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
Prof. Ramadan Nafae
Professor and Head of Chest Department
Zagazig , Faulty of Medicine
ILD is a heterogeneous syndrome with the following common clinical features:
Diffuse Parenchymal Lung Disease (DPLD) 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
Four proposed mechanisms and potential variations in lung clinical features:
responses to inhaled agents
Inhaled environmental agents
(fumes, dust, smoke)
Alveolar epithelial cell injury
Wound healing (inflammation,
LUNG INJURY clinical features:
– Etiologic agent
– Recurrent vs single
– Endothelial vs epithelial
Thannickal VJ, et al. Annu Rev Med. 2004;55:395-417.
Multidimensional and multidisciplinary
ILD presents a clinical conundrum as; clinical features:
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 :
History (cont.) clinical features:
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.
Computed tomography and high-resolution CT images clinical features:
Differential diagnosis by function clinical features::
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.
"Bronchoalveolar lavage" physiological test for ILD. The degree of arterial hypoxemia induced by exercise and the alveolar-arterial difference in P02 (PAO2
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
Other measures Lung Biopsy
Thank You Lung Biopsy