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Interstitial lung disease: A clinical overview and general approach. By Prof. Ramadan Nafae Professor and Head of Chest Department Zagazig , Faulty of Medicine. Items:. Definition Epidemiology Classification Pathogenesis Diagnosis Treatment Final comments. Items:. Definition
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Prof. Ramadan Nafae
Professor and Head of Chest Department
Zagazig , Faulty of Medicine
HAMMAN and RICHwere the first to describe (in 1935 and 1944) four patients who died of rapidly progressive lung disease characterized by diffuse interstitial pneumonia and fibrosis.
It is clear that the disease is not restricted to the interstitium as it involves epithelial, endothelial and mesenchymal cells, macrophages and recruited inflammatory cells, secreted proteins, and aberration of matrix component within the alveolar space. In addition, the disease process extends into the alveolar space, acini, bronchiolar lumen and bronchioles.
ILD is a heterogeneous syndrome with the following common clinical features:
Idiopathic interstitial pneumonias
Other forms of DPLD, eg, LAM, HX, etc
DPLD of known cause, eg, drugs or association, eg, collagen vascular disease
Granulomatous DPLD, eg, sarcoidosis
Idiopathic pulmonary fibrosis
IIP other than idiopathic pulmonary fibrosis
Respiratory bronchiolitis interstitial lung disease
Desquamative interstitial pneumonia
Cryptogenic organizing pneumonia
Acute interstitial pneumonia
Lymphocytic interstitial pneumonia
Nonspecific interstitial pneumonia (provisional)
ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2002;165:277-304.
HC, honeycombing; GGO, ground glass opacity; FF, fibrotic foci; M, macrophage
responses to inhaled agents
Inhaled environmental agents
(fumes, dust, smoke)
Alveolar epithelial cell injury
Wound healing (inflammation,
– Etiologic agent
– Recurrent vs single
– Endothelial vs epithelial
Thannickal VJ, et al. Annu Rev Med. 2004;55:395-417.
Multidimensional and multidisciplinary
The patient's age, cigarette-smoking status and sex may provide useful clues.
Thorough medical history that must include a review of environmental factors, occupations, exposures, medication, and drug usage and family medical history.
Smoking – related ILD :
Physical examination of the respiratory system is rarely helpful in the diagnostic evaluation of interstitial lung diseases.
The classical “Velcro rales” or inspiratory crackles, occur not only in most patients with IPF but also in many other interstitial lung diseases.
Extrathoracic findings can be insightful e.g.
Langerhans’ cell granulomatosis.
Document the existence, gauge the severity and provide clues that are useful in the differential diagnosis of ILD. Also they are useful in the monitoring of clinical progression of the disease or response to therapy.
Exercise affords the most sensitive diagnostic and physiological test for ILD. The degree of arterial hypoxemia induced by exercise and the alveolar-arterial difference in P02 (PAO2 – PaO2 gradient) correlate well with the degree of pulmonary fibrosis.
All major criteria and at least 3 minor criteria must be present to increase the likelihood of an IPF diagnosis
ATS/ERS. Am J Respir Crit Care Med. 2000;161:646-664.
A cytokine that stimulates the granulocytes, macrophages, dendritic cells, and bone marrow precursors of platelets. Administered by either S.C injection or aerosolized form. It has recently been demonstrated to effectively control disease course and provide a very useful alternative to traditional therapy of whole lung lavage