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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
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
• 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.