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 |  |  |