Bea Tan, MD

Internal
Interstitial Lung Medicine –
Adult
Disease Pulmonology
 Interstitial Lung Disease
•  Progressive exertional dyspnea or a persistent
nonproductive cough
•  Hemoptysis, wheezing, and chest pain
•  Opacities on chest x-ray focuses on one of the
ILDs
•  ILD: involve the parenchyma of the lung—the
alveoli, the alveolar epithelium, the capillary
endothelium, and the spaces between those
structures—as well as the perivascular and
lymphatic tissues
 INTERSTITIAL LUNG DISEASE

•  Major underlying histopathology:

1.  associated with predominant inflammation and fibrosis
2.  predominantly granulomatous reaction in interstitial or
vascular areas

•  Chronicity:
1.  Acute: (days to weeks) e.g. allergy, acute interstitial
pneumonia, hypersensitivity pneumonitis
2.  Subacute: (weeks to months) e.g. may occur in all ILDs but
is seen especially in sarcoidosis, drug-induced ILDs, the
alveolar hemorrhage syndromes, cryptogenic organizing
pneumonia (COP), and the acute, immunologic
pneumonia that complicates SLE or polymyositis

 Interstitial lung disease
3. Chronic: (months to years) IPF, sarcoidosis,
pulmonary Langerhans cell histiocytosis (PLCH),
pneumoconioses, and CTDs
4. Episodic presentations are unusual and
include eosinophilic pneumonia,
hypersensitivity pneumonitis, COP, vasculitides,
pulmonary hemorrhage, and Churg-Strauss
syndrome
Interstitial lung disease
Interstitial lung disease
Interstitial lung disease
 Interstitial lung disease
Granulomatous Lung Disease:
•  Accumulation of T lymphocytes, macrophages, and
epithelioid cells organized into discrete structures
(granulomas) in the lung parenchyma
•  May progress into fibrosis

Inflammation and Fibrosis:
•  Started as an injury to the epithelial surface that causes
inflammation in the air spaces and alveolar walls
•  chronic, inflammation spreads to adjacent portions of the
interstitium and vasculature and eventually causes
interstitial fibrosis
 Interstitial lung disease
•  History:
1.  Age of onset (e.g. IPF >60y.o.)
2.  Gender: (e.g. PLCH women)
3.  Smoking history (e.g. 75% of IPF are smokers)
4.  Occupational history
5.  Symptomatology: dyspnea is the most common presentation
•  Physical Examination:
1.  not specific
2.  reveals tachypnea and bibasilar end-inspiratory dry crackles,
which are common in most forms of ILD associated with
inflammation
•  less likely to be heard in the granulomatous lung diseases
 Interstitial lung disease
DIAGNOSTIC MODALITIES:
1.  Chest X-Ray: non-specific; most commonly
reveals a bibasilar reticular pattern, nodular or
mixed pattern of alveolar filling and increased
reticular markings
•  Subgroups of ILDs exhibit nodular opacities with
a predilection for the upper lung zones:
(sarcoidosis, PLCH, chronic hypersensitivity
pneumonitis, silicosis, berylliosis, RA [necrobiotic
nodular form], ankylosing spondylitis).
Interstitial lung disease
 Interstitial Lung disease
2. HRCT: better assessment than chest xray,
extent, distribution and pattern of disease

 Interstitial lung disease
4. Pulmonary function test: prognostication
•  Most forms of ILD produce a restrictive defect with
reduced total lung capacity (TLC), functional residual
capacity, and residual volume
5. DLCO: decreased due in part to effacement of the alveolar
capillary units but, more important, to mismatching of
ventilation and perfusion (V. /Q. )
6. ABG: normal or mild hypoxemia at rest, exacerbated by
exercise, hypercapnia: end stage ILD
7. Cardiopulmonary testing: severe exercise-induced
hypoxemia may go undetected, it is useful to perform
exercise testing with measurement of arterial blood gases to
detect abnormalities of gas exchange
 Interstitial lung disease
8. Fiberoptic bronchoscopy with
bronchoalveolar lavage: to rule out other
differentials
9. Lung biopsy: most effective method of
confirming the diagnosis ( transbronchial lung
biopsy or open lung biopsy)
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Bibasal infiltrates Honeycombing
 Interstitial lung disease
Egg-shell density Kerley’s B Lines
Interstitial lung disease
 Interstitial lung disease
TREATMENT:
•  course of ILD is variable, progression is common
and often insidious
•  therapy does not reverse fibrosis, the major goals
of treatment are permanent removal of the
offending agent, and early identification and
aggressive suppression of the acute and chronic
inflammatory process
•  Hypoxemia (Pao2 <55 mmHg) at rest and/or with
exercise should be managed with supplemental
oxygen
 Interstitial lung disease
Glucocorticoid Therapy:
•  recommended for symptomatic ILD patients with
eosinophilic pneumonias, COP, CTD, sarcoidosis,
hypersensitivity pneumonitis, acute inorganic dust
exposures, acute radiation pneumonitis, DAH, and drug-
induced ILD. In organic dust disease
•  Glucocorticoids are recommended for both the acute and
chronic stages
•  SD:prednisone, 0.5–1 mg/kg in a once-daily oral dose for
4–12 weeks
•  If stable or improved dose is tapered to 0.25–0.5 mg/kg
and maintained at this level for an additional 4–12 weeks
 Interstitial lung disease
•  Cyclophosphamide, azathioprine (1–2 mg/kg
per day), and mycophenolate mofetil with or
without glucocorticoids
•  have been tried with variable success in IPF,
vasculitis, progressive systemic sclerosis, and
other ILDs
•  Objective response usually requires at least
8–12 weeks to occur