SYNONYMS OR OTHER RELATED DISEASES UNDER THIS : DIFFUSE PARENCHYMAL LUNG DISEASE, IDIOPATHIC INTERSTITIAL FIBROSIS(IPF), DESQUAMATIC INTERSTITIAL PNEUMONIA(DIP), RESPIRATORY BRONCHIOLITIS INTERSTITIAL LUNG DISEASE(RBILD), ACUTE INTERSTITIAL PNEUMONITIS (AIP), NONSPECIFIC INTERSTITIAL PNEUMONITIS (NSIP), CRYPTOGENIC ORGANISING PNEUMONIA (COP), LYMPHOCYTIC INTERSTITIAL PNEUMONIA (LIP), SARCOIDOSIS, HYPERSENSITIVITY PNEUMONIA (HSP), PULMONARY LANGERHANS CELL HISTIOCYTOSIS (PLCH), LYMPHANGIOLEIOMYOMATOSIS (LAM).
Medical Care:
" Treatment is best determined by the specific diagnosis. Unfortunately, a specific etiology often is not determined. General supportive measures include the following:
o Smoking cessation should be counseled.
o Good pulmonary hygiene is important.
o Supplemental oxygen therapy may be useful for any patients who demonstrate significant hypoxemia (SaO2 <89% or PaO2 <55 mm Hg while breathing room air).
o If inhalational exposures are thought to be the etiology (occupational), removal from exposure is prudent.
o If a toxic medication is suspected, its discontinuation is mandated.
o Respiratory infections should be treated promptly.
" Pharmacologic therapy with corticosteroids (eg, prednisone) and/or cytotoxic agents for their potential steroid-sparing effect (eg, cyclophosphamide, azathioprine, or methotrexate) may be indicated for specific diagnoses.
" Other immunosuppressive or antifibrotic agents such as colchicine, cyclosporine, and D-penicillamine may have a role in specific cases. Empiric use of these medications without a specific diagnosis should be discouraged because they have significant toxicities.
o Interferon-gamma-1b, pirfenidone, and acetylcysteine have recently been studied for treatment of IPF. Interferon-gamma-1b initially appeared to have a favorable effect. This, however, was not supported in a larger follow-up study. There is some evidence to suggest that pirfenidone and acetylcysteine may have some benefit in IPF. Further investigation is still needed, particularly in other forms of DPLD.
Surgical Care:
" Surgery in the form of either thoracoscopic (preferred) or open lung biopsy is indicated to obtain tissue specimens for definitive diagnosis.
" More recently, lung transplantation has become a treatment option for selected patients with advanced disease.
o Survival rates worldwide after single lung transplantation are approximately 74% at 1 year, 58% at 3 years, 47% at 5 years, and 24% at 10 years.
o Survival rates are lower for bilateral lung transplantation.
o Following transplantation, patients overall report improved quality of life with better physical, social, and general health functioning.
Diet:
" No specific dietary restrictions are warranted for affected patients.
" Some suggest that antioxidants have a therapeutic benefit.
Activity:
" Encourage exercise and pulmonary rehabilitation because they may improve a patient's functional status. However, these activities generally have no effect on disease progression.