alternative therapies

IPF

• Prominent fibroblast proliferation and matrix deposition

Supportive care

Anti-reflux therapy

• Patchy, temporally heterogeneous changes

Consider anti-reflux measures

N-acetylcysteine

• Architectural distortion of tissue

- Anti-reflux surgery

Clinical trials

• Epithelial injury, microvascular remodeling

- Acid suppressants (e.g. PPI)

(experimental)

• Variable inflammatory component (usually minimal/mild)

Pirfenidone (not approved in US)

• Areas of NSIP- and DIP-like change often present

• PH frequently present with advanced disease

Lung transplantation

Sarcoidosis

• Well-formed non-caseating granulomata in tissues

Observation (mild/stable disease)

Infliximab

• Extra-pulmonary disease may be present

Other IS agent

• May be asymptomatic; may resolve spontaneously without therapy

Corticosteroids (oral or inhaled)

Lung transplantation

Methotrexate

NSIP

• Homogeneous, diffuse involvement of the lung

Corticosteroids

Other IS drugs

• Histopathologic subtypes include cellular (prominent lymphocyte influx; best prognosis), mixed (cellular & fibrotic), & fibrotic (worst prognosis)

Mycophenolate

Lung transplantation

• Usually responsive to IS (less likely to respond if advanced fibrosis is established)

COP

• Prominent inflammatory cell infiltrate (↑ lymphocytes, neutrophils, and/or eosinophils can all be present)

Corticosteroids

Other IS drugs

Macrolides

• Usually responds to IS therapy; relapse frequently occurs

HP

• Prominent lymphocyte influx with formation of loose granulomata

Exposure cessation

Other IS drugs

• Can have appearance of cellular NSIP or OP

Corticosteroids

Lung transplantation

• Can progress to advanced fibrosis (and masquerade as IPF or fibrotic NSIP)

Eosinophilic pneumonia

• Prominent influx of eosinophils

Corticosteroids

Other IS drugs

• Usually responsive to IS therapy

CTD-ILD

• Lung histopathology can reveal NSIP (common), UIP (less common); other ILD (e.g. OP, DIP, RBILD – very uncommon)

Corticosteroids

Anti-reflux therapy

Mycophenolate

Lung transplantation

• PH often present (with or without ILD)

Other DMARD agent(s)

Treatment of PH

AIP/DAD

• Intense inflammation and alveolar damage

Corticosteroids

Cytotoxic drugs

• Hyaline membrane formation

• Prominent neutrophil influx early

  
  1. *Therapies that are usually administered on the basis of expert opinion and clinical trial results; none have received US Food and Drug Administration approval for the indication of ILD/IPF (but pirfenidone is approved for treatment of IPF in some countries, and many DMARD agents are approved for treatment of CTD).
  2. Abbreviations: AIP acute interstitial pneumonia, COP cryptogenic organizing pneumonia, CTD-ILD connective tissue disease-associated ILD, DAD diffuse alveolar damage, DIP desquamative interstitial pneumonia, DMARD disease-modifying anti-rheumatic drug, HP hypersensitivity pneumonitis, IPF idiopathic pulmonary fibrosis, ILD interstitial lung disease, IS immunosuppression, NSIP non-specific interstitial pneumonia, OP organizing pneumonia, PH pulmonary hypertension, RBILD respiratory bronchiolitis with interstitial lung disease.