Interstitial lung diseases (ILD) are a heterogeneous group of
parenchymal lung disorders eventually resulting in respiratory failure
that can be classified into those with known and unknown causes.
ILD are characterized by deposition of cellular and non-cellular
components into the lung parenchyma but vary widely in etiology,
clinico-radiologic presentation, histiopathologic features, and
clinical course. A combination of clinical context, medical and
environmental history, physiologic testing, lung imaging, serologic testing
and histiologic findings obtained through bronchoscopic or surgical
lung biopsy provides the basis for a precise diagnosis. In particular, high-resolution
computed tomography of the chest and recognition of the prognostic
importance of separating usual interstitial pneumonia (UIP) from
other idiopathic interstitial pneumonias (IIP) have profoundly changed
the management of patients with ILD. Besides, smoking cessation,
avoidance of antigen or inorganic dust exposure, corticosteroids
alone or in combination with azathioprine and cyclophosphamide are still
the mainstay of therapy for most entities. However, the poor response
rates to immunosuppressive therapy for UIP patients and frequent
adverse effects highlight the need for new therapeutic strategies
confirmed by randomized clinical trials to guide treatment. So far,
lung transplantation remains the treatment of last resort for eligible
patients with progressive lung fibrosis and timely referral for
transplant evaluation is mandatory owing to limited donor availability.
1
American Thoracic Society/European
Respiratory Society International Multidisciplinary Consensus Classification
of the Idiopathic Interstitial Pneumonias.
Am J Respir
Crit Care Med.
2002;
165
(2)
277-304
2
American Thoracic Society .
Idiopathic pulmonary fibrosis: diagnosis and treatment. International
consensus statement. American Thoracic Society (ATS), and the European
Respiratory Society (ERS).
Am J Respir Crit Care Med.
2000;
161
(2 Pt 1)
646-64
4
Bradley B, Branley H M, Egan J J. et al .
British Thoracic Society
Interstitial Lung Disease Guideline Group, British Thoracic Society
Standards of Care Committee; Thoracic Society of Australia; New
Zealand Thoracic Society; Irish Thoracic Society. Interstitial lung
disease guideline: the British Thoracic Society in collaboration
with the Thoracic Society of Australia and New Zealand and the Irish
Thoracic Society.
Thorax.
2008;
63 Suppl 5
v1-58
5 Coultras D B, Hubbard R. Epidemiology of idiopathic
pulmonary fibrosis. In: Lynch III JP Idiopathic
pulmonary fibrosis, lung biology in health and disease. New
York; Marcel Dekker 2004: 772
6
Demedts M, Behr J, Buhl R. et
al, IFIGENIA Study Group .
High-dose acetylcysteine
in idiopathic pulmonary fibrosis.
N Engl J Med.
2005;
353
(21)
2229-42
8
Hoyles R K, Ellis R W, Wellsbury J. et al .
A multicenter, prospective, randomized,
double-blind, placebo-controlled trial of corticosteroids and intravenous
cyclophosphamide followed by oral azathioprine for the treatment
of pulmonary fibrosis in scleroderma.
Arthritis Rheum.
2006;
54
(12)
3962-70
12
Leuchte H H, Baumgartner R A, Nounou M E. et al .
Brain natriuretic
peptide is a prognostic parameter in chronic lung disease.
Am
J Respir Crit Care Med.
2006;
173
(7)
744-50
17
Orens J B, Estenne M, Arcasoy S. et al .
International guidelines for the selection
of lung transplant candidates: 2006 update – a consensus report
from the Pulmonary Scientific Council of the International Society
for Heart and Lung Transplantation.
J Heart Lung Transplant.
2006;
25
(7)
745-557
19
Swigris J J, Olson A L, Fischer A. et al .
Mycophenolate mofetil is safe, well tolerated,
and preserves lung function in patients with connective tissue disease-related
interstitial lung disease.
Chest.
2006;
130
(1)
30-6
20
Travis W D, Hunninghake G, King Jr T E. et al .
Idiopathic nonspecific
interstitial pneumonia: report of an American Thoracic Society project.
Am J Respir Crit Care Med.
2008;
177
(12)
1338-47
